Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome

Samar Alharbi^{1

2}, Zareen Ahmad^{1

2}, Arthur A Bookman^{1

2}, Zahi Touma^{1

2}, Jorge Sanchez-Guerrero^{1

2}, Nicholas Mitsakakis^{1

2}, Sindhu R Johnson^{3

4}

Affiliations

¹ From the Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Division of Rheumatology, Mount Sinai Hospital, University of Toronto; Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto, Toronto, Ontario, Canada; Taibah University, Medina, Saudi Arabia.
² S. Alharbi, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto, and Taibah University; Z. Ahmad, MD, Division of Rheumatology, Mount Sinai Hospital, University of Toronto; A.A. Bookman, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Z. Touma, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Institute of Health Policy, Management and Evaluation, University of Toronto; J. Sanchez-Guerrero, MD, MSc, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto; N. Mitsakakis, PhD, Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, University of Toronto, and Division of Rheumatology, Mount Sinai Hospital, and Institute of Health Policy, Management and Evaluation, University of Toronto.
³ From the Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Division of Rheumatology, Mount Sinai Hospital, University of Toronto; Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto, Toronto, Ontario, Canada; Taibah University, Medina, Saudi Arabia. Sindhu.Johnson@uhn.ca.
⁴ S. Alharbi, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto, and Taibah University; Z. Ahmad, MD, Division of Rheumatology, Mount Sinai Hospital, University of Toronto; A.A. Bookman, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Z. Touma, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Institute of Health Policy, Management and Evaluation, University of Toronto; J. Sanchez-Guerrero, MD, MSc, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto; N. Mitsakakis, PhD, Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, University of Toronto, and Division of Rheumatology, Mount Sinai Hospital, and Institute of Health Policy, Management and Evaluation, University of Toronto. Sindhu.Johnson@uhn.ca.

PMID: 30008448
DOI: 10.3899/jrheum.170953

Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome

Samar Alharbi et al. J Rheumatol. 2018 Oct.

. 2018 Oct;45(10):1406-1410.

doi: 10.3899/jrheum.170953. Epub 2018 Jul 15.

Authors

Samar Alharbi^{1

2}, Zareen Ahmad^{1

2}, Arthur A Bookman^{1

2}, Zahi Touma^{1

2}, Jorge Sanchez-Guerrero^{1

2}, Nicholas Mitsakakis^{1

2}, Sindhu R Johnson^{3

4}

Affiliations

¹ From the Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Division of Rheumatology, Mount Sinai Hospital, University of Toronto; Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto, Toronto, Ontario, Canada; Taibah University, Medina, Saudi Arabia.
² S. Alharbi, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto, and Taibah University; Z. Ahmad, MD, Division of Rheumatology, Mount Sinai Hospital, University of Toronto; A.A. Bookman, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Z. Touma, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Institute of Health Policy, Management and Evaluation, University of Toronto; J. Sanchez-Guerrero, MD, MSc, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto; N. Mitsakakis, PhD, Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, University of Toronto, and Division of Rheumatology, Mount Sinai Hospital, and Institute of Health Policy, Management and Evaluation, University of Toronto.
³ From the Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Division of Rheumatology, Mount Sinai Hospital, University of Toronto; Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto, Toronto, Ontario, Canada; Taibah University, Medina, Saudi Arabia. Sindhu.Johnson@uhn.ca.
⁴ S. Alharbi, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto, and Taibah University; Z. Ahmad, MD, Division of Rheumatology, Mount Sinai Hospital, University of Toronto; A.A. Bookman, MD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network; Z. Touma, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Institute of Health Policy, Management and Evaluation, University of Toronto; J. Sanchez-Guerrero, MD, MSc, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, and Division of Rheumatology, Mount Sinai Hospital, University of Toronto; N. Mitsakakis, PhD, Institute of Health Policy, Management and Evaluation, and Toronto Health Economics and Technology Assessment Collaborative, University of Toronto; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Division of Rheumatology, Toronto Western Hospital, University Health Network, University of Toronto, and Division of Rheumatology, Mount Sinai Hospital, and Institute of Health Policy, Management and Evaluation, University of Toronto. Sindhu.Johnson@uhn.ca.

PMID: 30008448
DOI: 10.3899/jrheum.170953

Abstract

Objective: Systemic sclerosis (SSc) may overlap with systemic lupus erythematous (SLE). Little is known about the epidemiology, clinical characteristics, and survival of SSc-SLE overlap. We evaluated the prevalence of SSc-SLE overlap and differences in SSc characteristics, and compared survival with SSc without SLE.

Methods: A cohort study was conducted including subjects who fulfilled the American College of Rheumatology (ACR)/European League Against Rheumatism classification criteria for SSc and/or the ACR criteria for SLE. The primary outcome was time from diagnosis to all-cause mortality. Survival was evaluated using Kaplan-Meier and Cox proportional hazard models.

Results: We identified 1252 subjects (SSc: n = 1166, SSc-SLE: n = 86) with an SSc-SLE prevalence of 6.8%. Those with SSc-SLE were younger at diagnosis (37.9 yrs vs 47.9 yrs, p < 0.001), more frequently East Asian (5.5% vs 20%) or South Asian (5.1% vs 12%), had lupus anticoagulant (6% vs 0.3%, p < 0.001), anticardiolipin antibody (6% vs 0.9%, p < 0.001), and pulmonary arterial hypertension (PAH; 52% vs 31%, p < 0.001). Those with SSc-SLE less frequently had calcinosis (13% vs 27%, p = 0.007), telangiectasia (49% vs 75%, p < 0.001), and diffuse subtype (12% vs 35%, p < 0.001). There were no significant differences in the occurrence of renal crisis (7% vs 7%), interstitial lung disease (ILD; 41% vs 34%), and digital ulcers (38% vs 32%). Those with SSc-SLE had better median survival time (26.1 vs 22.4 yrs), but this was not statistically significant (log-rank p = 0.06). Female sex and diffuse subtype attenuated survival differences between groups (HR 1.07, 95% CI 0.67-1.67).

Conclusion: Patients with SSc-SLE are younger at diagnosis, more frequently have PAH, and less frequently have cutaneous manifestations of SSc. They should be monitored for ILD, renal crisis, and digital ulcers.

Keywords: COHORT STUDIES; MORTALITY; SURVIVAL ANALYSIS; SYSTEMIC LUPUS ERYTHEMATOSUS; SYSTEMIC SCLEROSIS.

PubMed Disclaimer

Publication types

Actions

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions
Actions

Substances

Actions
Actions

LinkOut - more resources

Full Text Sources
- HighWire
Other Literature Sources
- scite Smart Citations
Medical
- MedlinePlus Health Information

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome

Affiliations

Epidemiology and Survival of Systemic Sclerosis-Systemic Lupus Erythematosus Overlap Syndrome

Authors

Affiliations

Abstract

Publication types

MeSH terms

Substances

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical