Overlap syndrome of systemic sclerosis with antineutrophil cytoplasmic antibody-associated vasculitis according to 2022 ACR/EULAR criteria

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Authors: Jang Woo Ha ; Jung Yoon Pyo ; Sung Soo Ahn ; Jason Jungsik Song ; Yong-Beom Park ; Sang-Won Lee

MeSH: Adult ; Aged ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / blood ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / complications ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / diagnosis ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / immunology ; Antibodies, Antineutrophil Cytoplasmic / blood ; Biomarkers / blood ; Female ; Humans ; Lung Diseases, Interstitial / diagnosis ; Lung Diseases, Interstitial / etiology ; Lung Diseases, Interstitial / immunology ; Male ; Middle Aged ; Predictive Value of Tests ; Retrospective Studies ; Scleroderma, Systemic* / complications ; Scleroderma, Systemic* / diagnosis ; Scleroderma, Systemic* / immunology ; Syndrome

Keywords: Antibodies, antineutrophil cytoplasmic ; Systemic sclerosis ; Vasculitis

Abstract: Background/Aims: This study applied the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with systemic sclerosis (SSc) and investigated the frequency of overlap syndrome of SSc and AAV (SSc-AAV-OS).

Methods: Among the 232 patients diagnosed with SSc, 105 with signs suggestive of small- or medium-vessel vasculitis, which were defined as the present of interstitial lung disease (ILD), peripheral neuropathy, or suspected renal vasculitis, were included in this study and analyzed.

Results: Among the 105 SSc patients, the detection rate of ANCA was 19.0%. When the 2022 ACR/EULAR criteria were applied, the frequency of SSc-AAV-OS was 20.0%, which was much higher than 1.7% reported with previous criteria for AAV. ANCA positivity contributed to the reclassification of SSc-AAV-OS more than ANCA negativity in SSc patients with signs suggestive of small- or medium-vessel vasculitis.

Conclusions: The frequency of SSc-AAV-OS in SSc patients with signs suggestive of small- or medium-vessel vasculitis at diagnosis was 20.0%. Therefore, we suggest that physicians should perform ANCA tests in SSc patients exhibiting signs suggestive of small- or medium-vessel vasculitis and apply the new criteria for AAV.