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Abstract: Aim: To describe the clinical features, treatment and long‐term outcomes in north Indian patients with granulomatosis with polyangiitis (GPA). Methods: Clinical details, Birmingham Vasculitis Activity Score (BVAS‐v3), laboratory data, histopathology findings, treatment details and outcomes of all consecutive patients diagnosed as having GPA between April 2005 and April 2016 were retrieved. European Vasculitis Study Group definitions were used to classify patients into localized, early systemic, generalized, severe and refractory categories. The findings were compared with large single center cohorts from USA, Germany and France. Results: One hundred and five patients (median age 40 years; 60 females and 45 males) were diagnosed as having GPA. Median duration of follow up was 28 months. Upper respiratory tract, renal and lung involvement were seen in 76.19%, 51.43% and 67.62% of patients, respectively. Ocular, ear, central nervous system, peripheral nervous system, heart, gastrointestinal and skin involvement were seen in 40.95%, 18.1%, 26.67%, 11.43%, 5.71%, 12.38% and 29.52%, respectively. The median BVAS v3 score was 17. Localized, early systemic, generalized, severe and refractory disease were seen in three, 44, 38, 17 and one patient, respectively. Eighteen patients expired and the cause of death was disease activity in 11, disease activity plus sepsis in four and sepsis alone in three patients. Twenty‐six patients experienced relapses. No hemorrhagic cystitis or bladder cancers were observed; however, one patient developed myelodysplastic syndrome during follow up. Conclusion: Indian patients are younger and have less arthritis, renal and peripheral nerve involvement as compared to the patients in Western cohorts. [ABSTRACT FROM AUTHOR]

Aim Many patients with systemic necrotizing vasculitis ( SNV) satisfy classification criteria of different disease entities when different classification systems are used. A new classification algorithm has been proposed recently by using the American College of Rheumatology criteria, Chapel Hill Consensus Criteria ( CHCC) and Sorensen surrogate markers for a more uniform classification of patients suffering from these rare disorders. Methods We applied this algorithm to patients diagnosed as having systemic vasculitis between 2007 and 2011. We also analyzed the data using this algorithm by incorporating the recently proposed revised CHCC nomenclature of vasculitis in place of the older criteria. Results Seventy-nine patients with SNV were studied. One patient diagnosed as microscopic polyangiitis ( MPA) had to be excluded from analysis as she had previously been diagnosed as having Behcet's disease. All patients of eosinophilic granulomatosis with polyangiitis ( EGPA), granulomatosis with polyangiitis ( GPA) and MPA were reclassified to the same diagnostic subcategory after application of the algorithm. Three (16.7%) of 18 polyarteritis nodosa patients were unclassifiable after application of the consensus algorithm while two (11.1%) were reclassified as MPA. All previously unclassifiable patients could be classified either as MPA or GPA after application of the new algorithm. There was no difference in the results when the CHCC 2012 nomenclature was used instead of the older CHCC in the consensus algorithm. Conclusion The new classification algorithm is a reliable method for classification of SNV for epidemiological purposes in our population. [ABSTRACT FROM AUTHOR]