Epidemiology and Survival of Systemic Sclerosis-Sarcoidosis Overlap Syndrome

Megan Himmel; Meyer Balter; Zareen Ahmad; Elvira Bangert; Shafina Hasmani; Rhea Siddha; Mohammad Movahedi; Sindhu R Johnson

doi:10.3899/jrheum.220877

Epidemiology and Survival of Systemic Sclerosis-Sarcoidosis Overlap Syndrome

J Rheumatol. 2023 May;50(5):656-661. doi: 10.3899/jrheum.220877. Epub 2023 Feb 1.

Authors

Megan Himmel¹, Meyer Balter², Zareen Ahmad¹, Elvira Bangert¹, Shafina Hasmani¹, Rhea Siddha¹, Mohammad Movahedi³, Sindhu R Johnson⁴

Affiliations

¹ M. Himmel, MD, PhD, Z. Ahmad, MD, E. Bangert, MD, S. Hasmani, R. Siddha, MBA, Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital.
² M. Balter, MD, Division of Respirology, Department of Medicine, Mount Sinai Hospital.
³ M. Movahedi, MD, PhD, Toronto General Hospital Research Institute, University Health Network, and Institute of Health Policy, Management and Evaluation, University of Toronto.
⁴ S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, and Schroeder Arthritis Institute, Krembil Research Institute, Toronto Western Hospital, and Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. Sindhu.Johnson@uhn.ca.

PMID: 36725057
DOI: 10.3899/jrheum.220877

Abstract

Objective: We evaluated the epidemiology, manifestations, serology, comorbidities, and survival among patients with systemic sclerosis (SSc) with and without sarcoidosis.

Methods: We conducted a retrospective cohort study comparing patients with SSc with and without sarcoidosis. All patients fulfilled the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for SSc. Sarcoidosis was based on physician diagnosis and/or confirmatory biopsy. The primary outcome was time from diagnosis to all-cause mortality. Survival was evaluated using Kaplan-Meier curves.

Results: We included 1977 patients (1971 with SSc, 6 with SSc-sarcoidosis) with a SSc-sarcoidosis prevalence of 0.30%. Sarcoidosis frequently preceded SSc (66.66%). The most frequent sarcoidosis manifestations were pulmonary (66.66%), lymphadenopathy (66.66%), arthritis (50%), cutaneous (33.33%), and hepatic (16.66%). Patients with SSc and SSc-sarcoidosis had female to male sex ratios of 4.5:1 vs 5:1 and median ages of SSc onset of 48.3 vs 43.8 years, respectively. Interstitial lung disease (35% vs 66.66%) and pulmonary hypertension (24.91% vs 50%) tended to occur more frequently whereas abnormal nailfold capillaries (34.7% vs 16.66%) and digital ulcers (33.33% vs 16.66%) tended to occur less frequently among patients with SSc-sarcoidosis, but the differences were not significant. There was an increased frequency of stroke among the patients with SSc-sarcoidosis (relative risk 8.59, 95% CI 1.02-72.00). The median survival times were 23.4 years for SSc-sarcoidosis and 18.6 years for SSc, with no differences in survival curves (log-rank test, P = 0.55).

Conclusion: Sarcoidosis in SSc is rare but appears to occur more frequently than in the general population. It is associated with pulmonary, lymph node, cutaneous, joint, and hepatic involvement. Stroke occurs more frequently in patients with SSc-sarcoidosis but with no differences in survival.

Keywords: cohort; epidemiology; sarcoidosis; scleroderma; survival; systemic sclerosis.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Autoimmune Diseases* / complications
Female
Humans
Lung Diseases, Interstitial* / diagnosis
Male
Retrospective Studies
Sarcoidosis* / complications
Scleroderma, Systemic* / complications

Supplementary concepts

digital ulcers