ANCA in systemic sclerosis, when vasculitis overlaps with vasculopathy: a devastating combination of pathologies

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• Scleroderma-associated ANCA vasculitis (SSc-AAV) often leads to severe pulmonary and renal complications.
• Antineutrophil cytoplasmic antibodies (ANCA) are common in systemic sclerosis (SSc), requiring clinical vigilance for SSc-AAV.
• Pathogenic overlap exists between SSc and AAV, potentially worsening vascular disease and organ damage.

Purpose of the Study:

• To outline a diagnostic approach for SSc-AAV.
• To address the limited data guiding SSc-AAV management.
• To propose further research into SSc-AAV treatment strategies.

Main Methods:

• Review of clinical presentations and diagnostic considerations for SSc-AAV.
• Analysis of potential pathogenetic interactions between SSc and AAV.
• Discussion of current treatment paradigms and their risks in SSc-AAV.

Main Results:

• SSc-AAV patients commonly exhibit microscopic polyangiitis or renal-limited vasculitis features.
• Anti-myeloperoxidase antibodies and p-ANCA are frequent findings.
• Standard AAV treatment can be detrimental in SSc-AAV, particularly high-dose steroids risking scleroderma renal crisis.

Conclusions:

• SSc-AAV is a rare but severe condition requiring high clinical suspicion.
• Current management of SSc-AAV relies on isolated AAV protocols, posing significant risks.
• Rigorous investigation and development of specific therapeutic strategies for SSc-AAV are urgently needed.