Systemic sclerosis & Raynaud's

Antineutrophil Cytoplasmic Antibody–Associated Vasculitides in Systemic Sclerosis: A Unique Clinical Overlap With Significant Implications for Treatment and Outcomes

Arthritis RheumatolPublished Retrospective cohort study

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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