Hanna Ramy M, Kaldas Marian, Arman Farid, Wang Melissa, Hammer Terrance, Sinkowitz Deren, Rastogi Anjay
Division of Nephrology.
Division of Rheumatology, Department of Medicine.
Clin Nephrol Case Stud. 2018 Sep 4;6:21-26. doi: 10.5414/CNCS109513. eCollection 2018.
. Sarcoidosis is a rare autoimmune disease resulting in formation of non-necrotizing "non-caseating" granulomas generally in the lung. The disease classically strikes African American females in their fourth and fifth decades. The resulting hypercalcemia is a result of 1-α hydroxylase overexpression in granulomas with increased 1,25-dihydroxy vitamin D levels. This phenomenon can also be observed in mycobacterial and fungal infections that produce granulomas in infected patients. Thus, chronic infectious diseases are part of differential diagnosis of granulomatous processes. We present an elderly Caucasian female who presented with hypercalcemia with serum calcium of 11 - 14 mg/dL and an elevated ionized calcium of 1.4 - 1.5 mmol/L. Initially cholecalciferol supplements were stopped, but hypercalcemia persisted for more than 2 months. 1,25-dihydroxy vitamin D levels were markedly elevated with low normal 25-hydroxy vitamin D levels, angiotensin-converting enzyme levels were also high, and chest computed tomography (CT) imaging was negative for any lymphadenopathy (including perihilar lymphadenopathy). Malignancy and infectious workups were negative for fungal and mycobacterial infections. Positron emission tomography revealed several small lymph nodes in right upper lobe of lung, and biopsy of bone marrow and lung lymph-nodes revealed non-caseating granulomata. We present an atypical case of occult sarcoidosis presenting mainly with biochemical findings without any definitive imaging findings, making diagnosis a clinical challenge.
结节病是一种罕见的自身免疫性疾病,通常会在肺部形成非坏死性“非干酪样”肉芽肿。这种疾病典型地侵袭40到50岁的非裔美国女性。由此导致的高钙血症是由于肉芽肿中1-α羟化酶过度表达,使1,25-二羟维生素D水平升高所致。在感染患者中产生肉芽肿的分枝杆菌和真菌感染中也可观察到这种现象。因此,慢性感染性疾病是肉芽肿性病变鉴别诊断的一部分。我们报告一例老年白人女性,其血清钙为11 - 14mg/dL,离子钙升高至1.4 - 1.5mmol/L,表现为高钙血症。最初停用了胆钙化醇补充剂,但高钙血症持续了2个多月。1,25-二羟维生素D水平显著升高,而25-羟维生素D水平正常偏低,血管紧张素转换酶水平也高,胸部计算机断层扫描(CT)成像未发现任何淋巴结病(包括肺门周围淋巴结病)。恶性肿瘤及感染检查未发现真菌和分枝杆菌感染。正电子发射断层扫描显示右肺上叶有几个小淋巴结,骨髓和肺淋巴结活检显示为非干酪样肉芽肿。我们报告一例隐匿性结节病的非典型病例,主要表现为生化检查结果,无任何明确的影像学表现,这使得诊断成为一项临床挑战。