Diagnosis and classification of systemic sclerosis.

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Systemic sclerosis (SSc) diagnosis historically relied on clinical and chest X-ray findings (1980 ARA criteria).
• These initial criteria excluded 10-20% of patients, necessitating revised classifications.
• An international consensus in 1988 introduced nail fold capillaroscopy and autoantibodies, defining diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc).

Purpose of the Study:

• To review the evolution of SSc classification criteria.
• To introduce the concept of early or limited SSc (lSSc) and SSc sine scleroderma.
• To highlight the ongoing debate regarding the distinctness of lSSc and SSc sine scleroderma.

Main Methods:

• Review of historical classification criteria for SSc.
• Analysis of diagnostic contributions from nail fold capillaroscopy and autoantibody determination.
• Examination of proposed subsets: dcSSc, lcSSc, lSSc, and SSc sine scleroderma.

Main Results:

• Early SSc criteria (1980) were insufficient, leading to revised classifications in 1988 and 2001.
• The 2001 criteria proposed an lSSc subset requiring Raynaud's phenomenon, specific capillaroscopy, and/or autoantibodies.
• SSc sine scleroderma, characterized by visceral involvement without skin thickening, emerged as a potential distinct subset.

Conclusions:

• SSc classification has advanced significantly, incorporating capillaroscopy and autoantibodies for improved diagnostic accuracy.
• The proposed lSSc subset and SSc sine scleroderma require further investigation to clarify their diagnostic and clinical significance.
• Distinguishing between lSSc and SSc sine scleroderma remains an open question in SSc research.