Clinical characteristics and prognosis of patients with isolated cardiac sarcoidosis: Insights from the ILLUMINATE‐CS study

Abstract

AIMData on the clinical features and prognosis of patients with isolated cardiac sarcoidosis (iCS) are limited. This study evaluated the clinical characteristics and prognostic impact of iCS.METHODS AND RESULTSThis was a secondary analysis of the ILLUMINATE‐CS study, a multicentre, retrospective registry investigating the clinical characteristics and prognosis of CS. iCS was diagnosed according to the 2016 Japanese Circulation Society (JCS) guidelines. Clinical characteristics and prognosis were compared between patients with iCS and systemic CS (sCS). The primary outcome was a combined endpoint of all‐cause death, hospitalisation for heart failure, or fatal ventricular arrhythmia events. Among 475 patients with CS (mean age, 62.0 ± 10.9 years; female ratio, 59%) diagnosed by the JCS guidelines, 119 (25.1%) were diagnosed with iCS. Patients with iCS had a higher prevalence of a history of atrial fibrillation or hospitalisation for heart failure, or lower left ventricular ejection fraction than those with sCS. During a median follow‐up of 42.3 (interquartile range, 22.8–72.5) months, 141 primary outcomes (29.7%) occurred. Cox proportional hazard analysis revealed that iCS was a significant risk factor for the primary outcome in the unadjusted model (hazard ratio [HR], 1.62; 95% confidence interval [CI], 1.12–2.34; P=0.011). However, this association was not retained after adjustment for other covariates (adjusted HR, 1.27; 95% CI, 0.86–1.88; P=0.226).CONCLUSIONSPatients with iCS had more impaired cardiovascular function at the time of diagnosis than those with sCS. However, iCS was not independently associated with poor prognosis after adjustment for prognostic factors.This article is protected by copyright. All rights reserved.

Utility of new FDG-PET/CT guidelines for diagnosing cardiac sarcoidosis in patients with implanted cardiac pacemakers for atrioventricular block

“…Second, the definition of iCS was established for the first time 8 . Until now, cases with findings strongly suggestive of clinical CS might not have been diagnosed with CS before disease progression because they did not meet the criteria of CS 21 . According to the new definition, iCS can be diagnosed without histology if the four above-mentioned items are met 8 .…”

Isolated cardiac sarcoidosis – a wild card

“…Recently, Maeda and colleagues presented data from a large multicentre registry (ILLUMINATE-CS) investigating the clinical characteristics and prognosis of patients with CS diagnosed according to the 2016 Japanese Circulation Society guidelines. 9 The study aimed to provide information related to isolated CS phenotype in comparison to CS patients with extra-cardiac histopathological or imaging evidence of systemic sarcoidosis. A significant number of patients (one fourth of the total CS population) were found to have isolated disease.…”

“…The multicentre study of 475 CS patients by Maeda et al 9 . reveals important baseline characteristics of isolated CS.…”