Department of Neurosurgery, Georgetown University School of Medicine, Washington, DC, USA
Division of General Internal Medicine, MedStar Georgetown University Hospital, Washington, DC, USA.
BMJ Case Rep. 2021 Jan 28;14(1):e237774. doi: 10.1136/bcr-2020-237774.
A man in his early 60s with a medical history of granulomatosis with polyangiitis (GPA) in remission for two decades without maintenance therapy presented with non-specific complaints of profound fatigue and 40-pound weight loss. He was seronegative for antinuclear antibodies and cytoplasmic antineutrophilic antibodies, but erythrocyte sedimentation rate and C reactive protein levels were elevated. Endocrinological testing revealed adrenal insufficiency, hypogonadism, hypothyroidism and diabetes insipidus. An MRI of the head revealed extensive sinonasal inflammation eroding through the floor of the sella turcica and into the pituitary gland and stalk. Biopsy of the sinonasal tissues was inconclusive. On review of his case, a multidisciplinary team diagnosed him with panhypopituitarism secondary to a recurrence of GPA. He responded well to glucocorticoids and methotrexate with marked reduction of pituitary enhancement on imaging and resolution of diabetes insipidus. He will require lifelong testosterone, levothyroxine and glucocorticoids for hormone replacement therapy.
一位 60 岁出头的男性，二十年来患有缓解期的肉芽肿伴多血管炎（GPA），未接受维持治疗，因非特异性的极度疲劳和 40 磅体重减轻而就诊。他的抗核抗体和细胞质抗中性粒细胞抗体均为阴性，但红细胞沉降率和 C 反应蛋白水平升高。内分泌检查显示肾上腺功能不全、性腺功能减退、甲状腺功能减退和尿崩症。头部 MRI 显示广泛的鼻旁窦炎症侵蚀通过鞍底进入垂体和垂体柄。鼻旁窦组织的活检结果不确定。在回顾他的病例时，一个多学科团队诊断他患有由于 GPA 复发引起的全垂体功能减退症。他对糖皮质激素和甲氨蝶呤反应良好，影像学上垂体增强显著减少，尿崩症得到缓解。他将需要终身接受睾酮、左甲状腺素和糖皮质激素进行激素替代治疗。
