Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists · 2013
Refractory pituitary granulomatosis with polyangiitis (Wegener's) treated with rituximab.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
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Hughes Jing, Barkhoudarian Garni, Ciarlini Pedro, Laws Edward R, Mody Elinor, Inzucchi Silvio E, Woodmansee Whitney W
Аннотация
OBJECTIVE: Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is an autoimmune disease characterized by inflammation of blood vessels most often seen in the upper respiratory tract, lungs, kidneys, and skin. Central nervous system (CNS) involvement of GPA is rare, particularly in the pituitary, and can be difficult to treat.
METHODS: Case report.
RESULTS: We present a 30-year-old woman with pituitary and ocular GPA, whose unusually recalcitrant disease led to the development of pan-hypopituitarism and near-total vision loss. After failing multiple systemic immunosuppressants, she was ultimately treated with the novel immunomodulatory agent rituximab together with pulse corticosteroids, which achieved a gratifying response.
CONCLUSION: Pituitary and optic chiasm involvement is a rare complication of GPA. We believe this case illustrates the complexity of management of pituitary GPA and provides insight into the potential utility of the biologic agent rituximab in this disease.
METHODS: Case report.
RESULTS: We present a 30-year-old woman with pituitary and ocular GPA, whose unusually recalcitrant disease led to the development of pan-hypopituitarism and near-total vision loss. After failing multiple systemic immunosuppressants, she was ultimately treated with the novel immunomodulatory agent rituximab together with pulse corticosteroids, which achieved a gratifying response.
CONCLUSION: Pituitary and optic chiasm involvement is a rare complication of GPA. We believe this case illustrates the complexity of management of pituitary GPA and provides insight into the potential utility of the biologic agent rituximab in this disease.