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

Highlights

• About 30% of Adult onset Still's disease developed cardiac involvement.

• AAdmission to intensive care and use of biotherapy were more frequent during follow-up in AOSD + C group than control group.

• Cardiac involvement was associated with refractory form in multivariate analyzed.

Abstract

Objective

Adult-onset Still's disease (AOSD) is a rare inflammatory disease that may be life-threatening if complicated by cardiac problems. We performed a retrospective multicenter study to describe the manifestations, treatments and outcomes of cardiac involvement in AOSD.

Methods

We reviewed the medical databases of eight centers. All AOSD patients identified as fulfilling Yamagushi's or Fautrel's criteria were included in the study. Cardiac involvement, clinical manifestations, laboratory features, the course of the disease and treatments were evaluated.

Results

We included 96 AOSD patients in this study: 28 (29%) had documented cardiac involvement (AOSD + C group) and 68 (71%) had no cardiac involvement (control group). Cardiac complications were observed at diagnosis in 89% of cases. It were pericarditis (n = 17), tamponade (n = 5), myocarditis (n = 5) and non-infectious endocarditis (n = 1). Levels of leukocytes, neutrophils and C-reactive protein were significantly higher (p = 0.02, p = 0.02 and p = 0.002, respectively in the AOSD + C group than in the control group. Admission to intensive care, and the use of biotherapy were more frequent during follow-up in the AOSD + C group than the control group (p = 0.0001 and p = 0.03 respectively). Cardiac involvement was associated with refractory form in multivariate analyzed (p = 0.01). Corticosteroids were effective with or without methotrexate in 71% of patients but not in severe involvement as myocarditis or tamponade.

Conclusion

Cardiac complications are frequent, inaugural, can be life-threatening and predictive of a refractory course in patients with AOSD. Systematic cardiac screening should be proposed at diagnosis and biotherapy early use should be considered especially in myocarditis.

Introduction

Adult-onset Still's disease (AOSD) is a rare multisystemic disease of unknown etiology, categorized as a polygenic autoinflammatory disorder [1]. The typical manifestations of AOSD include prolonged fever, arthralgia, polyarthritis, evanescent rash, leukocytosis and hyperferritinemia [2]. Two sets of classification criteria based on these manifestations have been proposed for AOSD: Yamaguchi's criteria [3], and Fautrel's criteria [4].

Other, less frequent manifestations of AOSD have also been described during the evaluation and follow-up of patients [1]. These manifestations include reactive hemophagocytic lymphohistiocytosis (RHL), pulmonary or neurological complications, acute hepatic or renal failure and cardiac involvement [5]. Cardiac complications can affect all layers of the heart and may have a very poor prognosis, with myocarditis [6], tamponade [7] or endocarditis [8]. AOSD treatment has long been essentially empirical, based on corticosteroids and disease-modifying antirheumatic drugs (DMARDs) such as methotrexate (MTX) [2]. Patients with AOSD refractory to treatment with steroids and methotrexate may be improved by biological DMARDs (bDMARDs), such as IL-1 and IL-6 antagonists, which are usually well tolerated [9,10].

To date, cardiac complications have been rarely studied, with only isolated observations or small series of cases published. Herein, we provide a retrospective multicenter study describing the clinical features, treatments, and outcomes of cardiac involvement in AOSD.