Acute myocarditis revealing autoimmune and inflammatory disorders: Clinical presentation and outcome

https://doi.org/10.1016/j.ijcard.2021.12.058Get rights and content

Highlights

  • Acute myocarditis (AM) may reveal autoimmune and inflammatory disorders (AIID).

  • Myocarditis revealing AIID may be life-threatening at the acute phase.

  • AIID-associated myocarditis has a good prognosis under specific treatment.

  • Pericardial effusion and high CRP level suggest an AIID as the cause for AM.

Abstract

Background

Acute myocarditis (AM) may be the heralding manifestation of autoimmune and inflammatory disorders (AIID). We aimed to describe the clinical presentation and outcome of patients with AM revealing AIID.

Methods

All consecutive adult patients with AM admitted in a department of Cardiology (Bichat Hospital, Paris, France) from January 2011 to January 2019 were included. Diagnosis of AM was based on clinical manifestations, elevated Troponin, myocardial inflammation on CMR and no evidence for coronary artery disease. AIID were classified using international criteria.

Results

Two-hundred and eighteen (35.3 [26.4–47.1] years, 75.2% males) patients with AM were included. Overall, AM revealed AIID in 15 (6.9%), including systemic lupus erythematosus (n = 3), adult onset Still's disease (n = 3), sarcoidosis (n = 2), mixed connective tissue disease (n = 1), anti-Jo1 syndrome (n = 1), eosinophilic granulomatosis with polyangiitis (n = 1), antiphospholipid syndrome (n = 1), reactive arthritis (n = 1), Graves' disease (n = 1) and Crohn's colitis (n = 1). Left ventricular ejection fraction (LVEF) at onset was <30% in 5 (33.3%) patients with AIID. All but 2 patients with AIID were treated with steroids, immunosuppressive and/or immunomodulatory drugs and LVEF normalized in all by the end of follow-up. By comparing patients with AIID to patients with idiopathic AM (n = 203), multivariable analysis showed that pericardial effusion, lack of chest pain and high CRP level at onset were independently associated with AIID.

Conclusion

Acute myocarditis revealing AIID may be life-threatening at the acute phase but has an overall good prognosis under specific treatment. Pericardial effusion and CRP level at admission suggest an AIID as the cause for AM.

Introduction

The cause for acute myocarditis (AM) often remains unknown. Several autoimmune and inflammatory disorders (AIID) - such as systemic lupus erythematosus (SLE), sarcoidosis, Behçet's disease or eosinophilic granulomatosis with polyangiitis (EGPA) - may affect all heart layers including myocardium [[1], [2], [3], [4], [5]]. Detection of myocardial involvement in AIID is warranted both because of the risk of death and the need for prompt immunosuppressive therapy in order to improve outcome. Importantly, AM may also be the first manifestation of an underlying AIID [2]. Published data on myocarditis in such setting are however scarce. We aimed to report on diagnosis and management of AM revealing an AIID.

Section snippets

Patients

All consecutive adult patients admitted from January 2011 to January 2019 for AM in the Department of Cardiology of Bichat Hospital, Paris, France were included. Consistent with the 2013 ESC statement on myocardial and pericardial diseases [6], AM was defined by the recent onset (up to 3 months) of clinical manifestations including acute chest pain and/or heart failure signs and/or arrhythmia and/or syncope and/or aborted sudden cardiac death and/or cardiogenic shock AND elevated Troponin AND

Characteristics of patients

From January 2011 to January 2019, 451 patients hospitalized in the Department of Cardiology (Bichat Hospital, Paris, France) were eligible for the study according to ICD-10 identification. After careful review of individual medical records, the diagnosis of AM was not confirmed in 216 patients. Among the remaining 235 patients, myocarditis was associated with an already known AIID (n = 8), an infectious disease (n = 6), a desmin mutation (n = 1) and immune checkpoint inhibitor treatment

Discussion

Analyzing consecutive patients admitted for acute myocarditis, we found that AIID-associated myocarditis may be life-threatening at the acute phase but has an overall good prognosis under specific treatment. Another important finding of our study was that high CRP level and pericardial effusion at diagnosis were both indicators for underlying AIID.

AIID-associated myocarditis is a rare condition. In our series, acute myocarditis was the heralding manifestation of a definite AIID in 6.9% of all

Conclusion

Acute myocarditis associated with AIID is rare but may be life-threatening at the acute phase, with an overall good prognosis under immunosuppressive therapy. CRP level and pericardial effusion at onset could help clinicians to identify patients with underlying AIID.

Declaration of Competing Interest

Authors did not declare conflict of interest.

Acknowledgements

We are thankful to Drs R Bertinchamps, N Chanson, MP Chauveheid, A Dossier, T Goulenok, R Jouenne, M Roriz, D Rouzaud, and D van Gysel for their help with patient screening.

Funding statement

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Contributorship statement

KS had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study design: GD, TP and KS. Acquisition of data: CC, AM, PO, GD, PM JFA, TP and KS. Analysis and interpretation of data: CC, AM, PO, GD, PM, JFA, TP and KS. Statistical analysis: AM and KS. Manuscript preparation: CC, TP and KS. Reviewed and approval of the manuscript CC, AM, PO, GD, PM, JFA, TP and KS.

References (23)

  • T. Guillemette et al.

    Lupus myocarditis: initial presentation and Longterm outcomes in a multicentric series of 29 patients

    J. Rheumatol.

    (2017)
  • All authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

    View full text