A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage.

Q1 Medicine Auto-Immunity Highlights Pub Date : 2017-12-01 Epub Date: 2017-04-28 DOI:10.1007/s13317-017-0094-5
Moris Sangineto, Chiara Valentina Luglio, Patrizia Suppressa, Carlo Sabbà, Nicola Napoli
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Abstract

Sarcoidosis is a systemic disease characterized by an immune-mediated disorder, which leads to the development of non-caseating granulomas in the involved organs. More than 90% of patients with sarcoidosis present lungs and lymphatic system involvement at onset, while less than 10% has an isolated extrapulmonary localization. Here, we describe the case of an elderly patient with isolated hepato-splenic onset (multiple splenic lesions at imaging and cholestasis), and subsequent pulmonary involvement. The liver biopsy showed the presence of non-caseating granulomas, suggesting sarcoidosis. Despite the complete recovery was obtained with steroid therapy, after dosage reduction the patient presented watery diarrhea. Endoscopic investigations with biopsies were performed, describing the presence of an important lympho-plasmacytic infiltrate of terminal ileum mucosa with typical aspects of inflammatory bowel disease. The symptomatology completely disappeared after steroid dosage increase. This case confirms that sarcoidosis could present in a very atypical way, involving several organs in a different manner at the same time and that every symptom should not be underestimated, despite the rare presentation.

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结节病伴孤立性肝脾发病,并在恢复期发展为炎症性肠病1例。
结节病是一种以免疫介导的疾病为特征的全身性疾病,可导致受累器官发生非干酪化肉芽肿。超过90%的结节病患者在发病时表现为肺和淋巴系统受累,而不到10%的患者有孤立的肺外定位。在这里,我们描述了一个老年患者的病例,孤立的肝脾发病(影像学和胆汁淤积多发性脾病变),随后肺部受累。肝活检显示非干酪化肉芽肿,提示结节病。尽管类固醇治疗完全恢复,但减少剂量后患者出现水样腹泻。内镜检查活检,描述了一个重要的淋巴浆细胞浸润的回肠末端粘膜的典型方面炎症性肠病的存在。类固醇剂量增加后症状完全消失。本病例证实结节病可能以非常不典型的方式出现,同时以不同的方式累及多个器官,尽管表现罕见,但每种症状都不应低估。
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