Pituitary-related granulomatosis with polyangiitis

WANG Liu; HU Yu-chen; ZHANG Yu-wei

doi:10.19538/j.nk2024110105

Abstract：Granulomatosis with polyangiitis(GPA)is a systemic disease closely related to anti-neutrophil cytoplasmic antibody(ANCA)and is characterized by immune necrotizing small vessel vasculitis.GPA can involve any organ system,with the most common target organs being the upper respiratory tract,lungs,and kidneys.GPA rarely involves the pituitary gland,accounting for approximately 1％of all GPA cases,and is often characterized by pituitary hormone dysfunction and/or imaging abnormalities in the saddle region.Pituitary involvement is often accompanied by symptoms of other organ involvement or might be the only clinical manifestation.Diagnosis requires a combination of systemic symptoms,saddle zone imaging,and serum ANCA measurement.Glucocorticoid hormones combined with cyclophosphamide or rituximab are routinely used to induce remission,with rituximab preferred in refractory or relapsed cases.Although other systemic disorders of GPA respond favorably to treatment,pituitary function,especially adenopituitary function,is often not restored,and some patients require lifelong hormone replacement therapy;early screening and treatment can help avoid irreversible pituitary damage and improve prognosis.