Rawala Muhammad Shabbir, Ahmed Amna Saleem, Helmick Kristen
Department of Internal Medicine, WVU-Charleston Division, Charleston, WV, USA.
Department of Medicine, Rapides Regional Medical Center, Alexandria, LA, USA.
Case Rep Rheumatol. 2020 Jun 25;2020:8840245. doi: 10.1155/2020/8840245. eCollection 2020.
Sarcoidosis is an idiopathic, chronic, multisystem, granulomatous, inflammatory disease involving almost all organs. Sarcoidosis can occur with an atypical presentation of hepatosplenic involvement, like in the case of our patient. In this case report, we present a rare case of extrapulmonary sarcoidosis with isolated involvement of the liver and spleen in a 39-year-old Caucasian female. There is a possibility of this isolated involvement of an organ in the complete absence of pulmonary disease, which makes the diagnosis of sarcoidosis very difficult as it is usually not suspected. Ultrasound and CT are important in ruling out other differential diagnoses, but a definitive diagnosis is possible only on histological examination, differentiating sarcoid lesions from , primary biliary cirrhosis, metastasis, malignancy, and other granulomatous infections or diseases. Hence, the most credible criterion for diagnosis remains histology. After diagnosis, regular follow-up for systemic manifestations is recommended. Asymptomatic patients with hepatosplenic sarcoidosis have a good prognosis without any medical intervention, while patients with abnormal labs or symptoms must commence treatment.
结节病是一种特发性、慢性、多系统、肉芽肿性炎症性疾病，几乎累及所有器官。结节病可表现为肝脾受累的非典型形式，就像我们的患者那样。在本病例报告中，我们呈现了一例罕见的肺外结节病，一名39岁的白种女性仅肝脏和脾脏受累。在完全没有肺部疾病的情况下，一个器官单独受累是有可能的，这使得结节病的诊断非常困难，因为通常不会怀疑到该病。超声和CT对于排除其他鉴别诊断很重要，但只有通过组织学检查，将结节病病变与原发性胆汁性肝硬化、转移瘤、恶性肿瘤以及其他肉芽肿性感染或疾病区分开来，才能做出明确诊断。因此，最可靠的诊断标准仍然是组织学。诊断后，建议对全身表现进行定期随访。无症状的肝脾结节病患者在无任何医学干预的情况下预后良好，而实验室检查异常或有症状的患者必须开始治疗。
