Department of Endocrinology and Metabolism, Ankara City Hospital, Ankara, Turkey.
Faculty of Medicine, Department of Endocrinology and Metabolism, Ankara Yildirim Beyazit University, Ankara, Turkey.
Ir J Med Sci. 2023 Jun;192(3):1171-1176. doi: 10.1007/s11845-022-03107-6. Epub 2022 Jul 27.
Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare multisystem necrotizing vasculitis that involves small- to medium-sized blood vessels. We report a rare case of syndrome of the inappropriate antidiuretic hormone (ADH) secretion (SIADH) secondary to EGPA. A 53-year-old man applied with complaints of pain in the large joints and morning stiffness in knee for 2 months. The patient had the history of impaired fasting glucose, asthma, nasal polyps, and urticaria. Physical examination revealed intrinsic muscle atrophy and weakness in the right hand. Peripheral eosinophil count was 9.78 × 109/L (0.02-0.5), erythrocyte sedimentation rate 39 mm/h (0-20), and C-reactive protein 5.77 mg/dL (0-0.5). Migratory ground-glass pulmonary opacities had been reported in previous chest computed tomography scans. Echocardiography revealed findings compatible with eosinophilic involvement. Electroneuromyographic evaluation showed acute distal axonal neuropathy of right ulnar nerve. EGPA was considered. Oral methylprednisolone treatment was initiated. Intravenous immunoglobulin (IVIG) and cyclophosphamide treatment and gradual tapering of oral steroids were planned. In 24-h urine analysis, sodium was 387 mEq, creatinine was 1156 mg, and volume was 3000 mL. When his medical records were investigated, it was observed that hyponatremia was present for nearly 2 years. While serum osmolality was 270, urine osmolality was 604 mOsm/kg HO. So, SIADH diagnosis was made. Fluid intake was restricted. Although the patient's sodium level did not return to normal, it rose up to 130 mEq/L. After second cycle of EGPA treatment (cyclophosphamide and IVIG), serum sodium was normal. There is only four other documented cases of SIADH associated with EGPA. We hypothesized that blood supply to the hypothalamus and/or posterior hypophysis might be affected from EGPA vasculitis. Here, in this case, with effective treatment of EGPA, SIADH was resolved which implies a causality between two conditions.
嗜酸性肉芽肿伴多血管炎（EGPA，变应性肉芽肿性血管炎）是一种罕见的多系统坏死性小到中等血管血管炎。我们报告了一例由 EGPA 引起的抗利尿激素不适当分泌综合征（SIADH）的罕见病例。一名 53 岁男性因大关节疼痛和膝关节晨僵 2 个月就诊。患者有空腹血糖受损、哮喘、鼻息肉和荨麻疹病史。体格检查发现右手内在肌萎缩和无力。外周血嗜酸性粒细胞计数为 9.78×109/L（0.02-0.5），红细胞沉降率为 39mm/h（0-20），C 反应蛋白为 5.77mg/dL（0-0.5）。先前的胸部计算机断层扫描显示有游走性磨玻璃样肺混浊。超声心动图显示符合嗜酸性粒细胞浸润的结果。电神经肌图评估显示右侧尺神经急性远端轴索性神经病。考虑 EGPA。开始口服甲泼尼龙治疗。计划静脉注射免疫球蛋白（IVIG）和环磷酰胺治疗，并逐渐减少口服类固醇剂量。在 24 小时尿液分析中，钠为 387mEq，肌酐为 1156mg，尿量为 3000mL。当检查他的病历时，发现低钠血症存在近 2 年。虽然血清渗透压为 270，但尿渗透压为 604mOsm/kg HO。因此，诊断为 SIADH。限制液体摄入。尽管患者的钠水平未恢复正常，但升至 130mEq/L。在 EGPA 治疗的第二个周期（环磷酰胺和 IVIG）后，血清钠恢复正常。仅有其他 4 例文献报道的与 EGPA 相关的 SIADH 病例。我们假设 EGPA 血管炎可能影响下丘脑和/或垂体后叶的血液供应。在这里，在这种情况下，随着 EGPA 的有效治疗，SIADH 得到解决，这表明两种情况之间存在因果关系。
