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Scleroderma overlap syndrome.

Alexandra Balbir-Gurman1, Yolanda Braun-Moscovici

  • 1Shine Rheumatology Unit, Rambam Health Care Campus, and Rappaport Faculty of Medicine, Technion-lsrael Institute of Technology, Haifa, Israel. a_balbir@rambam.health.gov.il

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|March 31, 2011
PubMed
Summary
This summary is machine-generated.

Overlap syndrome, where patients have systemic sclerosis (SSc) and another connective tissue disease (CTD), worsens clinical outcomes. Novel biological therapies may offer alternatives to high-dose steroids for these complex cases.

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Area of Science:

  • Rheumatology
  • Immunology
  • Internal Medicine

Background:

  • Overlap syndrome involves patients meeting criteria for at least two connective tissue diseases (CTDs).
  • Commonly associated CTDs include systemic sclerosis (SSc), dermatomyositis, Sjogren's syndrome, rheumatoid arthritis, and systemic lupus erythematosus.
  • Combined pathologies significantly influence clinical presentation, diagnosis, and treatment strategies.

Purpose of the Study:

  • To analyze the clinical features of SSc patients with overlap syndrome from the European (EUSTAR) database.
  • To review existing literature on clinical and diagnostic challenges in scleroderma overlap syndrome.
  • To explore emerging therapeutic options, including biological agents.

Main Methods:

  • Retrospective analysis of 165 consecutive SSc patient records.
  • Identification and review of cases meeting scleroderma overlap syndrome criteria.
  • Comprehensive PubMed literature search (1977-2009) using keywords: "overlap syndrome", "systemic sclerosis", "connective tissue disease", "biological agents".

Main Results:

  • Forty patients (24.2%) met criteria for scleroderma overlap syndrome.
  • High incidence of overlap conditions: dermatomyositis/polymyositis (47.5%), Sjogren's syndrome (42.5%), rheumatoid arthritis (15.4%), and SLE (5.0%).
  • Overlap syndrome significantly aggravated SSc clinical course, particularly affecting lungs, kidneys, digestion, vasculature, and joints, and influenced treatment choices.

Conclusions:

  • Defining scleroderma overlap syndrome is crucial, especially for patients requiring high-dose corticosteroids.
  • Novel biological therapies present a potential alternative to high-dose steroids, mitigating risks like renal crisis.
  • Biological agents may benefit one or both conditions, but their use requires a cautious, individualized approach due to limited trial data.