부산대학교 도서관

Granulomatosis with polyangiitis (GPA), formerly called Wegener granulomatosis, is a rare vasculitis affecting the small vessels. It commonly involves the upper respiratory tract, lungs and kidneys, which lead to dyspnea, hemoptysis or hematuria. The damage to the organs can be fatal. Though the exact mechanisms leading to GPA are not well understood, anti-neutrophil cytoplasmic antibodies are considered to be associated with the inflammation in GPA. The diagnosis of GPA is based on clinical presentation, laboratory findings and image studies. The 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology recently developed revised classification criteria for GPA. Treatment consists of an induction phase and a maintenance phase with glucocorticoid or immunosuppressive agents such as cyclophosphamide, rituximab or methotrexate. GPA is a rare disease in Taiwan. Here, we reported the case of a patient with GPA, who presented with bilateral lung cavitary nodules and consolidations. The diagnosis was proven by echo-guided lung biopsy. The patient showed a good response to cyclophosphamide pulse therapy and was under maintenance therapy with rituximab and oral prednisolone.