A 42‐year‐old African American woman presented to the hospital with a 1 week history of nasal congestion, sore throat, and fever of 38.8°C. Over 12 hours, she developed acute headache, photophobia, and neck stiffness. Review of systems was positive for a 6‐month history of 50‐pound weight loss and generalized weakness. Medical history was significant for possible sarcoidosis diagnosed 4 months prior to admission. She had never been treated for this condition. She was married and worked as a phlebotomist. She denied any sick contacts, recent travel, or substance abuse. On admission, the patient appeared acutely ill. Physical exam was remarkable for neck rigidity, photophobia, positive Brudzinski's sign, hepatomegaly, and splenomegaly. Lumbar puncture was significant for an opening pressure of 30 cm H2O, and a positive cerebrospinal fluid (CSF) cryptococcal antigen titer > 1:512. Blood and CSF fungal culture grew Cryptococcus neoformans. Chest x‐ray showed a right scapular lytic lesion measuring 5.7 × 4.8 cm; biopsy revealed 8 cc of brown purulent fluid that was culture positive for Cryptococcus neoformans. Further workup was supportive of sarcoidosis as the underlying cause of the patient's immunosuppression. Chest CT revealed diffuse small pulmonary nodules as well as mediastinal and bilateral hilar lymphadenopathy. Prior biopsy results of a mediastinal lymph node showed few epithelioid noncaseating granulomas. Laboratory workup was notable for a calcium of 12.2 mg/dL, ACE 167 U/L, and albumin 2.6 g/dL. Infectious studies were negative for HIV, hepatitis B, hepatitis C, and tuberculosis. Workup for malignancy was negative, including cytology of the CSF fluid, mediastinal lymph node biopsy, and scapular abscess. The patient improved with induction treatment with intravenous amphotericin B and oral flucytosine for 4 weeks, along with serial lumbar punctures. Repeat CSF fungal culture was negative at 4 weeks, and the patient was discharged home on oral fluconazole 400 mg daily for 10 weeks.
This case highlights sarcoidosis as a rare cause of disseminated cryptococcal infection, presenting as meningitis and a destructive scapular abscess. Sarcoidosis with or without prior steroid therapy predisposes to cryptococcal infection because of deficient cell‐mediated immunity. More than 48 cases of cryptococcal infection in sarcoidosis have been reported. It commonly manifests as meningitis (42%), osteomyelitis (38%), soft‐tissue abscess (25%), pneumonia (10%), and disseminated disease (8%). In a case series of patients with cryptococcal osteomyelitis, 10 of 40 patients had concomitant sarcoidosis. Conversely, a review of 753 sarcoid patients showed only 0.4% developed cryptococcal disease, and these patients were receiving immunosuppressive therapy. Early identification and treatment is vital given that mortality may be as high as 33% even with therapy.
Clinicians should maintain a high index of suspicion for cryptococcal infection in patients with sarcoidosis.
A. Shah ‐ none; U. Abed‐Alwahab ‐ none; S. Hussen ‐ none; C. Franco‐Paredes ‐ none; A. Kho ‐ none