Systemic sclerosis & Raynaud's
Antineutrophil Cytoplasmic Antibody–Associated Vasculitides in Systemic Sclerosis: A Unique Clinical Overlap With Significant Implications for Treatment and Outcomes
Authors' conclusion
AAV, predominantly microscopic polyangiitis with MPO-ANCA, can occur in SSc, particularly in patients with fibrosing ILD and anti-Scl70. Standard vasculitis treatments appear to be effective and do not worsen outcomes in SSc.
Study context
- Population
- Patients meeting 2013 American College of Rheumatology (ACR)/EULAR criteria for systemic sclerosis (SSc) and 2022 ACR/EULAR criteria for AAV, 18 French centresmedian age 51.5 years, 83% female
- Participants
- 30 patients
- Outcome
- Clinical phenotype, management and outcomes of overlapping SSc and AAV
Methodological detailsmulticentre
- Multicentre
Main finding
As reported in the abstract
- SSc preceded AAV
- all cases
- Diffuse cutaneous SSc
- 27%
- Limited cutaneous SSc
- 73%
- Anti-Scl70 antibodies
- 50%
- Interstitial lung disease (ILD)
- 80%
- Fibrosing nonspecific interstitial pneumonia pattern
- 54%
- MPA
- 90%
- MPO-ANCA positivity
- 93%
- Renal involvement
- 76%
- Serum creatinine, median (IQR 120-361 μmol/L)
- 170 μmol/L
- Urine protein to creatinine ratio (IQR 0.9-2.3 g/g creatinine)
- 2 g/g creatinine
- Glucocorticoids (GCs) plus CYC
- 50%
- GCs plus RTX
- 47%
- Scleroderma renal crisis
- 0
Observational data: associations, not effects.
Original publication
Antineutrophil Cytoplasmic Antibody–Associated Vasculitides in Systemic Sclerosis: A Unique Clinical Overlap With Significant Implications for Treatment and Outcomes. Arthritis Rheumatol. Published 27 February 2026. doi:10. 1002/ art. 70032
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