A rare case of cardiogenic shock due to lupus myocarditis in a patient with HIV

ABSTRACT

INTRODUCTION: Lupus Myocarditis is a rare and severe manifestation of systemic lupus erythematosus. We describe a patient with Human Immunodeficiency Virus (HIV) presenting with cardiogenic shock due to lupus myocarditis. DESCRIPTION A 33 year old man with history of congenital HIV infection on anti-retroviral therapy, CD4 count 338/ mm3 and undetectable viral load, recurrent Pneumocystis jirovecii pneumonia, disseminated zoster and chronic kidney disease stage 3 presented with shortness of breath for 2 weeks and hypotension with cold extremities and leg edema. Transthoracic echocardiogram demonstrated acute severe biventricular dysfunction with ejection fraction of 10%. CXR showed ground glass opacities with bibasilar consolidation. He was subsequently intubated for acute hypoxic respiratory failure and admitted to the cardiac intensive care unit for management of cardiogenic shock mixed with sepsis due to presumed multifocal pneumonia. He was treated with high dose vasopressors, inotropes and empiric antibiotics. Infectious work up revealed methicillin-resistant Staphylococcus aureus (MRSA) in respiratory culture and negative viral infection including SARS-CoV-2. His course was complicated by worsening renal function with proteinuria and refractory metabolic acidosis required continuous venovenous hemofiltration and he suffered pulseless electrical activity (PEA) arrest with return of spontaneous circulation in 5 minutes. Coronary angiogram was normal. Auto-immune work up revealed elevated serologies anti-Ds DNA >300 IU/ ml, Anti-Smith Ab 1 (0-0.9 AI), Anti-chromatin Ab >8 (0 to 0.9 AI) with markedly low complement levels. Endomyocardial biopsy revealed lymphocytic infiltrate in endocardium and myocardium with no granulomas or thrombi. Based on these findings, he was diagnosed with lupus myocarditis and lupus nephritis. The patient clinically improved after treatment with pulse dose steroids and cyclophosphamide. His renal function recovered and cardiac function improved. He was weaned off from the ventilator and discharged to rehabilitation facility. DISCUSSION: Lupus Myocarditis requires urgent clinical attention as it may progress to heart failure and fatal cardiogenic shock. Early diagnosis with high index of suspicion and treatment with steroids and immunotherapy are the keys for better outcome.