Difficulties in the early diagnosis of granulomatosis with polyangiitis (Wegener)

Abstract

Introduction: Granulomatosis with polyangiitis (GPA) is an autoimmune vasculitis, with a granulomatous necrotizing character, which predominantly affects small vessels, causing systemic damage to patients. The initial clinical manifestations are quite unspecific. Currently, the diagnosis is based on the patient's clinic, radiological, serological and pathological examinations. Objective: Discuss the difficulties in the early diagnosis of GPA, as the clinical manifestations are diverse, and sometimes similar to many other pathologies. Method: This is an integrative literature review, whose articles were collected in the SciELO, PubMed and BVS databases, using the descriptors Wegener's granulomatosis and granulomatosis with polyangiitis, combined with the Boolean operators AND diagnosis, AND clinical manifestations and NOT eosinophilic granulomatosis with polyangiitis. Case report articles published between 2011 and 2021 were selected, which contained the name of the disease in the title of the article and clearly addressed the diagnosis of the disease. Results: 16 reports that met the guiding question were included, being 11 reports in adults and 5 in children/adolescents. Conclusion: Nonspecific symptoms result in a delay in the diagnosis of GPA, however, the predominance of impairment of the respiratory and renal systems must be taken into account at the time of diagnosis. So far, there are no specific diagnostic criteria, but an association of factors. Even so, it is necessary a high suspicion of the physician, given the symptoms, making use of an earlier differential diagnosis, including ANCA analysis and biopsies, which are the gold standard in the diagnosis of GPA.