Dr Shefali Sharma, Rheumatology, PGIMER, Chandigarh

Systemic Lupus Erythematosus Presenting as Coronary Artery Disease

Aadhaar Dhooria, Senior Resident, D.M. Clinical Immunology and Rheumatology, PGIMER, Chandigarh

Mr. A, a 16-year-old boy, presented with complaints of exertional dyspnea and angina for the past 1.5 years that had increased over the past 3 months. He also had a history of Raynaud phenomenon with digital ulcers, photosensitivity, oral ulcers, and arthralgias. Physical examination was notable for tachycardia and a blood pressure of 90/60 mmHg. No other features suggestive of connective tissue disease or vasculitis were seen. Investigations revealed ANA 3+ speckled, dsDNA +ve (152.4 IU/mL), and low complements (C3: 36.8 mg/dL; C4: <8 mg/dL). Electrocardiogram revealed QS complexes in lead III, ST elevation in V3 and V4, and poor progression of R wave. Two-dimensional echocardiography revealed left ventricular systolic dysfunction with an ejection fraction of 40% and regional wall motion abnormality in the left anterior descending (LAD) territory. Computed tomography (CT) coronary angiography did not reveal any abnormality (Figure 1).

The MASsive Emergency in Rheumatology – Macrophage Activation Syndrome in Inflammatory Rheumatic Diseases

Macrophage activation syndrome (MAS), a secondary form of hemophagocytic lymphohistiocytosis (HLH), is characterized by activation of macrophages and T cells resulting in non-remitting fever, rash, cytopenia, transaminitis, coagulopathy, hepatosplenomegaly, neurological and other organ dysfunction. Whether MAS and secondary HLH are interchangeable is debatable, with some authors considering them to be heterogeneous [1]. The pathogenesis and difference in the incidence rate of inflammatory rheumatic diseases (IRDs) are still not clearly understood. Most of our understanding of IRDs comes from systemic-onset juvenile idiopathic arthritis (sJIA). It can be a presenting feature of a rheumatic disease or can be triggered by flare of the underlying disease and tends to occur in the early phase of IRDs but can occur as late as a decade. The prevalence of IRDs has been described in 7%–40% cases of sJIA [2] and 1%–3% cases of systemic lupus erythematosus (SLE). Other AIRDs that have been associated with MAS include Kawasaki disease, polyarticular JIA, AOSD, very rarely in Sjogren syndrome, MCTD, dermatomyositis, systemic sclerosis, rheumatoid arthritis, sarcoidosis, and ankylosing spondylitis. Sex ratio in MAS complicating sJIA was unfavorable to women with a ratio of 3:2 contrary to 1:1 seen in sJIA. Rajgopala et al. reported 156 published cases of HLH in 2012 in their systematic review from India. Rheumatic disease was the underlying trigger in 9.5% of the 63 adult patients in the review [4].