Unusual Manifestation of Sarcoidosis: Hepatic Involvement With Budd-Chiari Syndrome

Abstract Introduction: Sarcoidosis is a multisystem granulomatous disease commonly affecting the lungs and lymphatic system, with liver and bone involvement occurring less frequently. Hepatic sarcoidosis can lead to complications such as portal hypertension, although Budd-Chiari syndrome (BCS) due to sarcoidosis is exceedingly rare. Sarcoidosis with axial skeletal involvement and hepatic vein thrombosis poses significant diagnostic and therapeutic challenges. This case highlights an atypical presentation of sarcoidosis involving the liver and axial skeleton, complicated by Budd-Chiari syndrome. Case Description A 51-year-old female with a history of asthma presented with syncope and dyspnea. Initial workup revealed elevated blood pressure and tachycardia. Imaging indicated a right pleural effusion, right hilar mass, hepatomegaly, and mediastinal lymphadenopathy. A 1.1 cm omental nodule, scattered liver masses, and an endometrial soft tissue mass raised concerns for malignancy. B-HCG was elevated at 13.6 mIU/mL, with pregnancy ruled out, and CA 125 was elevated at 128 U/mL. Cytology from thoracentesis, endometrial and endocervical biopsies, were negative for malignancy. She developed right-sided flank pain, dyspnea on exertion, and recurrent pleural effusions requiring a PleurX catheter. MRI revealed diffuse hypoenhancement around portal tracts, characteristic of a “nutmeg liver” appearance, and occluded hepatic veins, consistent with Budd-Chiari syndrome. PET imaging demonstrated hypermetabolic thoracic and abdominal lymphadenopathy with liver involvement and ascites, with increased patchy uptake in the bone marrow and patchy consolidation in the lung with mildly avid nodules, suggestive of sarcoidosis. EUS-guided peripancreatic lymph node and pleural biopsies revealed noncaseating granulomas without malignant cells, confirming sarcoidosis.The patient was managed with prednisone, Aldactone, and anticoagulation. Liver biopsy was deferred due to stable liver function (MELD 12), and follow-up MRI showed stable liver findings with decreased lymph node and bone lesions. This case illustrates the rare complication of Budd-Chiari syndrome secondary to sarcoidosis, highlighting the diagnostic value of imaging and biopsy in distinguishing sarcoidosis from malignancy in complex presentations. Discussion: Sarcoidosis with hepatic involvement typically leads to granulomatous hepatitis, which can progress to portal hypertension but rarely results in Budd-Chiari syndrome. The pathophysiology of hepatic vein thrombosis in sarcoidosis may involve granulomatous inflammation within the hepatic vasculature. This case highlights the importance of considering sarcoidosis as a differential diagnosis in patients presenting with widespread lymphadenopathy, pleural effusion, and hepatic lesions, especially when non-caseating granulomas are present. The management of sarcoidosis complicated by Budd-Chiari syndrome involves addressing both the underlying sarcoid inflammation and the vascular thrombosis. This patient's treatment included corticosteroids and anticoagulation, leading to symptomatic improvement.