Multisystem sarcoidosis presenting with hepatic cirrhosis: When silent granulomas turn fibrotic

Abstract

Sarcoidosis is a multisystem granulomatous disorder of unknown cause, most commonly involving the lungs. Although hepatic involvement is frequent histologically, it is often clinically silent and rarely progresses to cirrhosis. Its variable presentation can resemble other infectious or malignant conditions, making diagnosis challenging, especially in regions where such diseases are prevalent.

Case Presentation: A 56-year-old diabetic, hypertensive woman presented with chronic right upper abdominal pain, prolonged low-grade fever, night sweats, anorexia, and 14 kg weight loss. No respiratory or gastrointestinal symptoms were noted. Laboratory evaluation showed elevated alkaline phosphatase (235 U/L), GGT (184 U/L), hypercalcemia (13.2 mg/dL), and raised serum ACE (119.5 U/L). Viral and autoimmune markers were negative. Chest imaging revealed bilateral hilar lymphadenopathy. Spiral CT showed a left-sided pulmonary inflammatory lesion with mild to moderate pleural effusion and extensive mediastinal lymphadenopathy involving paratracheal, pretracheal, subcarinal, aortopulmonary, and bilateral hilar regions, with multiple lobulated hypodense nodular lesions. Histopathology of liver and lymph node biopsy demonstrated non-caseating granulomas with bridging fibrosis and cirrhosis (METAVIR F4), without malignancy or infection.

Results & Treatment: Findings supported a diagnosis of multisystem Sarcoidosis with hepatic and pulmonary involvement, based on characteristic imaging, elevated ACE, hypercalcemia, and histological evidence of non-caseating granulomas with cirrhosis. Other infectious, malignant, and autoimmune causes were excluded. The patient was initiated on systemic corticosteroid therapy along with mycophenolate mofetil (MMF), with close monitoring of clinical status, liver function, and radiological response to assess treatment efficacy and disease progression.

Conclusion: This case underscores the importance of considering sarcoidosis in patients with granulomatous liver disease and systemic symptoms, even in tuberculosis-endemic settings. Early recognition is crucial to avoid misdiagnosis and ensure appropriate management.

Bangladesh J Medicine 2026; Vol. 37, No. 2(1): pp. 240-241