*Classify as SLE if total score ≥10 points

5. Antiphospholipid Antibody Syndrome

Revised classification criteria for antiphospholipid syndrome

Antiphospholipid antibody syndrome (APS) is present if at least one of the clinical criteria and one of the laboratory criteria that follow are met.*

Clinical criteria

1. Vascular thrombosis#

ne or more clinical episodes‡of arterial, venous, or small vessel thrombosis§in any tissue or organ. Thrombosis must be confirmed by objective validated criteria (i.e., unequivocal findings of appropriate imaging studies or histopathology). For histopathologic confirmation, thrombosis should be present without significant evidence of inflammation in the vessel wall

2. Pregnancy morbidity

a) One or more unexplained deaths of a morphologically normal foetusat or beyond the 10th week of gestation with normal foetal morphology documented by ultrasound or by direct examination of the foetusOR

b) One or more premature births of a morphologically normal neonate before the 34th week of gestation because of (i) eclampsia or severe preeclampsia defined according to standard definitions1 or (ii) recognized features of placental insufficiency∥or

c) Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded.

In studies of populations of patients who have more than one type of pregnancy morbidity, investigators are strongly encouraged to stratify groups of subjects according to a, b, or c above.

Laboratory criteria

*Classification of APS should be avoided if less than 12 weeks or more than 5 years separates the positive aPL test and the clinical manifestation.

#Coexisting inherited or acquired factors for thrombosis are not reasons for excluding patients from APS trials. However, two subgroups of APS patients should be recognized, according to (a) the presence and (b) the absence of additional risk factors for thrombosis. Indicative (but not exhaustive) such cases include age (>55 years in men and >65 years in women) and the presence of any of the established risk factors for cardiovascular disease (hypertension, diabetes mellitus, elevated LDL or low HDL cholesterol, cigarette smoking, family history of premature cardiovascular disease, BMI ≥30 kg/m2, microalbuminuria, estimated GFR <60 mL min−1, inherited thrombophilia, oral contraceptives, nephrotic syndrome, malignancy, immobilization, and surgery. Thus, patients who fulfil criteria should be stratified according to contributing causes of thrombosis.

‡A thrombotic episode in the past could be considered as a clinical criterion, provided that thrombosis is proved by appropriate diagnostic means and that no alternative diagnosis or cause of thrombosis is found.

Superficial venous thrombosis is not included in the clinical criteria.

∥Generally accepted features of placental insufficiency include (i) abnormal or nonreassuring foetal surveillance test(s) (e.g., a nonreactive nonstress test, suggestive of foetal hypoxemia), (ii) abnormal Doppler flow velocimetry waveform analysis suggestive of foetal hypoxemia (e.g., absent end-diastolic flow in the umbilical artery), (iii) oligohydramnios (e.g., an amniotic fluid index of ≤5 cm), or (iv) a postnatal birth weight less than the 10th percentile for gestational age.

¶ Investigators are strongly advised to classify patients with APS in studies into one of the following categories: I, more than one laboratory criterion present (any combination); IIa, LA present alone; IIb, aCL antibody present alone; and IIc, anti-β2 glycoprotein-I antibody present alone.

• 1. American College of Obstetricians and Gynecologists. Diagnosis and Management of Preeclampsia and Eclampsia. ACOG Practice Bulletin No. 33. Washington, DC: American College of Obstetricians and Gynecologists; 2002
• 2. Wisloff F, Jacobsen EM, Liestol S. Laboratory diagnosis of the antiphospholipid syndrome. Thromb Res. 2002;108:263-271.
• 3. Brandt JT, Triplett DA, Alving B, Scharrer I. Criteria for the diagnosis of lupus anticoagulants: an update. On behalf of the Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the ISTH. Thromb Haemost. 1995;74:1185-1190.
• 4. Tincani A, Allegri F, Sanmarco M, et al. Anticardiolipin antibody assay: a methodological analysis for a better consensus in routine determinations—a cooperative project of the European Antiphospholipid Forum. Thromb Haemost. 2001;86:575-583.
• 5. Harris EN, Pierangeli SS. Revisiting the anticardiolipin test and its standardization. Lupus. 2002;11:269-275.
• 6. Wong RC, Gillis D, Adelstein S, et al. Consensus guidelines on anti-cardiolipin antibody testing and reporting. Pathology. 2004;36:63-68.
• 7. Reber G, Tincani A, Sanmarco M, et al. Proposals for the measurement of anti-beta2-glycoprotein I antibodies. Standardization group of the European Forum on Antiphospholipid Antibodies. J Thromb Haemost. 2004;2:1860-1862.

Reference: Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306.

Preliminary Criteria for the Classification of Catastrophic Antiphospholipid Syndrome (APS)

• 1. Evidence of involvement of three or more organs, systems, or tissues*
• 2. Development of manifestations simultaneously or in less than 1 week
• 3. Confirmation by histopathology of small vessel occlusion in at least one organ or tissue†
• 4. Laboratory confirmation of the presence of antiphospholipid antibody (lupus anticoagulant or anticardiolipin or anti–β2-glycoprotein I antibodies)‡

Definite Catastrophic APS- All four criteria

Probable Catastrophic APS

Criteria 2 through 4 and two organs, systems, or tissues involved

Criteria 1 through 3, except no confirmation 6 week apart owing to early death of patient not tested before catastrophic episode Criteria 1, 2, and 4

Criteria 1, 3, and 4 and development of a third event more than 1 week but less than 1 month after the first, despite anticoagulation

*Usually, clinical evidence of vessel occlusions, confirmed by imaging techniques when appropriate. Renal involvement is defined by a 50% rise in serum creatinine, severe systemic hypertension, proteinuria, or some combination of these.

†For histopathologic confirmation, significant evidence of thrombosis must be present, although vasculitis may coexist occasionally.

‡If the patient has not been diagnosed previously with APS, laboratory confirmation requires that the presence of antiphospholipid antibody be detected on two or more occasions at least 6 weeks apart (not necessarily at the time of the event), according to proposed preliminary criteria for the classification of APS.

Reference: Asherson RA, Cervera R, de Groot PG, et al: Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines, Lupus 12:530–534, 2003.

6. Systemic Sclerosis

Establishing a Diagnosis of Scleroderma

1980 ACR Criteria

Must have (a) or two of (b), (c), or (d):

• 1. Proximal SSc (proximal to MCPs/MTPs)
• 2. gital pits
• 3. Sclerodactyly
• 4. Pulmonary fibrosis (chest radiograph; HRCT)

CREST Criteria

Must have three of the five features:

• 1. Calcinosis
• 2. Raynaud’s phenomenon
• 3. Esophageal dysmotility
• 4. Sclerodactyly
• 5. Telangiectasias

Minor Criteria*†

Must have all three:

• 1. Definite Raynaud’s phenomenon
• 2. Abnormal capillary loops
• 3. Specific scleroderma autoantibody

  *Some experts continue to classify patients with minor criteria only as

  undifferentiated connective disease with scleroderma features.

  †Autoantibody includesanticentromere,

  Anti-topoisomerase 1 (Scl-70), and anti-RNA polymerase III.

  2013 American College of Rheumatology/European League Against Rheumatism Criteria for the Classification of Systemic Sclerosis*

  Item	Sub-item	Weight or score†
  Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (sufficient criterion)		9
  Skin thickening of the fingers (only count the higher score)	Puffy fingers Sclerodactyly of the fingers (distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints)	2 4
  Fingertip lesions (only count the higher score)	Digital tip ulcers Fingertip pitting scars	2 br 3
  Telangiectasia		2
  Abnormal nailfold capillaries		2
  Raynaud phenomenon		3
  Pulmonary arterial hypertension or interstitial lung disease (maximum score is 2)	Pulmonary arterial hypertension Interstitial lung disease	2 2
  SSc-related autoantibodies (anticentromere, anti– topoisomerase I [anti–Scl-70], anti–RNA polymerase III) (maximum score is 3)	Anticentromere Anti–topoisomerase I Anti–RNA polymerase III	3

  *These criteria are applicable to any patient considered for inclusion in a systemic sclerosis (SSc) study. The criteria are not applicable to patients with skin thickening sparing the fingers or to patients who have a scleroderma-like disorder that better explains their manifestations (e.g., nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromyalgia, porphyria, lichen sclerosis, graft-versus-host disease, diabetic cheiroarthropathy).

  †The total score is determined by adding the maximum weight (score) in each category. Patients with a total score of ≥9 are classified as having definite SSc.

  Reference: van den Hoogen F, Khanna D, Fransen J, Johnson SR, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. Nov;65(11):2737-2747.

  Definitions of Items and Subitems in the American College of Rheumatology/European League Against Rheumatism Criteria for the Classification of Systemic Sclerosis

  Item	Definition
  Skin thickening	Skin thickening or hardening not caused by scarring after injury, trauma, and so on
  Puffy fingers	Swollen digits: a diffuse, usually nonpitting increase in soft tissue mass of the digits extending beyond the normal confines of the joint capsule. Normal digits are tapered distally with the tissues following the contours of the digital bone and joint structures. Swelling of the digits obliterates these contours. Not from other causes such as inflammatory dactylitis.
  Fingertip ulcers or pitting scars	Ulcers or scars distal to or at the proximal interphalangeal joint not thought to be caused by trauma. Digital pitting scars are depressed areas at digital tips as a result of ischemia rather than trauma or exogenous causes.
  Telangiectasia	Telangiectasias are visible macular dilated superficial blood vessels that collapse upon pressure and fill slowly when pressure is released. Telangiectasias in a scleroderma-like pattern are round and well demarcated and found on the hands, lips, or inside of the mouth or are large mat like telangiectasias. Distinguishable from rapidly filling spider angiomas with central arteriole and from dilated superficial vessels.
  Abnormal nailfold capillary pattern consistent with systemic sclerosis	Enlarged capillaries or capillary loss with or without pericapillary hemorrhages at the nailfold; may also be seen on the cuticle
  Pulmonary arterial hypertension	Pulmonary arterial hypertension diagnosed by right-sided heart catheterization according to standard definitions
  Interstitial lung disease	Pulmonary fibrosis seen on high-resolution computed tomography or chest radiography, most pronounced in the basilar portions of the lungs, or occurrence of “Velcro” crackles on auscultation not from another cause such as congestive heart failure
  Raynaud phenomenon	Self-reported or reported by a physician, with at least a two-phase color change in finger(s) and often toe(s) consisting of pallor, cyanosis, or reactive hyperemia in response to cold exposure or emotion; usually one phase is pallor
  Systemic sclerosis– related autoantibodies	Anticentromere antibody or centromere pattern seen on antinuclear antibody testing, antitopoisomerase I antibody (also known as anti-Scl-70 antibody), or anti-RNA polymerase III antibody; positive according to local laboratory standards

  Reference: van den Hoogen F, Khanna D, Fransen J, Johnson SR, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013 Nov65(11):2737-2747.

  7. IDIOPATHIC INFLAMMATORY MYOSITIS

  BOHAN AND PETER CRITERIA FOR THE DIAGNOSIS OF POLYMYOSITIS AND DERMATOMYOSITIS

  Full criteria can be found in Bohan A, Peter JB. Polymyositis and dermatomyositis (parts 1 and 2). N Engl J Med. 1975;292:34

  Definite PM defined as all first four elements, probable PM as three of first four and possible PM as two of first four; definite DM defined as rash plus three other elements, probable DM as rash plus two others and possible DM as rash plus one other. DM, Dermatomyositis; PM, Polymyositis.

  Reference: Lunberg IE, Miller FW, et al. Diagnosis and classification of idiopathic inflammatory myopathies. J Intern Med. 2016; 280:39-51.

  The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIMs)

  When no better explanation for the symptoms and signs exists, these classification criteria can be used

  	Score points
  Variable	Without muscle biopsy	With muscle biopsy	Definition
  Age of onset 1. Age of onset of first symptom assumed to be related to the disease ≥18 years and <40 years 2. Age of onset of first symptom assumed to be related to the disease ≥40 years	1.3 2.1	1.5 2.2	18 ≤ age (years) at onset of first symptom assumed to be related to the disease <40 Age (years) at onset of first symptom assumed to be related to the disease ≥40
  Muscle weakness 1. Objective symmetric weakness, usually progressive, of the proximal upper extremities 2. Objective symmetric weakness, usually progressive, of the proximal lower extremities 3. Neck flexors are relatively weaker than neck extensors 4. In the legs, proximal muscles are relatively weaker than distal muscles	0.7 0.8 1.9 0.9	0.7 0.5 0.5 1.2	Weakness of proximal upper extremities as defined by manual muscle testing or other objective strength testing, which is present on both sides and is usually progressive over time Weakness of proximal lower extremities as defined by manual muscle testing or other objective strength testing, which is present on both sides and is usually progressive over time Muscle grades for neck flexors are relatively lower than neck extensors as defined by manual muscle testing or other objective strength testing Muscle grades for proximal muscles in the legs are relatively lower than distal muscles in the legs as defined by manual muscle testing or other objective strength testing
  Skin manifestations 1. Heliotrope rash 2. Gottron’s papule 3. Gottron’s rash	3.1 2.1 3.3	3.2 2.7 3.7	Purple, lilac-colored, or erythematous patches over the eyelids or in a periorbital distribution, often associated with periorbital edema Erythematous to violaceous papules over the extensor surfaces of joints, which are sometimes scaly. May occur over the finger joints, elbows, knees, malleoli, and toes Erythematous to violaceous macules over the extensor surfaces of joints, which are not palpable
  Other clinical manifestations Dysphagia or esophageal dysmotility	0.7	0.6	Difficulty in swallowing or objective evidence of abnormal motility of the esophagus
  Laboratory measurements 1. Anti–Jo-1 (anti–histidyl–transfer RNA synthetase) autoantibody present 2. Elevated serum levels of creatine kinase (CK) or lactate dehydrogenase (LDH) or aspartate aminotransferase (ASAT/AST/SGOT) or alanine aminotransferase (ALAT/ALT/SGPT) above upper limits of normal	3.9 1.3	3.8 1.4	Autoantibody testing in serum performed with standardized and validated test, showing positive result The most abnormal test values during the disease course (highest absolute level of enzyme) above the relevant upper limit of normal
  Muscle biopsy features-presence of: 1. Endomysial infiltration of mononuclear cells surrounding, but not invading myofibers 2. Perimysial and/or perivascular infiltration of mononuclear cells 3. Perifascicular atrophy 4. Rimmed vacuoles		1.7 1.7 1.9 3.1	Muscle biopsy reveals endomysial mononuclear cells abutting the sarcolemma of otherwise healthy, non-necrotic muscle fibers, but there is no clear invasion of the muscle fibers Mononuclear cells are located in the perimysium and/or located around blood vessels (in either perimysial or endomysial vessels) Muscle biopsy reveals several rows of muscle fibers, which are smaller in the perifascicular region than fibers more centrally located Rimmed vacuoles are bluish by haematoxylin and eosin staining and reddish by modified Gomori trichrome stain

  Probability of IIM without muscle biopsy = 1/ [1+ exponential(5.33-score)]

  Probability of IIM including muscle biopsy = 1/ [1+ exponential(6.49-score)]

  Classification as IIM	Score without biopsy	Score with biopsy
  Not classified	< 5.3	<6.5
  Possible	5.3 – 5.49	6.5 - 6.69
  Probable	5.5 – 7.49	6.7 – 8.69
  Definite	³ 7.5	³ 8.7

  Reference: Lundberg IE, Tjärnlund A, Bottai M, et al. EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. ARTHRITIS & RHEUMATOLOGY. Vol. 69, No. 12, December 2017, pp 2271–2282.

  “GRIGGS CRITERIA” FOR INCLUSION BODY MYOSITIS

  1. Characteristic features

  A. Clinical

  Proximal and distal of arms and legs and must exhibit at least one of:

  D. Laboratory features

  1. CK <12× normal

  2. Muscle biopsy

  Diagnostic criteria: (i) definite inclusion body myositis (IBM) = all muscle biopsy features. None of the clinical or laboratory features are mandatory; (ii) possible IBM = partial invasion without other pathologic features + characteristic clinical and other laboratory features.

  Reference: Griggs RC, Askanas V, DiMauro S, et al. Inclusion body myositis and myopathies. Ann Neurol. 1995;38:705-713; and Hilton-Jones D, Brady S. Diagnostic criteria for inclusion body myositis. J Intern Med. 2016;280(1):52-62.

  The European Neuromuscular Centre Inclusion Body Myositis (IBM) 2011 Research Diagnostic Criteria

  Clinical and laboratory features	Classification	Pathologic features
  Duration >12 months Age at onset >45 years	Clinicopathologically defined IBM	All of the following: Endomysial inflammatory infiltrate Rimmed vacuoles Protein accumulation* or 15- to 18-nm filaments
  Knee extension weakness ≥ hip flexion weakness and/or Finger flexion weakness > shoulder abduction weakness and Serum CK no greater than 15 × ULN
  Duration >12 months Age at onset >45 years	Clinically defined IBM	One or more, but not all, of: Endomysial inflammatoryinfiltrate Upregulation of MHC class I Rimmed vacuoles
  Knee extension weakness ≥ hip flexion weakness and Finger flexion weakness > shoulder abduction weakness sCK no greater than 15*ULN		Protein accumulation* or 15- to 18-nm filaments
  Duration >12 months Age at onset >45 years	Probable IBM	One or more, but not all, of: Endomysial inflammatory infiltrate Upregulation of MHC class I Rimmed vacuoles
  Knee extension weakness ≥ hip flexion weakness or Finger flexion weakness > shoulder abduction weakness sCK no greater than 15*ULN		Protein accumulation* or 15- to 18-nm filaments

  MHC, Major Histocompatibility Complex; sCK, serum creatine kinase; ULN, Upper limit of normal

  *Demonstration of amyloid or other protein accumulation by established methods (e.g., for amyloid Congo red, crystal violet, thioflavin T/S or for other proteins p62, SMI-31, TDP-43). Current evidence favours p62 in terms of sensitivity and specificity but the literature is limited, and further work is required.

  Reference: Rose MR, et al; ENMC IBM Working Group.188th ENMC International Workshop: Inclusion Body Myositis, 2-4 December 2011, Naarden, The Netherlands. Neuromuscul Disord. 2013;23(12):1044-1055.

  8. MIXED CONNECTIVE TISSUE DISEASE

  Alarcòn Segovia Criteria

  Serologic criteria	Anti-RNP at hemagglutination titer of ≥1 : 1600
  Clinical criteria	1. Swollen hands 2. Synovitis 3. Myositis (biologically proven) 4. Raynaud’s phenomenon 5. Acrosclerosis
  MCTD present if:	Serologic criterion accompanied by 3 or more clinical criteria, one of which must include synovitis or myositis

  Kahn Criteria

  Serologic criteria	High-titer anti-RNP corresponding to a speckled ANA of ≥1 : 1200 titer
  Clinical criteria	1. Swollen fingers 2. Synovitis 3. Myositis 4. Raynaud’s phenomenon
  MCTD present if	Serologic criterion accompanied by Raynaud’s phenomenon and 2 or more of the 3 remaining clinical criteria

  Reference: Alarcon-Segovia D, Cardiel MH: Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients, J Rheumatol 16(3):328–334, 1989.

  For Kasukawa and Sharp’s criteria refer to review article on MCTD by Yolanda Farhey and Evelyn V. Hess at onlinelibrary.wiley.com/doi/pdf/10.1002/art.1790100508

  9. GIANT CELL ARTERITIS

  1990 American College of Rheumatology Criteria for the Classification of Giant Cell (Temporal) Arteritis*

  *For purposes of classification, a patient shall be said to have giant cell (temporal) arteritis if at least three of these five criteria are present.

  Reference: Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990;33(8):1122-1128.

  Polymyalgia rheumatica

  Required criteria: age ≥50 years, bilateral shoulder aching, and abnormal CRP or ESR*

  	Points without US (0-6)	Points with US (0-8)
  Morning stiffness duration ≥45 minutes	2	2
  Hip pain or limited range of motion	1	1
  Absence of RF or ACPA	2	2
  Absence of other joint involvement	1	1
  At least one shoulder with subdeltoid bursitis or biceps tenosynovitis or glenohumeral synovitis (either posterior or axillary) and at least one hip with synovitis or trochanteric bursitis	NA	1
  Both shoulders with subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis	NA	1

  *A score of 4 or more is categorized as polymyalgia rheumatica (PMR) in the algorithm without ultrasound (US), and a score of 5 or more is categorized as PMR in the algorithm with US.

  Reference: Dasgupta B, Cimmino MA, Kremers HM, et al. 2012 Provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. Arthritis Rheum. 2012;64(4):943-954.

  10. TAKAYASU ARTERITIS

  1990 American College of Rheumatology Criteria for the Classification of Takayasu Arteritis

  Criterion	Definition
  Age at disease onset ≤40 years	Development of symptoms or findings related to Takayasu arteritis at age ≤40 years
  Claudication of extremities	Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities
  Decreased brachial artery pulse	Decreased pulsation of one or both brachial arteries
  BP difference >10 mm Hg	Difference of >10 mm Hg in systolic BP between arms
  Bruit over subclavian arteries or aorta	Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta
  Arteriogram abnormality	Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not caused by arteriosclerosis, fibromuscular dysplasia, or similar causes; changes usually focal or segmental

  Patient shall be said to have Takayasu arteritis if at least three of these six criteria are present. The presence of any three or more criteria yields a sensitivity of 90.5% and a specificity of 97.8%.

  Reference: Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33(8):1129-1134.

  Ishikawa criteria [ 2 Major or One Major with ≥2 minor or ≥4 minor(representing vessel involvement)

  Obligatory
  Age of onset ≤ 40 years and symptoms duration > 1 month
  Major
  Involvement of Left mid-SCA
  Involvement of Right mid-SCA
  Y/ N
  Minor
  Raised ESR
  Carotid artery tenderness
  Hypertension
  AR or annuloaortic ectasia
  Pulmonary artery lesions
  Left mid-common carotid lesion
  Descending thoracic aorta lesion
  Abdominal aorta lesion

  AR, aortic regurgitation; ESR, Erythrocyte sedimentation rate; SCA, sub-clavian artery

  11. KAWASAKI DISEASE

  Classic Kawasaki disease (KD) is diagnosed in the presence of fever for at least 5 days (the day of fever onset is taken to be the first day of fever) together with at least four of the five following principal clinical features. In the presence of ≥4 principal clinical features, particularly when redness and swelling of the hands and feet are present, the diagnosis of KD can be made with 4 days of fever, although experienced clinicians who have treated many patients with KD may establish the diagnosis with 3 days of fever in rare cases:

  A careful history may reveal that ≥1 principal clinical feature was present during the illness but resolved by the time of presentation.

  Patients who lack full clinical features of classic KD are often evaluated for incomplete KD. If coronary artery abnormalities are detected, the diagnosis of KD is considered confirmed in most cases.

  Laboratory tests typically reveal normal or elevated white blood cell count with neutrophil predominance and elevated acute phase reactants such as C-reactive protein and erythrocyte sedimentation rate during the acute phase. Low serum sodium and albumin levels, elevated serum liver enzymes, and sterile pyuria can be present. In the second week after fever onset, thrombocytosis is common.

  Reference: McCrindle BW, Rowley AH, Newburger JW, et al; American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Council on Epidemiology and Prevention. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association. Circulation. 2017;135(17):e927-e999.

  EUROPEAN LEAGUE AGAINST RHEUMATISM/PAEDIATRIC RHEUMATOLOGY EUROPEAN SOCIETY CLASSIFICATION CRITERIA FOR KAWASAKI DISEASE

  Fever persisting for at least 5 days (mandatory criterion) plus four of the following five features:

  In the presence of coronary artery involvement (detected on echocardiography) and fever, fewer than four of the remaining five criteria are sufficient (the exact number of criteria required is to be defined in the validation phase).

  Reference: Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65(7):936-941.

  12. HENOCH-SCHÖNLEIN PURPURA and IgA VASCULITIS

  European League Against Rheumatism/Pediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society Henoch-Schönlein Purpura Criteria (with Glossary) and Classification Definition

  Criterion	Glossary
  Purpura (mandatory criterion)	Purpura (commonly palpable and in crops) or petechiae, with lower limb predominance* not related to thrombocytopenia
  1. Abdominal pain	Diffuse abdominal colicky pain with acute onset assessed by history and physical examination; may include intussusception and GI bleeding
  2. Histopathology	Typically, leukocytoclastic vasculitis with predominant IgA deposit or proliferative glomerulonephritis with predominant IgA deposit
  3. Arthritis and arthralgias	Arthritis of acute onset defined as joint swelling or joint pain with limitation on motion Arthralgia of acute onset defined as joint pain without joint swelling or limitation on motion
  4. Renal involvement	Proteinuria >0.3 g/24 hr or >30 mmol/mg of urine albumin/creatinine ratio on a spot morning sample Hematuria or RBC casts: >5 RBCs per high-power field or red blood cell casts in the urinary sediment or ≥2+ on dipstick

  1990 American College of Rheumatology Criteria for the Classification of Henoch-Schönlein Purpura

  Criterion	Definition
  Palpable purpura	Slightly raised “palpable” hemorrhagic skin lesions, not related to thrombocytopenia
  Age ≤20 years at disease onset	Patient 20 years or younger at onset of first symptoms
  Bowel angina	Diffuse abdominal pain, worse after meals, or the diagnosis of bowel ischemia, usually including bloody diarrhoea
  Wall granulocytes on biopsy	Histologic changes showing granulocytes in the walls of arterioles or venules

  For purposes of classification, a patient shall be said to have Henoch-Schönlein purpura if at least two of these four criteria are present.

  Reference: Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum. 1990;33(8):1114-1121.

  13. BEHҪET DISEASE

  International Criteria for Behçet Disease

  Point score system: scoring ≥4 indicates Behçet disease diagnosis

  Sign or symptom	Points
  Ocular lesions	2
  Genital aphthae	2
  Oral aphthae	2
  Skin lesions	1
  Vascular manifestations	1
  Neurologic manifestations	1
  *Positive pathergy test	1

  *Pathergy test is optional; when it is done, 1 additional point is given for a positive result.

  Reference: Davatchi F, Assaad-Khalil S, Calamia KT, et al, for the International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD). The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28(3):338-347.

  International Study Group criteria for Behçet Disease

  Recurrent oral ulceration	Minor aphthous, major aphthous, or herpetiform ulcers observed by the physician or reliably described by the patient, which recurred at least three times in one 12-month period
  Plus two of
  Recurrent genital ulceration	Aphthous ulceration or scarring observed by the physician or reliably described by the patient
  Eye lesions	Anterior or posterior uveitis or cells in the vitreous body on slit-lamp examination or retinal vasculitis observed by an ophthalmologist
  Skin lesions	Erythema nodosum observed by a physician or patient, pseudofolliculitis, or papulopustular lesions or acneiform nodules observed by a physician in postadolescent patients not on glucocorticoid treatment
  Positive Pathergy test	Read by physician at 24–48 hours

  Reference: International Study Group for Behcet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335:1078-1080.

  Mason and Barnes Criteria for a Diagnosis of Behçet Disease – At least3 Major or 2 Major + 2 Minor

  Major

  Minor

  From R.M. Mason, C.G. Barnes, Behçet’s syndrome with arthritis,Ann. Rheum. Dis. 28 (1969) 95–103

  14. CRYOGLOBULINEMIA

  CLASSIFICATION CRITERIA FOR CRYOGLOBULINEMIC VASCULITIS

  i. Questionnaire item: at least two of the following:

  iv. Clinical item: at least three of the following four (present or past)

  Constitutional symptoms	Fatigue Low-grade fever (37°–37.9°C >10 days; no other cause) Fever (>38°C; no other cause) Fibromyalgia
  Articular involvement	Arthralgias Arthritis
  Vascular involvement	Purpura Skin ulcers Necrotizing vasculitis Hyperviscosity syndrome Raynaud phenomenon
  Neurologic involvement	Peripheral neuropathy Cranial nerve involvement Vasculitic CNS involvement

  v. Laboratory item: at least two of the following three (present)†

  †The fulfilment of the laboratory item in a patient satisfying the criteria highlights the possible presence of cryoglobulinaemic vasculitis even in the absence of serum cryoglobulins by initial testing.

  *Satisfied if at least two of three items (questionnaire, clinical, laboratory) are positive. The patient must be positive for serum cryoglobulins in at least two determinations at a ≥12-week interval.

  Reference: De Vita S, Soldano F, Isola M, et al. Preliminary classification criteria for the cryoglobulinaemic vasculitis. Ann Rheum Dis. 2011;70(7):1183-1190; and Quartuccio L, Isola M, Corazza L, et al. Validation of the classification criteria for cryoglobulinaemic vasculitis. Rheumatology (Oxford). 2014;53(12):2209-2213.

  15. HYPERSENSITIVITY VASCULITIS

  1990 American College of Rheumatology Criteria and Definitions Used for the Classification of Hypersensitivity Vasculitis

  Criteria	Definition
  Age at disease onset >16 years	Development of symptoms after age 16 years
  Medication at disease onset	Medication was taken at the onset of symptoms that may have been a precipitating factor
  Palpable purpura	Slightly elevated purpuric rash over one or more areas of the skin; does not blanch with pressure and is not related to thrombocytopenia
  Maculopapular rash	Flat and raised lesions of various sizes over one or more areas of the skin
  Polymorphonuclear neutrophils in vessel wall	Biopsy demonstrating granulocytes in the wall of a venule or arteriole
  Eosinophils in biopsy	Biopsy demonstrating eosinophils in a venule or arteriole at any location

  For purposes of classification, a patient shall be said to have hypersensitivity vasculitis if at least three of these criteria are present.

  Reference: Calabrese LH, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. Arthritis Rheum. 1990;33(8):1108-1113.

  16. ANCA Vasculitis

  Granulomatosis with polyangiitis

  1990 American College of Rheumatology Criteria for the Classification of Granulomatosis with Polyangiitis (Wegener Granulomatosis)

  *- a patient shall be said to have Wegener granulomatosis if at least two of these four criteria are present

  Reference: Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33:1101-1107.

  Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

  1990 American College of Rheumatology Criteria for the Classification of Churg-Strauss Syndrome

  Four out of Six

  Asthma

  Eosinophilia >10%

  Neuropathy, mono- or poly-

  Pulmonary infiltrates

  Nonfixed paranasal sinus abnormality

  Extravascular eosinophils

  Criterion	Definition
  Asthma	History of wheezing or diffuse high-pitched rales on expiration
  Eosinophilia	Eosinophilia >10% in WBC differential count
  Mono or polyneuropathy	Development of mononeuropathy, multiple mononeuropathies, or polyneuropathy (i.e., glove-and-stocking distribution) attributable to a systemic vasculitis
  Pulmonary infiltrates, non-fixed	Migratory or transitory pulmonary infiltrates on radiographs (not including fixed infiltrates), attributable to a systemic vasculitis
  Paranasal sinus abnormality	History of acute or chronic paranasal sinus pain or tenderness or radiographic opacification of the paranasal sinuses
  Extravascular eosinophils	Biopsy including artery, arteriole, or venule, showing accumulations of eosinophils in extravascular areas

  *- a patient shall be said to have Churg-Strauss syndrome if at least four of these six criteria are positive. The presence of any four or more of the six criteria yields a sensitivity of 85% and a specificity of 99.7%.

  Reference: Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094-1100.

  17. Polyarteritis Nodosa

  3 of the 10 ACR criteria should be present when a radiographic or pathological diagnosis of vasculitis is made

  Reference

  Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990 Aug. 33(8):1088-93

  18. MACROPHAGE ACTIVATION SYNDROME

  Classification criteria - MAS
  2014 [sJIA] *	2016 [sJIA]	jSLE [≥ 1 clinical + ≥ 2lab]
  Laboratory[Lab] criteria - Platelet <2.62 lac/m - SGOT > 59 U/L - WBC <4000/ml - Fibrinogen ≤ 2.6 g/L	A febrile patient with sJIA with ferrtin > 684ng/ml and any two of the following - Platelet ≤ 1.81 lac/ml - SGOT > 48 U/L - TG > 156 mg/dl - Fibrinogen ≤ 360 mg/dl	Clincal - Fever (>380C) Hepatomegaly [3 cm below the costal margin(BCM)] . Splenomegaly (3 cm BCM) . Haemorrhagic manifestations . Central nervous system dysfunction
  Clinical criteria - CNS dysfunction - Haemorrhages - Hepatomegaly (≥3 cm below the costal margin) Histopathological criterion: Evidence of macrophage hemophagocytosis is found in the bone marrow aspirate sample in doubtful cases. *2 Lab or 1 Lab + 1 Clinical criteria	Exclude other causes like infectious hepatitis , ITP, famililial hyperlipidemia	Lab criteria Cytopenia affecting 2 or more cell ineages (WBC ≤ 4000/ml, Hb≤9g/dl or platelet ≤ 1.5lac/ml) . SGOT (>40 units/l) . LDH (>567 units/l) . Fibrinogen ≤ 1.5 gm/l) . Triglycerides >178 mg/dl) . Ferritin >500 mg/l)
  Other important lab parameters – falling ESR, High fibrin degradation products, d-dimer, high PT/APTT, hyponatremia , low albumin, anemia , high LDH, high sIL2αR/sCD25≥ 2400 U/ml, sCD163, low complements in MAS complicating sJIA. Ferritin/ESR ratio > 80

  19. ADULT ONSET STILL’S DISEASE

  Classification criteria by Yamaguchi et al

  Major criteria:

  Minor criteria:

  Exclusion criteria:

  5 criteria: at least 2 major and 3 minor

  Reference: Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19:424e30.

  Classification criteria by Fautrel et al

  Major criteria:

  Minor criteria:

  4 major (or) 3 major and 2 minor criteria are required for classification.

  Reference: Fautrel B, Zing E, Golmard JL, et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002;81:194e200.

  20. RELAPSING POLYCHONDRITIS

  McAdam et al1

  (Requirement: three of six criteria)

  Damiani and Levine2

  (Requirement: any of these)

  Michet et al3

  (Requirement: any of these)

  References:

  From Longo L, Greco A, Rea A, et al. Relapsing polychondritis: a clinical update. Autoimmun Rev. 2016;15(6):539-543

  21. IgG4 Related Disease

  COMPREHENSIVE CLINICAL DIAGNOSTIC CRITERIA FOR IGG4-RELATED DISEASE

  (1) Marked lymphocyte and plasmacyte infiltration and fibrosis

  (2) Infiltration of IgG4+ plasma cells: ratio of IgG4+/IgG+ cells >40% and >10 IgG4+ plasma cells/HPF

  Definite: 1 + 2 + 3

  Probable: 1 + 3

  Possible: 1 + 2

  owever, it is important to differentiate IgG4-RD from malignant tumours of each organ (e.g., cancer, lymphoma) and similar diseases (e.g., Sjögren syndrome, primary sclerosing cholangitis, Castleman disease, secondary retroperitoneal fibrosis, Wegener granulomatosis, sarcoidosis, Churg-Strauss syndrome) by additional histopathologic examination. Even when patients cannot be diagnosed using the CCD criteria, they may be diagnosed using organ-specific diagnostic criteria for IgG4-RD.

  Reference: Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21-30.

  22. OSTEOARTHRITIS

  1986 American College of Rheumatology Criteria for the Classification of Knee Osteoarthritis

  Clinical and laboratory	Clinical and radiographic	Clinical*
  Knee pain + at least five of the following:	Knee pain + at least five of the following:	Knee pain + at least three of six of the following:
  Age >50 years Stiffness <30 minutes Crepitus Bony tenderness Bony enlargement No palpable warmth ESR ≤40 mm/hr RF <1:40 SF signs of OA	Age >50 years Stiffness <30 minutes Crepitus + Osteophytes	Age >50 years Stiffness <30 minutes Crepitus Bony tenderness Bony enlargement No palpable warmth

  Alternate for the clinical category would be 4 out of 6.

  Reference: Altman R, Asch E, Bloch D, et al. The American College of Rheumatology criteria for the classification and reporting of osteoarthritis of the knee. Arthritis Rheum. 1986;29:1039-1049.

  1991 AMERICAN COLLEGE OF RHEUMATOLOGY CRITERIA FOR THE CLASSIFICATION OF OSTEOARTHRITIS OF THE HIP

  Hip pain and at least two of the following three features:

  Reference: Altman R, Alarcon G, Appelrouth D, et al. The American College of Rheumatology criteria for the classification and reporting of osteoarthritis of the hip. Arthritis Rheum. 1991;34:505-514.

  1990 AMERICAN COLLEGE OF RHEUMATOLOGY CLASSIFICATION CRITERIA FOR OSTEOARTHRITIS OF THE HAND

  Hand pain, aching, or stiffness and three or four of the following features:

  * The 10 selected joints are the second and third distal interphalangeal (DIP), the second and third proximal interphalangeal, and the first carpometacarpal joints of both hands.

  Reference: Altman R, Alarcón G, Appelrouth D, et al. The American College of Rheumatology criteria for the classification and reporting of osteoarthritis of the hand. Arthritis Rheum. 1990;33:1601-1610.

  23. GOUT

  American College of Rheumatology/European League Against Rheumatism Gout Classification Criteria

  		Categories	Score
  Step 1	Entry criterion (only apply criteria below to those meeting this entry criterion)	At least 1 episode of swelling, pain, or tenderness in a peripheral joint or bursa
  Step 2	Sufficient criterion (if met, can classify as gout without applying criteria below)	Presence of MSU crystals in a symptomatic joint or bursa (i.e., in synovial fluid) or tophus
  Step 3	Criteria (to be used if sufficient criterion not met)
  Pattern of joint or bursa involvement during symptomatic episode(s) ever†		Ankle or midfoot (as part of monoarticular or oligoarticular episode without involvement of the first metatarsophalangeal joint) Involvement of the first metatarsophalangeal joint (as part of monoarticular or oligoarticular episode)	1 2
  Characteristics of symptomatic episode(s) ever: Erythema overlying affected joint (patient reported or physician observed) Can’t bear touch or pressure to affected joint Great difficulty with walking or inability to use affected joint		One characteristic Two characteristics Three characteristics	1 2 3
  Time course of episode(s) ever: Presence (ever) of ≥2, irrespective of anti-inflammatory treatment Time to maximal pain <24 hours Resolution of symptoms in ≤14 days Complete resolution (to baseline level) between symptomatic episodes		One typical episode Recurrent typical episodes	1 2
  Clinical evidence of tophus: Draining or chalklike subcutaneous nodule under transparent skin, often with overlying vascularity, located in typical locations: joints, ears, olecranon bursae, finger pads, tendons (e.g., Achilles tendon)		Present	4
  Laboratory
  Serum urate: Measured by uricase method. Ideally should be scored at a time when the patient was not receiving urate-lowering treatment and it was >4 weeks from the start of an episode (i.e., during intercritical period); if practicable, retest under those conditions. The highest value irrespective of timing should be scored.		<4 mg/dL (<0.24 mmol/L)‡ 6–8 mg/dL (0.36–<0.48 mmol/L) 8– <10 mg/dL (0.48–<0.60 mmol/L) ≥10 mg/dL (≥0.60 mmol/L)	-4 2 3 4
  Synovial fluid analysis of a symptomatic (ever) joint or bursa (should be assessed by a trained observer) §		MSU negative	-2
  Imaging
  Imaging evidence of urate deposition in symptomatic (ever) joint or bursa: ultrasound evidence of double-contour sign¶ or DECT demonstrating urate deposition*		Present (either modality)	4
  Imaging evidence of gout-related joint damage: conventional radiography of the hands and/or feet demonstrates at least 1 erosion**		Present	4

  §-If polarizing microscopy of synovial fluid from a symptomatic (ever) joint or bursa by a trained examiner fails to show monosodium urate monohydrate (MSU) crystals, subtract 2 points. If synovial fluid was not assessed, score this item as 0.

  ¶- Hyperechoic irregular enhancement over the surface of the hyaline cartilage that is independent of the insonation angle of the ultrasound beam (note: false-positive double-contour sign [artefact] may appear at the cartilage surface but should disappear with a change in the insonation angle of the probe).

  *- The presence of color-coded urate at articular or periarticular sites. Images should be acquired using a dual-energy computed tomography (DECT) scanner, with data acquired at 80 and 140 kV and analyzed using gout-specific software with a two-material decomposition algorithm that colour codes urate. A positive scan is defined as the presence of color-coded urate at articular or periarticular sites. Nailbed, submillimetre, skin, motion, beam hardening, and vascular artefacts should not be interpreted as DECT evidence of urate deposition.

  **- Erosion is defined as a cortical break with sclerotic margin and overhanging edge, excluding distal interphalangeal joints and gull’s wing appearance.

  Reference: Neogi T, Jansen TL, Dalbeth N, et al. 2015 Gout Classification Criteria: an American College of Rheumatology /European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2015;67(10):2557-2568.

  24. FIBROMYALGIA

  1990 AMERICAN COLLEGE OF RHEUMATOLOGY CRITERIA FOR THE CLASSIFICATION OF FIBROMYALGIA

  1. History of widespread pain

  Definition: Pain is considered widespread when all of the following are present: pain in the left side of the body, pain in the right side of the body, pain above the waist, and pain below the waist. In addition, axial skeletal pain (cervical spine or anterior chest or thoracic spine or low back) must be present. In this definition, shoulder and buttock pain is considered as pain for each involved side. “Low back” pain is considered lower segment pain.

  2. Pain in 11 of 18 tender point sites on digital palpation

  Definition: Pain, on digital palpation, must be present in at least 11 of the following 18 sites:

  Occiput: bilateral, at the suboccipital muscle insertions

  Low cervical: bilateral, at the anterior aspects of the intertransverse spaces at C5–C7

  Trapezius: bilateral, at the midpoint of the upper border

  Supraspinatus: bilateral, at origins, above the scapula spine near the medial border

  Second rib: bilateral, at the second costochondral junctions, just lateral to the junctions on upper surfaces

  Lateral epicondyle: bilateral, 2 cm distal to the epicondyles

  Gluteal: bilateral, in upper outer quadrants of buttocks in anterior fold of muscle

  Greater trochanter: bilateral, posterior to the trochanteric prominence

  Knee: bilateral, at the medial fat pad proximal to the joint line

  Digital palpation should be performed with an approximate force of 4 kg.

  For a tender point to be considered “positive,” the subject must state that the palpation was painful. “Tender” is not to be considered “painful.”

  For classification purposes, patients will be said to have fibromyalgia if both criteria are satisfied. Widespread pain must have been present for at least 3 months.

  The presence of a second clinical disorder does not exclude the diagnosis of fibromyalgia.

  Reference: Wolfe F, Smythe HA, Yunus MB, et al. The American College of Rheumatology 1990 criteria for the classification of fibromyalgia: report of the multicentre criteria committee. Arthritis Rheum. 1990;33:160-172.

  2010 AMERICAN COLLEGE OF RHEUMATOLOGY PRELIMINARY CRITERIA FOR THE DIAGNOSIS OF FIBROMYALGIA

  Criteria

  A patient satisfies diagnostic criteria for fibromyalgia if the following three conditions are met:

  Ascertainment

  1. WPI: Note the number of areas in which the patient has had pain over the past week. In how many areas has the patient had pain? Score will be between 0 and 19.

  10. SS scale score:

  *- Somatic symptoms that might be considered: muscle pain, irritable bowel syndrome, fatigue or tiredness, thinking or remembering problem, muscle weakness, headache, pain or cramps in the abdomen, numbness or tingling, dizziness, insomnia, depression, constipation, pain in the upper abdomen, nausea, nervousness, chest pain, blurred vision, fever, diarrhoea, dry mouth, itching, wheezing, Raynaud phenomenon, hives or welts, ringing in ears, vomiting, heartburn, oral ulcers, loss of or change in taste, seizures, dry eyes, shortness of breath, loss of appetite, rash, sun sensitivity, hearing difficulties, easy bruising, hair loss, frequent urination, painful urination, and bladder spasms.

  Reference: Wolfe F, Clauw DJ, Fitzcharles MA, et al. The American College of Rheumatology preliminary diagnostic criteria for fibromyalgia and measurement of symptom severity. Arthritis Care Res (Hoboken). 2010;62(5):600-610.

  2016 Revisions to the 2010/2011 Fibromyalgia Diagnostic Criteria

  “A diagnosis of fibromyalgia is valid irrespective of other diagnoses. A diagnosis of fibromyalgia does not exclude the presence of other clinically important illnesses.”

  Criteria A patient satisfies modified 2016 fibromyalgia criteria if the following 3 conditions are met:

  Ascertainment (1) WPI: note the number of areas in which the patient has had pain over the last week. In how many areas has the patient had pain? Score will be between 0 and 19

  Region	Areas	Score
  Left Upper	Jaw, shoulder girdle, Upper arm, lower arm	0-4
  Right Upper	Jaw, shoulder girdle, Upper arm, lower arm	0-4
  Axial	Neck, Upper back, lower back, upper chest, lower chest	0-5
  Left Lower	Hip, Upper thigh and Lower thigh	0-3
  Right Lower	Hip, Upper thigh and Lower thigh	0-3

  (2) Symptom severity scale (SSS) score

  For the each of the 3 symptoms above, indicate the level of severity over the past week using the following scale:

  0 = No problem

  1 = Slight or mild problems, generally mild or intermittent

  2 = Moderate, considerable problems, often present and/or at a moderate level

  3 = Severe: pervasive, continuous, life-disturbing problems

  The symptom severity scale (SSS) score: is the sum of the severity scores of the 3 symptoms (fatigue, waking unrefreshed, and cognitive symptoms) (0–9) plus the sum (0–3) of the number of the following symptoms the patient has been bothered by that occurred during the previous 6 months:

  (1) Headaches (0–1) (2) Pain or cramps in lower abdomen (0–1) (3) And depression (0–1)

  The final symptom severity score is between 0 and 12 The fibromyalgia severity (FS) scale is the sum of the WPI and SSS. The FS scale is also known as the polysymptomatic distress (PSD) scale.

  Wolfe F, Clauw DJ, FitzCharles M, Goldenerberg D, Häuser W, Katz RS, Russell IJ, Mease PJ, Russell A, Walitt B. 2016 Revisions to the 2010/2011 Fibromyalgia Diagnostic Criteria [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/2016-revisions-to-the-20102011-fibromyalgia-diagnostic-criteria/. Accessed March 21, 2019.

  25. HYPERMOBILITY (Benign Joint Hypermobility Syndrome)

  BRIGHTON CRITERIA

  Major criteria

  Minor criteria

  Requirement for diagnosis

  Any one of the following:

  1- Beighton score

  	Score
  Component	Left	Right
  1. Passive dorsiflexion and hyperextension of the fifth MCP joint beyond 90°	1	1
  2. Passive apposition of the thumb to the flexor aspect of the forearm	1	1
  3. Passive hyperextension of the elbow beyond 10°	1	1
  4. Passive hyperextension of the knee beyond 10°	1	1
  5. Active forward flexion of the trunk with the knees fully extended so that the palms of the hands rest flat on the floor	1

  Beighton P, Horan F. Orthopaedic aspects of the Ehlers-Danlos syndrome. J Bone Joint Surg Br. 196951(3):444-4453.

  Reference: Grahame R. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol. 2000;27:1777-1779.

  26. COMPLEX REGIONAL PAIN SYNDROME

  INTERNATIONAL ASSOCIATION FOR THE STUDY OF PAIN 2007 PROPOSED DIAGNOSTIC CRITERIA FOR COMPLEX REGIONAL PAIN SYNDROME - Budapest consensus criteria

  General definition of the syndrome

  CRPS describes an array of painful conditions that are characterized by a continuing (spontaneous, evoked, or both) regional pain that is seemingly disproportionate in time or degree to the usual course of any known trauma or other lesion. The pain is regional (not in a specific nerve territory or dermatome) and usually has a distal predominance of abnormal sensory, motor, sudomotor, vasomotor, or trophic findings. The syndrome shows variable progression over time.

  To make the clinical diagnosis, the following criteria must be met:

  1. Continuing pain that is disproportionate to any inciting event

  2. Must report at least one symptom in three of the four following categories:

  3. Must display at least one sign at time of evaluation in two or more of the following categories:

  4. There is no other diagnosis that better explains the signs and symptoms

  For research purposes, the diagnostic decision rule should be at least one symptom in all four symptom categories and at least one sign (observed at evaluation) in two or more sign categories.

  Reference: Harden RN, Bruehl S, Stanton-Hicks M, Wilson PR. Proposed new diagnostic criteria for complex regional pain syndrome. Pain Med. 2007;8(4):326-331.

  27. DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS

  Criteria of Resnick and Niwayama

  Reference: Resnick D, Niwayama G. Radiographic and pathologic features of spinal involvement in diffuse idiopathic skeletal hyperostosis (DISH). Radiology, 1976;119:559.

  Criteria of Utsinger

  Reference: Utsinger PD. Diffuse idiopathic skeletal hyperostosis. Clin Rheum Dis. 1985;11:325.

  • • Symmetric proximal muscle weakness
    • Muscle biopsy evidence of myositis
    • Elevated serum muscle enzymes
    • Myopathic changes on electromyography
    • Typical rash of DM (a distinguishing feature for DM and PM)
    • Duration >6 months
    • Age of onset >30 years
    • Weakness
  • Finger flexor weakness
  • Wrist flexor >extensor weakness
  • Quadriceps weakness ≤grade 4 MRC
    • Inflammatory myopathy (with partial invasion)
    • Rimmed vacuoles
    • Either
      • Intracellular amyloid deposits,
      • 15- to 18-nm tubulofilaments by EM
    • 1. Age at disease onset ≥50 years: development of symptoms or findings beginning at age 50 or older
    • 2. New headache: new onset of or new type of localized pain in the head
    • 3. Temporal artery abnormality: temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries
    • 4. ESR rate: ESR ≥50 mm/hr by the Westergren method
    • 5. Abnormal artery biopsy: biopsy specimen with artery showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells
    • 1. Erythema and cracking of lips, strawberry tongue, or erythema of the oral and pharyngeal mucosa
    • 2. Bilateral bulbar conjunctival injection without exudate
    • 3. Rash: maculopapular, diffuse erythroderma, or erythema multiforme–like
    • 4. Erythema and edema of the hands and feet in the acute phase or periungual desquamation in the subacute phase
    • 5. Cervical lymphadenopathy (≥1.5-cm diameter), usually unilatera
    • Changes in peripheral extremities or perineal area
    • Polymorphous exanthema
    • Bilateral conjunctival injection
    • Changes of lips and oral cavity: injection of oral and pharyngeal mucosa
    • Cervical lymphadenopathy
    • Buccal ulceration
    • Genital ulceration
    • Eye lesions
    • Skin lesions
    • Gastrointestinal lesions
    • Thrombophlebitis
    • Cardiovascular lesions
    • Arthritis
    • Central nervous system lesions
    • Family history of Behçet disease
    • Do you remember one or more episodes of small red spots on your skin, particularly involving the lower limbs?
    • Have you ever had red spots on your lower extremities, which leave a brownish colour after their disappearance?
    • Has a doctor ever told you that you have viral hepatitis?
    • Reduced serum C4
    • Positive serum rheumatoid factor
    • Positive serum monoclonal component
    • 1. Nasal or oral inflammation: development of painful or painless oral ulcers or purulent or bloody nasal discharge
    • 2. Abnormal chest radiograph: chest radiograph showing the presence of nodules, fixed infiltrates, or cavities
    • 3. Urinary sediment: microhematuria (>5 RBCs per high-power field) or RBC casts in urine sediment
    • 4. Granulomatous inflammation on biopsy: histologic changes showing granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area (artery or arteriole)
    • Weight loss of 4 kg or more
    • Livedo reticularis
    • Testicular pain/tenderness
    • Myalgia or leg weakness/tenderness
    • Mononeuropathy or polyneuropathy
    • Diastolic blood pressure greater than 90 mm/Hg
    • Elevated blood urea nitrogen (BUN) or creatinine level unrelated to dehydration or obstruction
    • Presence of hepatitis B surface antigen or antibody in serum
    • Arteriogram demonstrating aneurysms or occlusions of the visceral arteries
    • Presence of polymorphonuclear neutrophils in a biopsy specimen from a small- or medium-sized artery
    • Fever >39°C, intermittent, ≥1 week
    • Arthralgia ≥2 weeks
    • Characteristic rash
    • WBC count >10,000/mL (>80% granulocytes)
    • Sore throat
    • Lymphadenopathy or splenomegaly
    • LFT abnormal
    • Negative ANA/RF
    • Infections
    • Malignancies
    • Rheumatic diseases
    • Spiking fever ≥39°C
    • Arthralgia
    • Transient erythema
    • Pharyngitis
    • PMN leukocytes ≥80%
    • Glycosylated ferritin ≤20%
    • Maculopapular rash
    • WBC count >10,000/mL
    • 1. Recurrent chondritis of both auricles
    • 2. Nonerosive inflammatory polyarthritis
    • 3. Chondritis of nasal cartilages
    • 4. Inflammation of ocular structures
    • 5. Chondritis of respiratory tract
    • 6. Cochlear or vestibular damage
    • 1. Three of six McAdam et al’s criteria
    • 2. One of six McAdam et al’s criteria and a positive histologic confirmation
    • 3. Two of six McAdam et al’s criteria and response to corticosteroid or dapsone
    • Proven inflammation in two of three cartilages: auricular, nasal, and laryngotracheal
    • Proven inflammation in one of the above and meeting two other signs from ocular inflammation, hearing loss, vestibular dysfunction, or seronegative inflammatory arthritis
    • 1) McAdam LP, O’Hanlan MA, Bluestone R, Pearson CM. Relapsing polytonicities: prospective study of 23 patients and a review of the literature. Medicine (Baltimore). 1976;55:193-215.
    • 2) Damiani JM, Levine HL. Relapsing polytonicities. Report of ten cases. Laryngoscope. 1979;89: 929-46.
    • 3) Michet CJ, McKenna CH, Luthra HS, O’Fallon WM. Relapsing polytonicities: survival and predictive role of early disease manifestations. Ann Intern Med. 1986;104:74-78.
    • 1. Clinical examination showing characteristic diffuse or localized swelling or masses in single or multiple organs
    • 2. Hematologic examination shows elevated serum IgG4 concentrations (≥135 mg/dL)
    • 3. Histopathologic examination shows:
    • ESR <20 mm/hr
    • Radiographic femoral or acetabular osteophytes
    • Radiographic joint space narrowing (superior, axial, or medial)
    • Hard tissue enlargement of two or more of 10 selected joints
    • Hard tissue enlargement of two or more DIP joints
    • Fewer than three swollen MCP joints
    • Deformity of at least one of 10 selected joints*
    • 1. Widespread pain index (WPI) 7 and symptom severity (SS) scale score 5 or WPI 3–6 and SS scale score 9
    • 2. Symptoms have been present at a similar level for at least 3 months.
    • 3. The patient does not have a disorder that would otherwise explain the pain.
    • Shoulder girdle, left, Shoulder girdle, right
    • Upper arm, left, Upper arm, right
    • Lower arm, left, Lower arm, right
    • Hip (buttock, trochanter), left, Hip (buttock, trochanter), right
    • Upper leg, left, Upper leg, right
    • Lower leg, left, Lower leg, right
    • Jaw, left, Jaw, right
    • Chest, Abdomen
    • Upper back, Lower back, Neck
    • Fatigue
    • Waking unrefreshed
    • Cognitive symptoms
    • For the each of the 3 symptoms above, indicate the level of severity over the past week using the following scale:
    • 0 = no problem
    • 1 = slight or mild problems, generally mild or intermittent
    • 2 = moderate, considerable problems, often present or at a moderate level
    • 3 = severe: pervasive, continuous, life-disturbing problems
    • Considering somatic symptoms in general, indicate whether the patient has:*
    • 0 = no symptoms
    • 1 = few symptoms
    • 2 = a moderate number of symptoms
    • 3 = a great deal of symptoms
    • The SS scale score is the sum of the severity of the three symptoms (fatigue, waking unrefreshed, cognitive symptoms)
    • plus the extent (severity) of somatic symptoms in general. The final score is between 0 and 12.
    • (1) Widespread pain index (WPI) ³ 7 and symptom severity scale (SSS) score ³5 OR WPI of 4–6 and SSS score ³ 9.
    • (2) Generalized pain, defined as pain in at least 4 of 5 regions, must be present. Jaw, chest, and abdominal pain are not included in generalized pain definition.
    • (3) Symptoms have been generally present for at least 3 months.
    • 4) A diagnosis of fibromyalgia is valid irrespective of other diagnoses. A diagnosis of fibromyalgia does not exclude the presence of other clinically important illnesses.
    • a) Fatigue
    • b) Waking unrefreshed
    • c) Cognitive symptoms
    • Beighton score of ≥41
    • Arthralgia for longer than 3 months in four or more months
    • Beighton score1 of 1, 2, or 3
    • Arthralgia (>3-month duration) in one to three joints or back pain (>3-month duration) or spondylosis, spondylolysis, or spondylolisthesis
    • Dislocation or subluxation in more than one joint or in one joint on more than one occasion
    • Three or more soft tissue lesions (e.g., epicondylitis, tenosynovitis, bursitis)
    • Marfanoid habitus: tall, slim, span greater than height (>1.03 ratio), upper segment less than lower segment (<0.89 ratio), arachnodactyly
    • Skin striae, hyperextensibility, thin skin, or abnormal scarring
    • Ocular signs: drooping eyelids, myopia, antimongoloid slant
    • Varicose veins, hernia, or uterine or rectal prolapse
    • Mitral valve prolapse
    • Two major criteria
    • One major plus two minor criteria
    • Four minor criteria
    • Two minor criteria and unequivocally affected first-degree relative in family history
    • Sensory: Reports of hyperesthesia or allodynia
    • Vasomotor: Reports of temperature asymmetry, skin colour changes, or skin colour asymmetry
    • Sudomotor or edema: Reports of edema, sweating changes, or sweating asymmetry
    • Motor or trophic: Reports of decreased range of motion, motor dysfunction (weakness, tremor, dystonia), or trophic changes (hair, nail, skin)
    • Sensory: Evidence of hyperalgesia (to pinprick), allodynia (to light touch, temperature sensation, deep somatic pressure, or joint movement)
    • Vasomotor: Evidence of temperature asymmetry (>1°C), skin colour changes, or asymmetry
    • Sudomotor or edema: Evidence of edema, sweating changes, or sweating asymmetry
    • Motor or trophic: Evidence of decreased range of motion, motor dysfunction (weakness, tremor, dystonia), or trophic changes (hair, nail, skin)
    • 1. The presence of flowing calcification and ossification along the anterolateral aspect of at least four contiguous vertebral bodies with or without associated localized pointed excrescences at the intervening vertebral body–intervertebral disk junctions
    • 2. The presence of relative preservation of intervertebral disk height in the involved vertebral segment and the absence of extensive radiographic changes of “degenerative” disk disease, including vacuum phenomena and vertebral body marginal sclerosis
    • 3. The absence of apophyseal joint bony ankylosis and sacroiliac joint erosion, sclerosis, or intraarticular osseous fusion
    • 1. Continuous ossification along the anterolateral aspect of at least four contiguous vertebral bodies, primarily in the thoracolumbar spine. Ossification begins as a fine, ribbon-like wave of bone but commonly develops into a broad, bumpy, buttress-like band of bone.
    • 2. Continuous ossification along the anterolateral aspect of at least two contiguous vertebral bodies
    • 3. Symmetric and peripheral enthesopathy involving the posterior heel, superior patella, or olecranon, with the entheseal new bone having a well-defined cortical margin.