Sarcoidosis is an inflammatory disorder categorized by the presence of noncaseating granulomas, which has the ability to affect multiple organs of the body [1],[2]. The pulmonary system is the most common site of involvement (90% of cases) [1]. The liver is the third most commonly affected organ system after the lungs and lymph nodes. It has been found that 50–65% of sarcoid patients have hepatic involvement as per liver biopsy [2].

Budd–Chiari syndrome is a heterogeneous group of clinical conditions presenting with hepatic venous outflow obstruction, which can be located at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium [3]. It is classified as being primary if flow is obstructed due to an endoluminal aberration, or secondary if the flow is obstructed by compression or invasion of a lesion outside the hepatic venous outflow track [3].

The Budd–Chiari syndrome is not common as a complication of hepatic sarcoidosis but the association is possible. A dozen cases are reported in the literature, to which we add our case of Budd–Chiari syndrome in the context of a sarcoidosis with liver injury.

We report the case of a 32-year-old female patient with a history of mediastino-pulmonary, hepatic, and splenic sarcoidosis since her young age, under corticotherapy. The patient was lost to follow-up for two years and she willingly stopped her medication. She was readmitted at our Emergency Department for recurrent threatening high digestive bleeding. The oeso-gastroduodenal fibroscopy found esophageal varices. Biological assessment found a cytolysis and cholestasis with elevated alkaline phosphatase (289 IU/L), gamma-glutamyltransferase (GGT) (147 IU/L), total bilirubin (5.4 mg/dL) mostly conjugated, alanine aminotransferases (ALATs) (36 IU/L), and serum alanine aminotranspeptidases (ASAT) (47 IU/L).

Hepatic Doppler ultrasound showed nodular bumpy hepatomegaly, with outflow obstruction of the hepatic veins on pulse wave and color flow Doppler, and a laminated aspect of the inferior vena cava that remains permeable.

Abdominal CT showed multiple hepatic and splenic nodules enhanced after contrast injection, multiple peritoneal and retroperitoneal lymphadenopathies, and a lack of individualization of hepatic veins. The portal vein and its branches were permeable (Figure 1, Figure 2, Figure 3).

Chest CT showed an interstitial syndrome with diffuse parenchymal reticulomicronodular infiltrate associated with bilateral mediastinal and hilar adenopathies (Figure 4A and B). Viral serologies B and C were negative.

The search for thrombophilia, namely dosage of protein C, protein S, antithrombin III, nocturnal paroxysmal hemoglobinuria, antiphospholipid syndrome, Leiden’s factor V mutation, and the prothrombin gene mutation, was negative.

Hepatic sarcoidosis was previously confirmed by liver biopsy, which objectified noncaseating granulomas, with a negative tuberculosis testing (IDR, BK in sputum, and GeneXpert) and a high level of angiotensin-converting enzyme (97 UECA).

All these assessments led to the diagnosis of Budd–Chiari syndrome complicating hepatic sarcoidosis.

Cholestasis and portal hypertension represent the most common complications of hepatic sarcoidosis. Budd–Chiari syndrome is actually an exceptional complication of sarcoidosis. It has been reported in a dozen cases in literature [4],[5],[6],[7],[8],[9],[10],[11],[12],[13]. The pathophysiology is not clearly elucidated; however, two hypotheses are proposed to explain this causality. On the one hand, the extrinsic compression of the hepatic veins by the adjacent nodules, which would lead to venous stasis with endoluminal thrombus formation [13], causing a secondary Budd–Chiari syndrome [14]. On the other hand, granulomatous infiltration of the vascular wall [11] by contiguity, without formation of vascular thrombi, could lead to a primary Budd–Chiari syndrome [14].

Clinically, depending on the number of occluded veins and the speed of occlusion, Budd–Chiari syndrome may remain asymptomatic, or manifest as hepatomegaly, abdominal pain, hepatocellular insufficiency, or ascites.

The imaging will find portal hypertension signs with suprahepatic obstacle, manifested by disorders of the flow on the Doppler ultrasound and of a lack of enhancement of hepatic veins and/or inferior vena cava, with development of collateral venous circulation. We can also find a dysmorphic liver with multiple hepatic nodules (related to hepatic parenchymal involvement of sarcoidosis), perfusion disorders, or ascites.

Treatment of Budd–Chiari syndrome will be based on the management of its etiology, in this case sarcoidosis (corticosteroids, immunosuppressants, synthetic antimalarials), the prevention of venous thrombosis and the restoration of hepatic venous drainage [15] (surgical or endovascular). Liver transplantation may be considered in patients with rapidly worsening hepatocellular insufficiency, which was the case of our patient.

Budd–Chiari syndrome is thus an exceptional complication, but possible in case of hepatic sarcoidosis. Therefore, it is worthwhile to look for it in case of any worsening of hepatic sarcoidosis, and to suggest possible sarcoidosis in case of Budd–Chiari syndrome.