Classification and classification criteria for vasculitis: achievements, limitations and prospects

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• Disease classification is crucial for research and understanding relationships between conditions.
• Vasculitis classification presents challenges due to the diverse and complex nature of these diseases.
• Standardized classification criteria are essential for homogenizing patient populations in research studies.

Purpose of the Study:

• To review recent advances in vasculitis classification.
• To highlight the impact of the 2012 Chapel Hill Consensus Conference (CHCC) nomenclature.
• To discuss the clinical relevance and limitations of current vasculitis classification systems.

Main Methods:

• Analysis of the 2012 Chapel Hill Consensus Conference (CHCC) nomenclature.
• Review of updated classification criteria for specific vasculitis entities.
• Evaluation of the primary categorization criterion based on affected vessel size.

Main Results:

• The 2012 CHCC nomenclature introduced a 'variable-vessel vasculitis' category for Behçet's disease and Cogan's syndrome.
• Classification was expanded to include secondary and single-organ vasculitis.
• New or revised classification criteria were published for Behçet's disease and cryoglobulinemic vasculitis.

Conclusions:

• Vasculitis classification is continually evolving with advancements in understanding and diagnosis.
• The reliance on vessel size for classification remains questionable in clinical practice.
• While criteria exist for many vasculitis types, polyarteritis nodosa, microscopic polyangiitis, and IgA vasculitis require further definition.