Department of Otolaryngology, Head and Neck Surgery, Charité-Universitätsmedizin Berlin, Mittelallee 2, 13353, Berlin, Germany.
Department of Medicine B-Hepatology, Gastroenterology, Endocrinology, Diabetes, Hematology, Oncology, Palliative Care, Brandenburg Medical School, Neuruppin, Germany.
J Med Case Rep. 2022 Jul 28;16(1):291. doi: 10.1186/s13256-022-03492-7.
Granulomatosis with polyangiitis, formerly known as Wegener granulomatosis, is a necrotizing vasculitis with granulomatous inflammation that belongs to the class of antineutrophil cytoplasmic antibodies-positive diseases. It occurs in a localized and a systemic form and may present with a variety of symptoms. Involvement of the upper respiratory tract is very common, while neurologic, endocrinological, and nephrological dysfunction may occur.
We describe the case of a 29-year-old Central European male patient presenting with severe bilateral sensorineural hearing loss, otorrhea, and one-sided facial nerve paralysis. The patient was unsuccessfully treated with i.v. antibiotics at another hospital in Berlin, and tympanic tubes were inserted. After presentation to our emergency room, he was hospitalized and further diagnostics started. Increased fluid intake and 12 kg weight gain over the last months were reported. The patient was diagnosed with granulomatosis with polyangiitis and diabetes insipidus. The patient's condition improved after treatment with rituximab.
A comprehensive PubMed search of all articles with granulomatosis with polyangiitis and diabetes insipidus was conducted to assess which combination of symptoms occurs simultaneously and whether other parts of the pituitary are commonly involved. The 39 selected articles, describing 61 patients, showed that ear-nose-throat involvement occurred most commonly, in 71% of cases. Of patients, 59% had involvement of the anterior pituitary gland, while true panhypopituitarism occurred in 13% of cases. Only one case report featured the same set of symptoms as described herein.
Granulomatosis with polyangiitis is a highly variable disease, commonly involving the upper airways, but that may present with symptoms solely related to the pituitary gland. Clinicians should have a low threshold to investigate for granulomatosis with polyangiitis in patients with therapy-resistant otorrhea. Patients may present with a complex set of symptoms, and integrating different specialists when additional symptoms occur may lead to faster diagnosis.
肉芽肿性多血管炎，以前称为 Wegener 肉芽肿，是一种坏死性血管炎，伴有肉芽肿性炎症，属于抗中性粒细胞胞质抗体阳性疾病类别。它分为局限性和系统性两种形式，可能表现出多种症状。上呼吸道受累非常常见，而神经、内分泌和肾脏功能障碍也可能发生。
我们描述了一位 29 岁的中欧男性患者，他表现为严重双侧感音神经性听力损失、耳漏和单侧面神经麻痹。该患者在柏林的另一家医院接受静脉注射抗生素治疗无效，并插入了鼓膜管。在我们的急诊室就诊后，他被住院并开始进一步诊断。据报告，过去几个月来，他的液体摄入量增加了 12 公斤，体重增加了 12 公斤。该患者被诊断为肉芽肿性多血管炎和尿崩症。该患者在用利妥昔单抗治疗后病情有所改善。
我们对所有包含肉芽肿性多血管炎和尿崩症的文章进行了全面的 PubMed 搜索，以评估同时出现的哪些症状组合以及垂体的其他部位是否常见受累。选择的 39 篇文章描述了 61 例患者，结果显示耳鼻喉受累最常见，占 71%。59%的患者有前垂体腺受累，而真正的全垂体功能减退症发生率为 13%。仅有一例病例报告出现了与本文所述相同的症状组合。
肉芽肿性多血管炎是一种高度可变的疾病，通常累及上呼吸道，但也可能出现仅与垂体有关的症状。对于治疗无效的耳漏患者，临床医生应降低对肉芽肿性多血管炎的调查阈值。患者可能出现复杂的症状组合，当出现其他症状时，整合不同的专家可能会更快地做出诊断。
