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Scleroderma overlap syndromes.

¹Department of Medicine, Division of Rheumatology, University of Western Ontario, London, Canada. janet.pope@sjhc.london.on.ca

Current Opinion in Rheumatology

|November 1, 2002

Summary

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Scleroderma, an autoimmune connective tissue disease, often overlaps with other autoimmune conditions like rheumatoid arthritis and Sjögren syndrome. Understanding these overlaps is key for accurate diagnosis and treatment.

Area of Science:

Rheumatology
Immunology
Internal Medicine

Background:

Scleroderma is a connective tissue disease characterized by fibrosis and vascular issues, often with autoimmune features.
Positive antinuclear antibodies and family histories of autoimmune diseases like SLE and rheumatoid arthritis are common in scleroderma patients.
Some patients present with overlapping features of scleroderma and other autoimmune conditions.

Purpose of the Study:

To review recent literature on scleroderma with overlap features.
To enhance the understanding of scleroderma's association with other autoimmune diseases.

Main Methods:

Literature review of recent reports on scleroderma overlap syndromes.
Analysis of serological markers and clinical presentations in overlapping conditions.

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Main Results:

Scleroderma overlaps with rheumatoid arthritis show distinct features, including diffuse scleroderma, positive Scl-70, pulmonary fibrosis, and later erosive rheumatoid arthritis.
While SLE rarely co-occurs with scleroderma, Sjögren syndrome symptoms are frequent.
Specific scleroderma antibodies like Scl-70 can be found in other connective tissue diseases, including SLE.
Myositis can be a feature, with anti-PM Scl antibody positivity indicating a better prognosis in scleroderma-polymyositis overlap.
Vasculitis and eosinophilic fasciitis are rare but reported associations.

Conclusions:

Scleroderma frequently overlaps with other autoimmune diseases, influencing clinical presentation and prognosis.
Understanding these overlaps is crucial for accurate diagnosis and management of patients with scleroderma.
Serological markers, while sometimes specific, can be present in other connective tissue diseases, necessitating comprehensive evaluation.