Myocarditis as a Lupus Challenge: Two Case Reports

Shamma Ahmad Al-Nokhatha; Hiba Ibrahim Khogali; Maryam Abdulla Al Shehhi; Imad Tarik Jassim

Disclosures

J Med Case Reports. 2019;13(343) 

In This Article

Abstract and Introduction

Abstract

Background: Myocarditis is an uncommon manifestation of systemic lupus erythematosus in which the clinical presentation can range from subclinical to life-threatening. We report cases of two patients who presented to our hospital with myocarditis as an initial manifestation of systemic lupus erythematosus despite negative results of extensive workup that excluded other diagnoses. The mainstays of treatment are corticosteroids, immunosuppressive agents, and anti-heart failure medications, with use of the latter being case-specific. Mycophenolate mofetil was the cornerstone of the proposed treatment for induction of remission, although it is well known to be used as a maintenance therapy in lupus myocarditis.

Case presentation: Both Emirati patients described satisfied the diagnostic criteria for mixed connective tissue disease (systemic lupus predominant) and systemic lupus erythematous. Other differential diagnoses of myocarditis were excluded. The patients were started on pulsed steroid followed by oral steroid, with hydroxychloroquine, mycophenolate mofetil, and anti-heart failure medications used as needed. Dramatic responses were noted in the first few weeks in terms of symptoms.

Conclusion: Early recognition and treatment of lupus myocarditis is needed to avoid fatal consequences.

Introduction

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with cardiac involvement in up to 50% of cases.[1] It can manifest as pericardial disease, myocardial dysfunction, valvular heart disease, conduction system abnormalities, or atheromatous disease. Myocarditis is one form of cardiac involvement in SLE; it is reported to occur in 5–10% of symptomatic patients, whereas around 50% have a subclinical form proved by autopsy. The clinical presentation of myocarditis in SLE ranges from asymptomatic patients with self-limited disease to fulminant heart failure that can lead to death. The management of lupus myocarditis is challenging, and few studies have described the optimal treatment options.[2–4] In the present report, to shed light on this condition and the importance of mycophenolate mofetil (MMF) in therapy induction, we describe two patients with such a presentation. MMF was the cornerstone of the proposed treatment for induction of remission, although it is well known to be used as a maintenance therapy in lupus myocarditis.

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