Early diagnosis of granulomatosis with polyangiitis: An introduction to the newly designed Iran criteria

Abstract Backgrounds In the absence of practical diagnostic criteria for diagnosis of granulomatosis with polyangiitis (GPA), a new diagnostic criteria for GPA is proposed based on literature review for characteristic manifestations of GPA and expert opinion. The sensitivity of the new criteria, Iran criteria for GPA, is assessed in comparison with 1990 American College of Rheumatology (ACR) criteria for Wegener's granulomatosis (WG). Methods Evaluation of three organs (ear, nose and throat (ENT); lung; kidney) and two laboratory findings (anti-neutrophil cytoplasmic antibody; biopsy), abbreviated mnemonically as ELKAB, is suggested in our criteria. A retrospective sensitivity analysis was performed based on medical records of 35 patients. Clinical diagnosis of GPA by a single rheumatologist was used as the gold standard. Results Records of a total of 15 male and 20 female patients with a mean follow-up duration of 21.26 ± 4.13 months were considered. Mean age at diagnosis and mean disease duration were 32.37 ± 2.33 years and 19.06 ± 5.41 months, respectively. The sensitivity for Iran criteria for GPA and 1990 ACR classification criteria for WG were calculated as 100% and 80%, respectively. Conclusions Iran criteria for GPA is a highly sensitive instrument for detecting GPA patients in comparison with 1990 ACR classification criteria for WG.