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The differential diagnosis of systemic sclerosis
Alan Tyndall1, Susanna Fistarol
1aDepartment of Rheumatology bDepartment of Dermatology, University Hospital Basel, Basel, Switzerland.
Current Opinion in Rheumatology
|September 25, 2013
Summary
Accurate diagnosis of systemic sclerosis (SSc) is crucial for timely treatment. Differentiating SSc from other causes of skin thickening, such as eosinophilic fasciitis, requires careful evaluation including autoantibody testing and skin biopsy.
Area of Science:
- Rheumatology
- Dermatology
- Immunology
Background:
- Systemic sclerosis (SSc) diagnosis is improving with new classification criteria.
- Early diagnosis enables timely treatment with novel modalities.
- Distinguishing SSc from other conditions causing diffuse skin thickening is critical.
Purpose of the Study:
- To highlight the importance of excluding non-SSc causes of diffuse skin thickening.
- To discuss conditions that mimic SSc, such as gadolinium-induced nephrogenic systemic fibrosis.
- To emphasize the role of new diagnostic tools and treatments in SSc management.
Main Methods:
- Review of recent literature on SSc diagnosis and mimic conditions.
- Discussion of advanced autoantibody detection (topoisomerase 1, centromere, RNA polymerase).
- Consideration of skin biopsy findings (eosinophil infiltration, mucin, amyloid).
Main Results:
- Gadolinium-induced nephrogenic systemic fibrosis can mimic SSc.
- Immunoablation and stem cell transplantation show promise for some SSc patients.
- SSc-specific autoantibodies aid in precise subclassification, impacting prognosis and treatment.
Conclusions:
- Skin thickening is nonspecific; early scleroderma has characteristic features (symmetrical thickening, Raynaud's, nailfold changes, antinuclear antibodies).
- Absence of these features necessitates excluding other conditions, primarily eosinophilic fasciitis.
- Skin biopsy, autoantibody testing, and clinical evaluation are key to differentiating SSc from mimics.
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