key: cord-0989654-d6cd3e68
authors: Cohen, Raphaël; Nhan, Pascal; Cholet, Clément; Jachiet, Vincent; Ederhy, Stéphane; Mékinian, Arsène; Boccara, Franck; Fain, Olivier; Cohen, Ariel
title: Acute Myocarditis Revealing Adult-Onset Still’s Disease
date: 2021-05-05
journal: JACC Case Rep
DOI: 10.1016/j.jaccas.2021.03.008
sha: 1bbdcfa8e2829afdee70e9ec4e0da21413a76298
doc_id: 989654
cord_uid: d6cd3e68

A 34-year-old man presented with fever, palpitations, maculopapular rash, pharyngitis, left cheilitis, and bilateral gonalgia. High-sensitivity troponin I concentration was 4,900 ng/l. Transthoracic echocardiogram revealed reduced global longitudinal strain. Cardiac magnetic resonance imaging showed acute myocarditis. Adult-onset Still’s disease was diagnosed, and treatment with intravenous corticosteroids and tocilizumab was initiated. (Level of Difficulty: Beginner.)

associated with palpitations, maculopapular rash (Supplemental Figure 1) , pharyngitis, and bilateral gonalgia without synovitis. The patient reported no chest pain. At admission, his blood pressure was 117/ 74 mm Hg, heart rate was 94 beats/min, and temperature was 38.3 C. Clinical examination revealed a fixed, pseudourticarial, nonpruritic erythematous rash of the extremities.

Differential diagnosis included acute coronary syndromes, Takotsubo syndrome, parvovirus B19 infection, COVID-19 infection, and Kawasaki disease.

The patient had no documented allergies, cardiovascular risk factors, or signs of recent acute infection.

He reported recurrent sinusitis in recent years. He had not traveled abroad.

The pharyngeal streptococcal test result was negative. Cardiac auscultation revealed systolic murmur

To consider systemic disease as a possible cause during a myocarditis workup. To consider using a biologic immunomodulator as the first-line treatment in myocarditis with an inflammatory disease. (Figure 1 ).

High-sensitivity troponin I concentration was raised (4,900 ng/l). The patient was transferred to intensive care. 

The patient was diagnosed with AOSD because of the coexistence of myocarditis, fever, atypical cutaneous involvement, gonalgia, pharyngitis, biological inflammatory syndrome, and elevated ferritin level with a decreased glycosylated fraction. with an efficacy of 50% to 60% (7). Second-line therapies include methotrexate and biotherapies (interleukin-1 and -6 antagonists). Acute endocarditis with valvular vegetations due to fibrinoid or fibrinocruoric components has been reported, particularly involving the tricuspid valve (8, 9) .

A rare complication in AOSD is group 1 pulmonary arterial hypertension (9) 

A comprehensive review on adult onset Still's disease

Diagnostic and management of life-threatening adult-onset Still disease: a French nationwide multicenter study and systematic literature review

Macrophage activation syndrome in patients affected by adultonset still disease: analysis of survival rates and predictive factors in the Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale Cohort

Adult Still's disease: manifestations, disease course, and outcome in 62 patients

Myocarditis in adult-onset Still's disease: case-based review

Cardiac involvement in adult-onset Still's disease: Manifestations, treatments and outcomes in a retrospective study of 28 patients

Myocarditis in adult-onset still disease

Cardiovascular magnetic resonance in rheumatology: current status and recommendations for use

Pulmonary arterial hypertension in adult-onset Still's disease: a case series and systematic review of the literature

KEY WORDS adult-onset Still's disease, myocarditis APPENDIX For a supplemental figure, please see the online version of this paper

FIGURE 4 Cardiac Manifestations of Adult-Onset Still's Disease IFN ¼ interferon