Extensive Gastrointestinal Manifestations as the Main Relapsing Disease in Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA-Wegener’s) is a small vessel vasculitis associated with the presence of antineutrophil cytoplasm autoantibodies. Gastrointestinal involvement ranges from 5 to 20% of cases. We describe a case in whom relapsing disease with widespread gastrointestinal manifestations including pharyngeal, esophageal, small and large intestine, and anus was present for the first time. A 43-years-old male, with a 10-year history of proteinase-3 (PR3-ANCA) positive GPA which presented initially with general (fever, malaise), articular, otorhinolaryngological, ocular (scleritis), and pulmonary manifestations (nodular disease, biopsy-proven), was treated with glucocorticoids (GC) and methotrexate (MTX) as remission induc- tion therapy, followed by MTX as maintenance treatment for 5 years. After remission was achieved at month 6, gradual tapering of MTX during the following 4.5 years allowed for its withdrawal in 2010.