Abstract:

Objective: Granulomatosis with polyangiitis (GPA) is one of the systemic vasculitis included in antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Clinical characteristics, ANCA serotypes, and their frequencies vary depending on the type of vasculitis. In this study, we aimed to investigate the link between ANCA serotypes and clinical variables documented at the time of admission. Methods: This study was a retrospective cross-sectional study including 40 patients with GPA. The demographic, clinical, laboratory, and radiological findings were extracted from medical records. Logistic Regression Analysis was performed to estimate the relationship between ANCA serotypes and other variables. Results: The mean age was 53.95±12.94 years, and there was a delay of 15.85±23.03 months in diagnosis. While constitutional symptoms were observed in 42% of patients, symptoms related to ear-nose-throat in 70%, lung in 87.5%, kidney in 62.5%, neurological findings in 30%, eye in 25%, skin in 25% and musculoskeletal system findings in 90% were noted. Male gender was seen more frequently in cANCA (60.7%) positive patients; however, pANCA-positive patients were mainly female (83.3%) (p=0.050). Delay in diagnosis was longer (p=0.001) and subglottic stenosis was more common (p<0.001) in pANCA-positive patients. However, the inflammatory response was more severe, and renal involvement (p=0.007) and arthralgia (p=0.048) were higher in cANCA-positive patients. Conclusion: While renal involvement seemed to be related to cANCA, subglottic stenosis was mostly associated with pANCA in GPA. However, larger studies are needed to make a more accurate conclusion about the effect of ANCA serotypes on diversity in the clinical spectrum.

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