Classification of systemic sclerosis. Visions and reality

Area of Science:

• Rheumatology
• Dermatology
• Internal Medicine

Background:

• Systemic sclerosis (SSc), or scleroderma, is a severe condition impacting life expectancy.
• Classifying SSc is complex due to its heterogeneous nature and overlap with similar conditions.
• Limited and diffuse cutaneous SSc are recognized subsets with distinct prognoses, but an intermediate form remains debated.

Purpose of the Study:

• To evaluate the clinical utility of capillaroscopy in differentiating SSc subtypes.
• To assess the potential of nailfold video-capillaroscopy for improved SSc classification and prognosis.
• To determine if an intermediate cutaneous SSc classification offers advantages over limited and diffuse forms.

Main Methods:

• Review of capillaroscopy techniques, including digitized video-capillaroscopy.
• Analysis of described capillaroscopic patterns ('early', 'active', 'late', 'slow').
• Evaluation of evidence supporting SSc subset classification.

Main Results:

• Capillaroscopy aids in distinguishing SSc from idiopathic Raynaud's phenomenon.
• Digitized video-capillaroscopy offers advanced capillary assessment.
• No convincing evidence supports an intermediate SSc subtype over limited and diffuse classifications.

Conclusions:

• Nailfold video-capillaroscopy's prognostic value in daily practice requires further assessment.
• CREST syndrome features are not exclusive to specific SSc subsets.
• Current evidence favors classifying SSc into limited and diffuse forms, without clear benefit from an intermediate category.