Hepatic and mesenteric sarcoidosis without thoracic involvement: a case of severe noncirrhotic portal hypertension and successful pregnancy

Abstract

Here we report a case of a 25-year-old woman with mesenteric and hepatic sarcoidosis without lung involvement complicated by severe noncirrhotic portal hypertension. In 1992, at the clinical presentation, she had abdominal pain, asthenia, and weight loss. Splenomegaly, signs of flogosis, sideropenic anemia, and cholestasis were observed. Laparoscopic abdominal exploration and histological analysis demonstrated noncaseating granulomas of the liver, abdominal lymph nodes, and mesenteric connective tissue. The clinical course was severe with episodic remissions and recrudescences characterized by ascites (mild or moderate), elevation of bilirubin levels (mean: 1.1 mg/dl; range: 0.9-3.5 mg/dl), reduction of albumin levels (mean: 4 g/dl; range: 3.4-4.2 g/dl), and prolongation of elevated international normalized ratio (mean: 1; range: 0.9-1.4). In 1997, the patient had variceal bleeding. Complete hemostasis was obtained with band ligation. Liver function was preserved, and until 2000 the disease remained stable. In 2001, the patient became pregnant. At the 36th week of gestation, the patient delivered a healthy female infant and afterwards remained in clinical remission. This report stresses that sarcoidosis can have a hepatic and mesenteric involvement in absence of thoracic lymphadenopathy. Portal hypertension may be severe, and in absence of cirrhosis it may be associated with portal thrombosis. Finally, portal hypertension in patients with hepatic sarcoidosis and preserved liver function should not be considered as an absolute contraindication to pregnancy.

Hepatic sarcoidosis with symptomatic portal hypertension: A report of 12 cases with review of the literature

“…Variceal bleeding at diagnosis is exceptional and limited to case reports ( 20 ). Prevalence of symptomatic PH is not clear, as the few studies about hepatic sarcoidosis only report the presence of PH, without specifying its consequences, and the rare symptomatic patients are limited to case reports or small retrospective studies ( 5 , 11 , 12 , 20 – 26 ). While digestive bleeding and ascites are often described, hepatic encephalopathy is much rarer, with only a few case reports, often related to an advanced and untreated disease ( 27 – 29 ).…”

Localized Hepatosplenic Sarcoidosis with an Elevated Serum CA-125 Level

“…Extrapulmonary sarcoidosis in addition to pulmonary involvement has been well described and occurs in 50% of patients. However, sarcoidosis without any pulmonary involvement is uncommon [1]. Cancer Antigen 125 (CA-125) is a tumor marker that is associated with ovarian malignancy.…”