Primary Granulomatous Hepatitis: An Unusual Presentation of Disseminated Histoplasmosis

Abstract

Introduction: Histoplasmosis is an infection that presents initially with pulmonary symptoms but can become disseminated and commonly cause extrapulmonary manifestations in the immunocompromised patient population. We highlight a case of disseminated histoplasmosis that presented as a primary granulomatous hepatitis prior to clinical pulmonary involvement, which is extremely rare. Case: A 63-year-old Caucasian male presented with right upper quadrant abdominal pain, nausea, and vomiting over a 5-month period without cardiac or pulmonary symptoms. His initial labs showed elevated levels of total bilirubin, alkaline phosphatase, and transaminases. An initial comprehensive workup, including hepatic imaging, was negative. Liver biopsy revealed Histoplasma capsulatum organisms and hepatic granulomas. Eight days after initial presentation, the patient began to complain of dyspnea, cough, and confusion. He became progressively hypoxic and chest x-ray showed new diffuse bilateral interstitial infiltrates. Due to the concern for disseminated histoplasmosis, he was admitted to the intensive care unit (ICU), placed on mechanical ventilation, and started on intravenous Amphotericin B. His urine and serum Histoplasma antigens were positive and blood cultures grew mold. Further questioning revealed that the patient had significant recent exposure to bat guano while renovating his barn. The patient was worked up for an underlying immunodeficiency, which revealed low absolute T-cell counts and abnormal T-cell function. The patient had a 14-day hospital stay complicated by septic shock, acute respiratory failure, acute respiratory distress syndrome, disseminated intravascular coagulation, and acute kidney injury. Despite being in multi-system organ failure, the timely initiation of antifungal therapy led to the patient's rapid recovery and he was eventually discharged to home. Discussion: Histoplasmosis primarily causes a self-resolving pulmonary infection but can cause lifethreatening opportunistic infections in immunocompromised patients. Disseminated histoplasmosis carries a high mortality rate with treatment and is rapidly fatal if left untreated. There are very few cases reported that show extrapulmonary hepatic histoplasmosis as a primary manifestation of disseminated histoplasmosis, such as in our patient. This case highlights the need for a high index of suspicion and the importance of a prompt diagnosis as any delay in treatment can be fatal.Figure 1Figure 2