The Indian Rheumatology Association

The Professional Organization of Rheumatologists and other Health Professionals in India

Patient Education

Disease Specific Information

Ankylosing SpondylitisX

Who is more prone to get AS?
AS is usually diagnosed in young adults, with a peak age of onset of 20 and 30 years and it is three times more, in males than in females. Studies have shown that individual's risk of developing AS increased 5 to 16 fold if there was a first degree relative (parent, sibling, or child ) with AS. The presence of a gene called HLA-B27 could explain the risk of developing AS.

What causes it?
Though the origin of AS is still unknown, we do know a lot about how it progresses and why it causes spinal stiffening. It is now thought that it involves the body's immune system and that a triggering agent causes the disease and only in people with a genetic or inherited susceptibility.

What happens in AS?
The disease initially begins in the bone at the periphery of the joint and at the site where the ligament is attached to the bone, called as enthesis. The inflammatory cells at the periphery of the bone progressively increase resulting in the weakening of the bone and the body resist the weakening by attempting continuous new bone formation, As the disease process continues the bone may become weaker and weaker. When the inflammation finally burn out the body resond with excessive bone formation in an attempt to repair the damaged bone and in this process this calcification spreads to the ligamentous and even the discs. This finally results in fusion of the adjacent bone also called as bony ankylosis, which is the characteristic feature of the disease as the name suggests.

Symptoms
The most common symptom of AS is chronic low back pain and buttock pains. This back pain has the following characteristic: Begins in early adulthood (age 20 to 30); Gradual onset (rather than sudden onset after an acute injury); lasts longer than three months; worse after rest(for example in the morning); improved with activity; Wakes patients up in the second half of the night ; can cause morning stiffness lasting more than 30 minutes and can cause buttock pain that alternates between the left and right side. In the long term the flexibility of the back mat be reduced due to fusion. Arthritis in other joints may also occur. Enthesitis also causes pain in the elbow, heel and rib etc . Chronic inflammation may cause significant constitutional symptoms in the form low grade fever, fatigue and sometimes weight loss.

AS can affect eyes in 25% of patients. The symptoms of iritis include sudden onset of pain and redness, blurred vision.

Diagnosis
The diagnosis of AS is based upon a combination of a patient's symptoms, physical examination and imaging test. Patient with AS develop characteristic changes in the sacroiliac joints (the joint that connect the tailbone [sacrum] and large pelvic bone [llium]) and spine. These changes can be seen on x-ray images , although the changes take time to develop and may not be apparent until years after symptoms of AS begin. X-ray may be used to diagnose AS in someone whose symptoms, history and physical exam suggest AS. Magnetic resonance imaging (MRI) and computed tomography (CT scanning) are more sensitive than plain X-rays, and may be used if As is suspected but not clearly seen on X-ray .there is no blood test that definitively diagnose ankylosing spondylists, although testing for a gene. HLA-B27, can be helpful in selected groups of patients. Other blood tests, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may be used in some patients.

Management
Treatment depends on the characteristic and severity of the disease. the sheet anchor in the management is primarily therapeutic exercises, non steroid anti-inflammatory agents, and sometimes disease modifying anti-rheumatic drugs and in those not responding to the above modalities, anti tumor necrosis factor agents has shown promising results in various studies.

Exercise
The exercise consist of posture training, deep breathing, back extension and other stretching movements. The details demonstration of exercises for AS patients is available on the following websites: www.nass.co.uk/exercise.htm safety issues. To avoid developing deformities of the neck, a thin, rather than a thick pillow and prone sleeping may be recommended.

Medications
Nonsteroidal Anti-Inflammatory Drugs(NSAID) are the mainstay of treatment-NSAIDs need to be taken on regular basis and studies have shown that in the long term usage it is known to modify the course of disease also. The therapeutic efficacy of the NSAIDs can be better judged at the recommended dosages and needs at least 2 weeks of regular intake and subsequent long term intake of these medications. The most common side effect of NSAIDs is stomach upset. patient with a current or past history of stomach or small bowel (duodenal) ulcers, bleeding problems, or are on blood thinners (anticoagulants) may require antiulcer agents or other medications such as celecoxib.

Sulfasalazine is a disease – modifying antirheumatic drug, or DMARD, that may be given to slow or stop the progression of AS. This drug provides some relief of arthritis symptoms but has little effect on spinal pain and stiffness. Sulfasalazine can be discontinued if it is not effective after six months of treatment. Side effects of rash, low blood counts can also occur. Patients talking this medicine may need to have blood tests done periodically to monitor blood counts and liver function. Rarely other DMARs like methotrexate may be helpful.

In those patients not responding to NSAIDs and sulfasalazine, theraphy with Anti-tumor necrosis factor agents like infliximab, Etanercept, adalimumab may be considered after careful evaluation by a rheumatologist. These drugs proven to be very efficacious and improvement occurs within a few days to a few weeks of starting the drugs. However, these drugs are probably not very effective in stopping the progression of the disease

Steroids oral steroids is rarely necessary in the treatment of AS and in fact long term use of it may cause increase in bone loss and risk of developing early osteoporosis (weak bones). However steroid ( glucocorticoid injection into the sacroiliac joint and other joints may provide relief in patients who has not therapies.

AS patients are more prone to osteoporosis, hence regular consumption of more calcium and vitaminD can reduce the risk of bone loss. There are other medications that risk of bone loss, such as aledronate, risedronate, zolendronic acid, which can be used in appropriate situations.

Surgery
Total hip replacement, insertion of an artificial hip may be recommended in patients with AS who have severe, persistent hip pain or severely limited mobility due to hip joint arthritis. Spinal surgery-Fusion of the bones in the cervical spine may be recommended for a small number of patients who develop dislocation of these bones and to prevent spinal cord damage. Spinal corrective surgeries like Wedge osteotomy may be required in some patients with severe spinal deformities.

Preventing Complications
As the bones are weak in these patients risk of serious vertebral fractures from slips and falls potentially exists and utmost care to be taken to avoid such mishaps. Sedatives, narcotics and alcohol should be avoided as these agents increases the risk of fall and serious spinal injury. Contact sports and other high impact activities should be avoided. Smoking not only make the disease worse but also places lungs in a mechanically disadvantageous position in already deformed spinal and rib disease due to AS. Hence smoking is absolutely contraindicated in this disease.

Mission
Our efforts are driven by a passionate belief that we can significantly reduce the suffering of Ankylosing Spondylitis. For those yet undiagnosed, it can be reduced by raising awareness & cutting down on the diagnostic delay. For AS warriors, it can be reduced by empowering them to take informed decisions to live a healthy and fulfilling life. We would like to grow as a platform for raising awareness & for mutually beneficial patient- rheumatologist interaction.

Objectives -

Raise awareness about Ankylosing Spondylitis (AS) among general public.

Raise awareness about Ankylosing Spondylitis among Family physicians & other specialties.

Provide the correct information & help AS warriors in taking the right decisions.

Create a platform for healthy interaction between Rheumatologists, AS warriors & allied healthcare professionals for finding solutions & research.

Forming local support groups for Ankylosing Spondylitis (AS) warriors

Work with Rheumatologists, IRA & government bodies towards insurance inclusion & reducing costs of biologics.

Systemic Lupus ErythematosusX

What is SLE (Lupus)?
SLE [Systemic lupus erythematosus] is an autoimmune disease involving multiple body organs, usually affecting women of childbearing age. Our immune system normally protects us against infections, but in illnesses such as lupus it attacks the body's own tissues. The symptoms depend on the organ affected like joints, skin, kidneys, brain etc.

What happens in lupus?
Tirendness, joint pains and skin rashes are common. You may have a rash over parts of the body that are exposed to the sun. A butterfly-shaped rash over the cheeks and the bridge of the nose, mouth ulcers, hair loss and joint pains are common. You may also develop problems in other internal organs like kideneys(protein in urine, high blood pressure etc.), the brain(abnormal behavior, depression, fits, stroke), heart, lungs, blood ( anemia, low platelets, abnormal bleeding or clotting ), and other systems.

Who suffers from Lupus?
Lupus is about nine times as common in women as in men.

Lupus is more common in younger women. Lupus also affects children but only rarely. We don't know exactly why this happens.

Why does Lupus occur?
Our immune system is the army protecting us from foreign foes. In Lupus, the protecting army gets abnormal and fails to recognize its own tissues and attacks them. This is probably caused by a combination of environmental, hormonal and genetic factors.

Lupus isn't contagious and is not directly inherited from your parents, though some of the genes inherited from parents may contribute to the development of the disease.

Are there any long-term risks in Lupus?
Lupus is a variable and unpredictable condition and can be life threatening for a minority of patients whose vital organs like kidneys, brain, heart, blood etc. are affected. However, with modern treatments and careful monitoring of the condition the disease can be brought under in most patients.

Anything more that I should know?
Keep out of the sun: Use a sun-blocking cream, SPF 25 a greater.

Contraception: You should use contraception pills that contain only progesterone or low-dose estrogen, or consider phyiscal/barrier mehods of contraception. Your treating doctor might help.

Infections: If you have Lupus, and especially if you are on immunosuppressive drugs, then you will more prone to infection. Take sensible precautions and avoid contact with family and friends who are known to have infectious diseases like tuberculosis, chickenpox etc.

Vaccinations: Check with your doctor if you might need vaccinations.

Stress: Stress will make your illness seem worse even if it dosen't affect the course of the disease. Learn how to manage any in your life.

Fatigue: Tirendness can be a significant problem. Specific causes of fatigue such as anemia or an underactive thyroid gland can be identified by a blood test and treated.

How is Lupus diagnosed?
A diagnosis of Lupus is made based on symptoms, a physical examination, and the results of blood tests like anti- nuclear antibody (ANA) test, Anti-DNA antibody test etc.

How is Lupus managed?
Lupus cannot be cured but it can be controlled. A number of different drugs may be needed depending on which symptoms you experience.

What are the various drugs to treat Lupus?
Skin rashes can be treated with creams containing steroids and/or with hydroxychloroquine tablets. They are also effective at treating most other problems in Lupus. Other drugs like steroids, azathioprine, cyclophosphamide, mycophenolate, cyclosporine etc are often used by doctors to treat Lupus. The drugs and modality of treatment depends on the organ involved. You might also require other drugs like blood pressure lowering drugs, calcium, etc. These are taken care of by your doctor/rheumatologist and you are advised to follow their advice.It is extremely dangerous to take or to stop these drugs on your own.Further, you require regular follow up with your doctor. Please keep your doctor informed about any problems while taking them or in case you develop new illness or contemplate or become pregnant.

Day to day activities
Exercise: Exercise as advised by your doctor can improve fitness without putting too much strain on the joints.

Diet and nutrition: There is only limited evidence available on the effect of diet in controlling Lupus. However, people with Lupus have an increased risk of having a heart attack or stroke. Therefore, it is important to take less fat and salt in your dietand stop smoking.

Pregnancy and Lupus
If you thinking of having a baby, discuss your plans with doctor before conception. There is conflicting evidence whether pregnancy is likely to cause a flare -up of Lupus. If the disease is well under control at the time baby is conceived, and you do not have kidney disease, you will number probably go through pregnancy with little trouble. A small number of women with very severe Lupus may be advised against having a baby as pregnancy can put a great strain on the heart, lungs and kidneys.The doctor looking after your preganancy check for this and consult your rheumatologist if necessary.

If you have high levels of antiphospholipid antibodies, then there is an increased risk of miscarriage.There is a small risk that babies born to Lupus mothers may have neonatal (newborn) Lupus syndrome.This means the baby may have a rash and / or a slow heartbeat. Most babies born to mothers with Lupus will be fine.Doctors are naturally cautious about which drugs are used during pregnancy. However, if required, some drugs like hydroxychloroquine, steroids and azathioprine are continued during pregnancy.

FibromylgiaX

What is fibromyalgia?
Fibromyalgia (FM) is a disease or sympton complex characterised by widespread pain and profound fatigue.

What causes FM?
The exact cause of FM is not known. It is likely to be multifactorial. Genetic predisposition with disease running families is well recognised. Environmental insults including viral infections, stress (both emotional and physical) and depression in some cases can contribute to the onset of illness. FM can coexist with rheumatic disease like rheumatic arthritis, systemic lupus erythematosus and Sjogren's syndrome.

How common is FM?
It is very common. Upto 3% of the population suffers from it. It is most common around the 4th decade. FM is more common in women but it can occur in men and children too.

How is FM diagnosed?
There is no single diagnostic test for fibromyalgia unlike blood sugar test for the diagnosis of diabetes. The diagnosis is base on the combinations of symptoms, physical examination. Widespread aches and pains affecting both sides of the body are the hallmarks of FM. Neck pain and back pain are the common symptoms. Extreme fatigue, early morning stiffness, non-refreshing and non-restorative sleep, subjective feeling of swelling of limbs and joints, numbness of peripheries are also common. Migraine like headaches, abdominal symptoms like bloating, heart burn, tendency to visit toilet frequently, particularly after food ( irritable bowel syndrome ) and urgency to pass urine frequently (irritable bladder) are other associated symptoms. FM patients also have multiple tender points over the body, which the doctor can identify on examination. So it is the combination of symptoms and the presence of tender points that lead to the diagnosis of FM.

As mentioned earlier, there are no laboratory tests in the diagnosis of FM. Many patients are suspected to have other diseases and are investigated extensively before FM is diagnosed. Your doctor would have ruled our diseases like cancer and arthritis.

What does current research say?
FM is shown now to be a syndrome of low pain threshold and altered pain perception. The volume setting for pain perception is found to be higher in FM patients compared with the normal population. Because of this FM patients would feel more pain compared with non-FM persons for the same amount of stimulus. This process is called central sensitisation, high lighting that the origin of pain is form nervous system rather than in the places where pain in felt. Electrophysiology study of sleep has shown disturbance in sleep apnoea syndrome. Alteration in pain related neurotransmitters, Serotonin and substance P have been demonstraded in cerebrospinal fluid. Abnormalities in sympathetic in cerebrospinal fluid. Abnormalities in sympathetic nervous system, hypothalamo-pituitary adrenal axis, growth hormone secretion have been demonstrated in FM. This might indicate defective mechanisms to fight stress. Some association with depressive illness and other psychiatric illness diseases like chronic fatigue syndrome, non cardiac chest pain, non ulcer dyspepsia, chronic pelvic pain, depression, anxiety and fibromyalgia indicating shared aetiology.

How is FM treated?
Usually FM symptoms are mild and patients mange to lead a normal life in spite of the pain. In some cases, pain can be severe and is comparable with the pain of patients with Rheumatoid Arthritis.

There is no cure. But the symptoms can always be helped. Painkillers like paracetamol and NSAIDs like lbuprofen may be of some help. Unlike the pain of arthritis, the pain of FM does't respond to the above drugs well. It is a different type of pain requiring a different type of medicines. Antidepressant drugs used in doses much lower than what is needed to treat depression are useful is correcting sleep disturbance and in improving pain threshold. Amitriptyline is one such drug. Dothiepin, nortriptyline, Duloxentine, milnacipran are some of the anti depressant drugs found to be useful in managing the symptoms of FM. Anti convulsant drugs ( Anti fits drugs ) like pregablin and gabapentin also help the pain of fibromyalgia.

Cognitive behavioural therapy (CBT) will help patients to take control of his of her illness. This is usually done by trained pain psychologists. Chronic pain leads to in changing the way one think about the illness and encougaes patients to lead a more productive life. Stretching five times a day ) are an important aspect of the therapy.

Walking swimming and cycling are some of the exercise strategies to improve one's stamina and physical conditioning. Pain and fatigue make one de conditioned. Graded increase in the intensity and the duration is essential to overcome post exercise increase in pain. Relaxation techniques like yoga, Tai Chi and low impact dancing and aerobics may help many.

Fibromyalgia is generally treated by a team of pain specialists including rheumatologists, pain consultants, physiotherapists and pain psychologists. More importantly, the patient is an active member of the treatment team.

How to cope up with Fibromyalgia?

1.knowing that FM, though a chronic painful disease is not a life threatening or a crippling should reassure you.

2.Learn to relax, pace your activities according to pain levels and energy levels.

3.Pain does not always mean tissue damage. So don't stop your activities totally.

4.Ignore some discomfort, count on functional improvement.

5.Be prepared for some bad days and some good days.

6.A brief period of rest in between work ( micro-rest) and an afternoon nap can be enterprising.

7.Hot water shower and heat in any form will refresh you. A shower in the evening may help you to fresh at dinner time.

8.Regular sleep habits will help. Avoid caffeine and cola drinks after 6 PM. Avoid watching movies from the bed. Condition your mind that bed for sleeping. Having the fan on can act as the white noise filter extraneous noises at night.

9.Be open with parents, and friends so that they will understand your illness and be more supportive.

10.By understanding the nature of the illness, you may be able to avoid unnecessary investigations and harmful therapy.

11.Be regular with your exercise and taking the prescribed medicine.

12.Discuss with your physician, your doubts and the concerns regarding the disease and the medications.

Scleroderma or systemic SclerosisX

What is Scleroderma ?
Scleroderma literally means " hard skin," which is a common finding to this group of diseases. It accurs due to the abnormal growth of connective tissue. There are broadly two categories of scleroderma: localized scleroderma ( affects skin and rarely muscles ) and systemic sclerosis ( affects skin and internal organs like heart, lung, intestines etc).

Localized scleroderma generally remains limited and does not progress to the systemic form. Localized scleroderma further can two types; morphea and linear.

Systemic sclerosis affects the skin, muscles, blood vessels and internal organs. It is often divided into diffuse and limited disease. Diffuse systemic sclerosis is a rare disease that can be severe and sometimes life-threatening.

What causes it ?
The exact cause of scleroderma is not known. However, it has been known that body makes too much of collagen ( substance that heals and gives us scars ) that gets deposited in the skin and other body organs, causing the skin to tighten and harden and the organs to dysfunction. Another component is abnormal function of cells in the lining of blood vessels, which leads to Raynaud's phenomenon (pallor, blue fingers or toes) and telangiectasias (red spots). These abnormalities are triggered by abnormal functioning of our own immune cells, some environmental agents of hormones that stimulates cells to produce excess collagen.

Symptoms

Localized Scleroderma
The symptoms of localized scleroderma are isolated ot the skin and underlying tissues. Two types are recognized: morphea and linear scleroderma.

Morphea : These are local patches of hardened skin. Red patches of skin develop white centers with purplish borders. Lesions remain active for weeks to several years. Spontaneous softening that leaves a darkened area of skin often occurs. It may be localized or generalized.

Linear: A single line or band of thickened and discolored skin develops. The line usually runs down and arm or leg, but sometimes it runs down the forehead.

Systemic Sclerosis
This disease affects not only the skin and underlying tissues, but also affects the blood vessels and major organs of the body. Two types of systemic disease are recognized: limited and diffuse.

Limited: In this form, sin thickening is generally limited to the fingers, forearms, legs, face and neck. Raynaud's phenomenon ( blue fingers ) may be present for years before any other symptoms develop. People with this form are less likely than people with diffuse disease to develop severe organ involvement.

Diffuse: In this form, skin thickening may occur anywhere on the body, including the trunk. Only a short interval of time will elapse between the onset of Raynaud's phenomenon and significant organ involvement. Damage typically occurs over the first three to five years, after which most patients enter a stable phase that varies in length. During this phase, your skin will stay about the same and the rate of damage to interval organs slows or stops. After the stable phase is over, your skin will start to soften and more serious damage to internal organs is unlikely to occur.

Depending on the form and severity of the disease, you may experience these different symptoms:

Raynaud's Phenomenon: Cold temperatures or emotional distress cause constriction of blood vessels in hands feet leading to cold extremities and whitish or bluish discoloration of fingers and toes.Once the vessels open back up ( after approximately 10 to 15 minutes), the hands turn red. More than 90% of people with scleroderma have this problem, it is often the first symptom of the disease. Skin ulcers on the fingertips can result from severe Raynaud's phenomenon.

Skin changes: In the early stage skin may appear edematous and it may progressively harden with loss of flexibility. As the disease progresses, the skin will become more thickened, sweat and oil glands will stop functioning, and the skin surface will become very dry and itchy. This stage will persist for one to three years, at which time the skin will begin to soften and thin.

Sclerodactyly: Thickening of the skin of the fingers or toes.

Calcinosis: Calcium deposits can form under the skin or in the muscles. These deposits may cause recurrent ulcers on the overlying skin.

Telangiectasias: Red spots due to dilated capillaries near the surface of the skin can be seen.

Arthralgias and myalgias: Pain and stiffness of the joints and muscles are common symptoms early in the course of systemic sclerosis. Later in the disease course, muscle atroply and weakness are common.

Dental disease: If the skin around your mouth tightens, you may not be able to open your mouth very wide.You may also have a dry mouth due to secondary Sjogren;s syndrome. Damage to connective tissues in the mouth can lead to loose teeth. These problems can lead to periodontal disease, tooth loss and difficulty chewing, which can compromise your nutritional status.

Esophageal dysmotility: difficulty in swallowing due to loss of normal movement in the food pipe. It may cause chronic heartburn

Gastrointestinal dysfunction: Dysmotility along the whole gastrointestinal tract can cause reflux, early satiety, bloating, nausea, vomiting, cramping abdominal pain, diarrhea, weight loss and malnutrition.

Lung disease: difficulty in breathing or sense of suffocation are the most common initial symptoms. Similar to skin thickening, lungs can become hard too and loose their elasticity leading to decreased oxygenation. Lung impairment is the leading cause of death in systemic sclerosis. Getting your lung function tested regularly is important for detecting lung disease early.

Kindney disease:

sudden rise in blood pressure leading to kidney failure is a dangerous complication. About 80 percent of cases of renal crisis occur within the first four or five years of disease, usually in people with diffuse disease.

Heart disease: Symptoms of heart disease are not seen until late in the course of systemic sclerosis. Symptoms include breathlessness on exertion, palpitations and, less frequently, chest pain.

Is it a contagious disease?
No.

How to confirm the diagnosis?
A diagnosis could take months as the disease unfolds and as the doctor is able to rule out other possible causes of your symptoms. Diagnosis is made clinically by the doctor familiar with the disease. There is no direct blood test that can confirm the diagnosis. Special tests may be ordered to evaluate your lung status ( such as a chest X-ray, pulmonary function tests) and gastrointestinal tract function.

What is the Treatment
No treatment till date has been proven effective in reducing the skin thickening. However, several treatments are very effective at preventing and/ or reducing organ damage from this disease. While treatments are not able to reverse the disease, they may actually prolong life. So treatment dicisions are made on a symptom-by-symptom, organ-by-organ basis.

Raynaud's phenomenon
Dont't smoke. Somoking narrows blood vessels, making Raynaud's worse.

Avoid the cold when possible.
Dress warmly, in layrs. Mitterns are warmer than gloves. Use drugs perscribed by your doctor to improve your circulation ( Calcium channel blockers, angiotensin receptor blockers, ACE inhibitors etc.) Skin sores and ulcers can be treated with nitroglycerin paste or antibiotic cream. Narcotic analgesics may be necessary to treat the pain of these ulcers.

Skin problems
Apply moisturizing creams and lotions frequently, especially after bathing. Apply sunscreen before going outdoors.

Use only warm water in your bath or shower ( hot water is too drying)

Avoid harsh soaps, household cleaners and caustic chemicals. Use rubber gloves if you cannot avoid harsh chemicals.

Itching can be treated with oral antihistamines, topical analgesics and topical corticosteroids

Stiff muscles and joints
Get regular exercise to maintain range of motion and muscle strength.

Use pain killers as prescribed by your doctor.

DO NOT mix up two or more NSAIDs on the same day.

DO NOT take pain killers empty stomach.

See a physiotherapist to stretch contracted joints.

Dental problems
Practice good oral hygience- brush and floss regularly. Use fluoride rinses or prescription toothpastes.

See your dentist regularly.

Keep your mouth moist by taking frequent sips of water, chewing sugarless gum or using saliva substitutes.

Perform facial exercises to help keep your mouth and face flexible.

Gastrointestinal problems
Eat small, frequent meals.

Remian standing or sitting at least one hour after eating.

Avoid late-night meals.

Chew food well.

Proton pump inhibitors, such as omeprazole and lansoprazole, can be taken for heartburn.

Promotility agents may help move food through the gastrointestinal tract.

Antibiotics may relieve diarrhea caused by bacterial overgrowth.

Vitamin supplementation may be needed.

Lung disease
Immunosuppressive drugs such as cyclophosphamide and azathioprine, along with low-dose steroids can be used to treat pulmonary fibrosis.

Vasodilators such as prostacyclin, Bosentan, sildenafil can be used to treat pulmonary hypertension.

Get regular check-ups with your doctor and have lung function tests performed often so that lung disease can be caught early and treated.

Get flu and pneumonia vaccines.

Kidney disease
Measure BP regularly and alert your doctor if it is high. Angiotensin- Converting Enzymes (ACE) inhibitors, including captopril, enalapril and ramipril can effectively manage acute renal crisis by lowering high blood pressure.

Angiotensin II receptor inhibitors may be used to control hypertension when your are not in renal crisis.

Kidney dialysis may be necessary for some patients, atleast temporarily.

Renal transplantation may be necessary if kidney are damaged permanently.

Heart disease
Treatments for heart disease vary according to the way the heart is being affected by systemic sclerosis.

Who is at risk of getting this disease ?
Anyone can get scleroderma at any age. Women are more commonly diagnosed with scleroderma than men. In fact women with the disease outnumber men with it by about four-to-one.

Localized scleroderma is more common in children and young women.

Morphea usually appears between the ages of 20 and 40.

Linear scleroderma usually occurs in children or teenagers.Systemic sclerosis, whether limited or diffuse, typically occurs in people 30 to 50 years of age.

Life expectancy
Localized scleroderma is not life-threatening. Systemic sclerosis patients are at risk of serious complications, kidney disease, Pulmorary Arterial Hypertension (PAH) (high blood pressure in the arteries around the lugs), alveolitis ( inflammation of the lungs), Gastro-intestinal ( GI ) disease and heart damage that can be fatal and adversely affect the quality of life.

Pregnancy
Any female diagnosed with scleroderma needs to check with her physician and obstetrician to discuss possible complications associated with having children. If scleroderma has stabilized, then the physicians are better able to determine if the body can handle pregnancy and childbirth. The genetic risk of transmission of disease to children is negligible.

Juvenile Idiopathic ArthritisX

What is juvenile idiopathic arthritis ( JIA )
JIA is the most common cause of chronic (lasts more than 6 week) arthritis in children. It is of different types depending on the clinical signs and symptoms. In JIA child has joint pain associated with swelling and along with this may have fever, skin rash, lymph node enlargement, backache, red eye, or pain in sole/heel.

Why did my chlid get it?
The exact reason why it occurs is not know. Genetic and environmental factors are thought to play a role but it is not a hereditary disease as only 5% of children have a family history.

What are its common symptoms?
It usually with joint pain associated with swelling, which may involve one to several joints. The child may have malaise, fever fatigue and stiffness in the morning. Some children have high grade fever, skin rash and gland enlargement along with joint pain.

How is it diagnosed?
There is no diagnostic test for JIA. The diagnosis is made by presence of symptoms and signs. Some simple tests are done to excule other diseases and to know the severity of disease.

How is it treated?
To provide immediate pain relief most children are treated with NSAIDs like Naproxen, Ibuprofen etc. In a child with a few joint affected injection of cortisone into the joint is an affective way of controlling disease. Since it is chronic disease a significant proportion of children require disease modifying drugs like Methotrexate. Other drugs used are Salazopyrin, Leflunomide and biologics. Along with medication the child needs balanced diet, splints to prevent contractures, physiotherapy and psychological support.

What dietary precautions should the child take?
No relationship between any special food and JIA has been shown. Potato, pulses etc do not increase joint pains. Balanced diet is must for maintenance of muscle mass, bone strength and adequate hemoglobin in blood. A child who is getting cortisone should avoid food rich in fat and salt.

How long would my child need to take medication?
In oligoarticular variety of JIA the child may need only short term treatment but in majority, long term treatment is required. Drugs are gradually reduced after the disease is controlled and can be stopped once the disease is in remission for 1 year. In some types of JIA treatment is given for long term.

Can the disease be cured?
About one third of children may outgrow the disease and may not have symptoms in adulthood. In the rest the disease follows a relapsing remitting course. In a small fraction the disease may be present throughout.

Can my child go to school?
All effort should be made to help the child attend school and indeed most children with JIA can attend school. Some children may need assistance during disease flares. A discussion with the class teacher often helps.

Will my child be able to do job?
If the disease is managed early and well, most children can study and do a profession. However some children have physical limitation and thus cannot do jobs that involve manual labor. It is good to have a session with your physician when you are making decision regarding career.

What is the long term outcome of this disease?
JIA is a disease with good outcome however 50% of children would have some joint pains during adulthood. Most children can attain good education level and are employable. Good outcome depends on early and effective treatment to control disease activity.

What are its complications?
In systemic variety ( So JIA ) child have fluid around the heart ( pericarditis ) around lungs (pleuritis), myocarditis, marked weight loss rarely a serious condition called macrophage activation syndrome. In other varieties limb length discrepancy, hip joint damage and joint deformities may occur. Uveitis ( inflammation in eye ) can present with redness of eyes, blurring of vision or gradual visual loss. Thus regular eye checkup is needed.

Complications can also be related to therapy like weight gain, short stature, acne related to cortisone. Methotrexate, sulphasalazing and leflunomide may rarely cause lowering of blood counts or liver enzyme abnormalities.

Does it have any effect on puberty?
In most children if the disease is well controlled there is no impact on pubertal growth. In children with SoJiA or polyarticular JIA puberty may be delayed due to persistent disease activity.

Rheumatoid ArthritisX