Unveiling the heart of lupus: A rare presentation of SLE as first episode myocarditis with catastrophic antiphospholipid syndrome.

Abstract

Background: Anti-phospholipid syndrome (APLS) is an auto-immune condition characterised by a predisposition to vascular thrombosis owing to the presence of anti-phospholipid antibodies (a-PL). Case presentation: We present a case of a 32-year-old male with a history of APLS without any regular anticoagulant use, presenting with fatigue and jaundice. Biochemical findings were in keeping with severe haemolytic anaemia. Admission electrocardiogram (ECG) demonstrated inferior ST segment elevation with reciprocal aVL, V2, V3 and V4 ST segment depression. Transthoracic echocardiogram (TTE) demonstrated inferolateral hypokinesis. No obstructive coronary artery disease was noted on coronary angiography with a subsequent cardiac MRI demonstrating features of myocarditis with associated microvascular occlusion (MVO). A diagnosis of probable catastrophic antiphospholipid syndrome (CAPS) was made in light of further thrombotic complications; a left popliteal vein thrombosis and diffuse alveolar haemorrhage (DAH) on chest imaging. The constellation of myocarditis and thrombotic events involving multiple vascular beds was ultimately unified by the diagnosis of systemic lupus erythromatous (SLE). Management of auto-immune haemolytic anaemia, myocarditis and probable CAPS included use of high dose systemic glucocorticoid therapy with adjunctive intravenous immunoglobulin and Rituximab. Angiotensin-Converting Enzyme Inhibitor (ACEi) and beta-blockade therapy was commenced in light of the lupus cardiomyopathy. Furthermore, the patient was commenced on warfarin and hydroxychloroquine in setting of SLE with secondary APLS. Conclusion: To our knowledge, this is the only documented case of myocarditis as the first manifestation of SLE with secondary catastrophic antiphospholipid syndrome and haemolytic anaemia.