Still's Disease and Myopericarditis

Abstract

Adult-onset Still’s disease (AOSD) has a vast array of clinical presentations. Myopericarditis is one of the rarest cardiopulmonary manifestations of the disease and due to its rarity, the literature on the association of myocarditis with AOSD is sparse. Herein, we describe an interesting case of a 44-year-old male who presented with chest pain following exertion. He was febrile at the time of presentation and exam revealed inflammation in various joints. Electrocardiogram showed diffuse ST segment elevations in the precordial leads. Laboratory results revealed elevated troponin of 3.17 (<0.05 ng/mL) and CK-MB of 6 ng/mL along with elevated ferritin of 6225 (16-336 ng/mL). Cardiac MRI showed early and late gadolinium enhancement consistent with myocarditis. The patient was started on steroids and non-steroidal anti-inflammatory drugs (NSAID) resulting in clinical improvement. This case highlights the critical importance of diagnosis of pericarditis and myocarditis in patients with AOSD, as a missed diagnosis can lead to significant morbidity and mortality.

A rare presentation of acute myocarditis as a manifestation of adult-onset Still’s disease: a case report

“… 2 , 10 Furthermore, myocardial involvement tends to confer a worse prognosis, and is associated with higher rates of intensive care admission. 1 , 3 , 10 , 11 Pro-inflammatory cytokines IL-1, IL-6, interferon-gamma (IFN-γ), and TNF-α are involved in the pathogenesis of AOSD, and thus remain potential targets for future therapies. 6 , 7 , 12 …”

“… 1–3 , 8–11 Myocarditis is a rare clinical manifestation of AOSD, with a prevalence of roughly 7% in these patients. 1 , 2 , 11 This presents both diagnostic and therapeutic challenges. As AOSD typically affects younger people, it should be considered as a differential when managing patients with myocarditis.…”

A Rare Case of Adult-onset Still's Disease Revealed by Acute Myopericarditis

“…Treatment of acute myocarditis in AOSD is not different from acute myocarditis basic treatment in the general population, including symptomatic treatment such as loop diureticsin case of congestive signs, and heart failure treatment with betablockers and/or angiotensin-converting enzyme (ACE) inhibitors or Angiotensin receptor blockers (ARBs) in case of LV systolic dysfunction. In addition to etiological treatment with corticosteroids as first-line treatment in patients with myocarditis, since they proved their effectiveness in this indication [12]. Their initiation should not be delayed if there is a high probability for the disease.…”

Cardiac Magnetic Resonance in Rheumatology to Detect Cardiac Involvement Since Early and Pre-clinical Stages of the Autoimmune Diseases: A Narrative Review

“…It is not surprising that the presence of myocarditis, which is often symptomatic in 96% of cases, with electrocardiogram abnormalities mentioned above and LV dysfunction with reduced LVEF <50% in 67% of cases ( 185 ); can manifest with arrhythmias and ventricular dysfunction as it has been described, based on the cytokine storm that is part of this disease ( 180 , 184 , 186 ). CMR has good diagnostic accuracy in this disease ( 182 , 187 , 188 ) and can demonstrate the presence of myocarditis found with the endomyocardial biopsy ( 185 ). It is crucial to identify the presence and severity of cardiac involvement, mainly in myocarditis cases.…”