A 78 year old man presented with right upper quadrant abdominal pain to the emergency department. He had no signs of acute abdomen. Labs were significant for low platelet count, elevated alkaline phosphatase. CT abdomen showed cholelithiasis, no evidence of acute cholecystitis; bilateral adrenal nodules left 2.8 and right 1.8 cm. He was advised general surgery and gastroenterology follow up. He underwent elective cholecystectomy and liver biopsy. Pathology was reported as granulomatous hepatitis, negative for microorganisms. However, per biopsy report primary suspicion remained an infectious process. Infectious work up at that time was negative which included histoplasma antibody. The gastroenterologist started him on prednisone for management of granulomatous hepatitis of unknown etiology.
4 months later he presented again to the ED with right upper quadrant abdominal pain and fever. Labs again showed low platelet count and elevated alkaline phosphatase. CT abdomen showed bilateral adrenal masses which had increased in size to 5.5cm on the left and 4.7 cm on the right. Patient was admitted and further work up was done to rule out pheochromocytoma and infectious etiology. Extensive infectious work up was done and only histoplasma antigen was positive. Finally biopsy of the adrenal mass and bone marrow was diagnostic for histoplasmosis. Diagnosis was made of disseminated histoplasmosis affecting the liver, adrenals and bone marrow. Patient was given 2 weeks of induction amphotericin B and then switched to Itraconzole which he will continue for at least one year.
Progressive disseminated histoplasmosis occurs in 1 in 2000 patients with acute infection. Risk factors for disseminated histoplasmosis include AIDS, other immunosuppressive disorders, patients on immunosuppressive medications and extremes of age. People who have no known underlying immunosuppression may develop chronic disseminated histoplasmosis due to unknown defects in cellular immunity. Clinical presentation may be varied depending on the organs involved ranging from dermatitis; gastrointestinal manifestation in the form of ulcerations, polypoid masses; adrenal masses, adrenal insufficiency; cavitary pulmonary lesions; CNS involvement with meningitis, encephalitis, focal brain or spinal cord lesions, etc. Diagnostic tests: Histoplasma antigen, serology, fungal cultures, cytology and histopathology. Treatment: Amphotericin B is used for severe cases that require hospitalization. Itraconazole is used for mild to moderate disease or as a step down after initial treatment with Amphotericin B.
The disease may mimic conditions such as sarcoidosis and other immune mediated disorders. Therefore, a high index of suspicion and knowledge of common presentations and diagnostic tests is essential in making timely diagnosis otherwise patients may get treated with steroids or immunosuppressive agents as in this patient that may result in progression of disease.