P044 Life-threatening acute myocarditis as initial presentation of systemic lupus erythematosus

SLE is a chronic autoimmune inflammatory disease which can involve any organ in the body with features varying from mild arthralgia to severe life-threatening organ system inflammation. Cardiac involvement in SLE can be seen in up to 50% of cases and can manifest as valvular disease, pericarditis, myocardial dysfunction and coronary artery disease. Clinical presentation of myocarditis varies from mild tachycardia to fulminant congestive cardiac failure to cardiogenic shock with a mortality of 20%.

A 33-year-old South African woman attended the emergency department (ED) with night sweats, joint swellings, chest pain and dyspnoea. She had received a pneumococcal vaccination 5 weeks previously. She had a history of HIV infection in remission with daily anti-retroviral therapy. Rheumatology review demonstrated widespread joint swellings with severe restriction in movement and functional limitation. Pulse was 94/min, BP 126/75, CRP 145 with unremarkable systemic examination. She was prescribed Prednisolone 30mg daily for a provisional diagnosis of post vaccine reactive arthritis. Two weeks later she re-attended ED with worsening chest pain and dyspnoea on minimal exertion. She had tachycardia >130. CT pulmonary angiogram showed no PE but cardiomegaly, pericardial and pleural effusions. Transthoracic echocardiogram (TTE) revealed ejection fraction (EF) <35% and global 1.3cm pericardial effusion. Serial TTE demonstrated progressive deterioration in EF to < 20% with global hypokinesia despite vasopressor support. ANA was positive with strongly positive SM, Ro and RNP antibodies, DNA binding antibodies >300 (NR0 -10.1). Urine PCR 47 (0-20). C3 0.67 (0.9-1.8); C4 <0.08 (0.1-0.4). IgG 24 (7-16), IgA <0.10 (0.7-4), IgM 2.36 (0.4-2.3). She was pulsed with 1 g IV methylprednisolone x 3 and IV immunoglobulin x 1. She developed PEA cardiac arrest while awaiting IV cyclophosphamide. She was resuscitated and transferred immediately for consideration of ECMO, and the transplant team were informed. She improved on inotrope support without ECMO, 3 cycles of IV cyclophosphamide and input from multi-disciplinary teams including rheumatology, cardiology, virology, sexual health, respiratory and radiology. She was discharged home 7 weeks later. Repeat Echo 4 months on showed EF improved to 50% with normal LV filling pattern. She is currently in clinical and serological remission of SLE on reducing course of prednisolone, hydroxychloroquine 200 mg OD, MMF 1 g BD and cardiac supportive drugs including bisoprolol, eplerenone and sacubitril/valsartan.

NA

This case demonstrated the whole spectrum of cardiac involvement by SLE and rapid deterioration from tachycardia to life threatening cardiogenic shock. She fulfilled the classical risk factors: age, gender and race. Though reported as rare, there are higher rates of subclinical cases and post-mortem diagnosis of lupus myocarditis. Primary lupus myocarditis should be high in the differential diagnosis in the presence of risk factors and disproportionate tachycardia.

S. Zeb: None. H. Cooke: None. K. Yein: None. R. Waller: None.