No abstract

Purpose of reviewGranulomatosis with polyangiitis (GPA), a multisystem disease with diverse systemic and protean ocular manifestations. Its pathophysiology, spectrum of the ocular manifestations, changing paradigms in the diagnosis of the disease, recent updates in the treatment patterns are clinically relevant to the treating clinician. They are described in this review.Recent findingsThe ocular manifestations are not included in the updated diagnostic criteria of GPA. Hence, high index of suspicion is required when a clinician encounters eye as the first clinical presentation.The concentration of interleukin -32 and 6 are correlated to disease activity of GPA.Enzyme-linked immunoassay for antineutophilic antibodies are recommended over indirect immunofluorescence to detect the subtypes with clinical correlation. The false positivity of test results in tuberculosis needs to be ruled out.Both rituximab and cyclophosphamide are equally considered as a first line of therapy of GPA in the induction phase as per randomized studies. During the continuation phase, switch over to methotrexate and mycophenolate can be considered.SummaryThe changing trends in the diagnosis, treatment can be adapted to real time clinical practice to provide the best quality of life to patients with GPA.