Department of Internal Medicine and Infectious Disease, Vrije Universiteit Brussel, Brussel, UZ Brussel, Belgium.
Department of Anatomopathology, Vrije Universiteit Brussel, Brussel, UZ Brussel, Belgium.
Acta Clin Belg. 2022 Aug;77(4):767-771. doi: 10.1080/17843286.2021.1977510. Epub 2021 Sep 12.
Sarcoidosis is a systemic granulomatous disease, characterized by the formation of non-necrotizing granulomas. Even though granulomas are frequently found in liver biopsy, related symptoms rarely occur. In the current article, a case report is pictured to increase the knowledge on portal hypertension in hepatic sarcoidosis.
A 62-year-old female was diagnosed with variceal bleeding for which elastic banding was performed. The patient was admitted to the intensive care unit (ICU) as the bleeding persisted and she evolved in hemorrhagic shock. Liver ultrasound detected nodular hepatomegaly and partial portal thrombosis. Chest CT showed diffuse hilar adenopathies and interstitial micronodular lesion. Finally, PET-CT detected metabolic active liver, bone marrow, and upper and lower diaphragmatic adenopathies.
Multidisciplinary discussion brought major advantages in rapid diagnosis and prompt effective treatment. Cirrhosis was diagnosed by liver nodularity on imaging and liver biopsy. Sarcoidosis diagnosis was supported by the biopsies of liver and lymph node, which yielded non-caseating granulomas infiltration. Chest CT scan and PET-CT were also consistent with this diagnosis. The complementary analysis excluded differential diagnosis. The patient was treated with high-dose methylprednisolone with notable clinical improvements and discharge from the ICU.
Hepatic sarcoidosis can present as life-threatening bleeding due to variceal bleeding caused by portal hypertension. Differential diagnosis is broad when hepatic sarcoidosis is suspected. Therefore, a multidisciplinary discussion is warranted. Anatomopathological examination of two potentially involved organs should be considered to make the appropriate diagnosis. Further studies are requested to investigate the pathophysiological mechanism of portal hypertension.
结节病是一种系统性肉芽肿性疾病，其特征为非坏死性肉芽肿的形成。尽管肝活检常可见肉芽肿，但相关症状很少发生。在本文中，我们报告了一例病例，旨在提高对肝结节病门脉高压的认识。
一名 62 岁女性因静脉曲张出血接受了弹性带结扎治疗。由于出血持续存在且患者进展为出血性休克，她被收入重症监护病房（ICU）。肝脏超声显示肝肿大伴结节，部分门静脉血栓形成。胸部 CT 显示弥漫性肺门淋巴结肿大和间质性小结节病变。最后，PET-CT 显示代谢活跃的肝脏、骨髓和上下膈肌淋巴结。
多学科讨论在快速诊断和及时有效治疗方面带来了显著优势。肝脏结节和肝活检提示肝硬化。肝和淋巴结活检发现非干酪样肉芽肿浸润，支持结节病的诊断。胸部 CT 扫描和 PET-CT 也符合这一诊断。补充分析排除了鉴别诊断。患者接受了大剂量甲基强的松龙治疗，临床症状显著改善，并从 ICU 出院。
肝结节病可因门脉高压导致静脉曲张出血而出现危及生命的出血。当怀疑肝结节病时，鉴别诊断范围广泛。因此，需要多学科讨论。应考虑对两个潜在受累器官进行解剖病理学检查以做出适当诊断。需要进一步研究以探讨门脉高压的病理生理机制。
