Untypical Manifestations of Granulomatosis with Polyangiitis—A Review of the Literature

Granulomatosis with polyangiitis (GPA, previously known as Wegener’s granulomatosis) is a type of ANCA-associated vasculitis that affects small- to medium-sized vessels. GPA occurs with a prevalence of 24–152:1000000. The disease affects all races at every age. Various factors may have an impact on the etiology of GPA which is treated as an autoimmune disease. Genetic factors, infectious agents (like Staphylococcus aureus ), environmental factors (like silica, hydrocarbons, fumes, pesticides, and farming) are considered elements for the development of the disease. Mostly, GPA affects the upper and lower respiratory tracts and kidneys and associated with otorhinolaryngological and renal manifestations. However, numerous untypical manifestations may also occur. Our review is aimed at discussing the most significant of them, including the neurological, cardiac, gastrointestinal tract, joints and muscles, skin, and ophthalmological manifestations. The whole literature was searched in PubMed. It has been used phrases ‛granulomatosis with polyangiitis’, ‛Wegener’s granulomatosis’ and ‛GPA’. The initial research for every sentence yielded subsequently, 4472, 5043, and 7110 results. Only studies with available full text were retrieved. After a three-stage evaluation including a language evaluation, a heading evaluation, and an abstract evaluation, we obtained 139 relevant papers on which our review is based. GPA is a huge challenge for contemporary diagnostics and medicine. Our review is aimed at demonstrating the multiplicity of untypical manifestations and proving that every doctor may come into contact with a patient with GPA.