垂体相关肉芽肿性多血管炎
Pituitary-related granulomatosis with polyangiitis
王柳 1胡雨晨 1张雨薇1
作者信息
- 1. 四川大学华西医院内分泌代谢科,四川成都 610041;四川大学华西医院糖尿病与代谢研究中心,四川成都 610041
- 折叠
摘要
肉芽肿性多血管炎(GPA)是一种与抗中性粒细胞胞浆抗体(ANCA)密切相关的全身性疾病,其特征是免疫性坏死性小血管炎.GPA可累及任何器官系统,最常见的靶器官为上呼吸道、肺和肾脏.GPA累及垂体很少见,约占1％,常表现为垂体激素功能障碍和(或)鞍区影像学异常.垂体受累时常伴有其他器官受累的症状,也可能是唯一的临床表现,诊断需要结合全身症状、鞍区影像学检查及血清ANCA测定.糖皮质激素联合环磷酰胺或利妥昔单抗是常规的诱导缓解方案,难治或复发病例首选利妥昔单抗.尽管GPA其它系统疾病对治疗有良好的反应,垂体功能尤其是腺垂体功能常不能得到恢复,部分需终生激素替代治疗,早期筛查和治疗有助于避免垂体的不可逆性损伤,从而改善预后.
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.
关键词
肉芽肿性多血管炎/垂体炎/中枢性尿崩症Key words
granulomatosis with polyangiitis/hypophysitis/central diabetes insipidus引用本文复制引用
出版年
2024
国家科技期刊平台
NSTL
万方数据