SESSION TYPE: Infectious Disease Cases I
PRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PM
INTRODUCTION: Sarcoidosis is characterized by impaired cell-mediated immunity that increases individuals’ risk for cryptococcal infection, especially meningitis. Because the clinical picture of cryptococcal meningitis is similar to neurosarcoidosis, a high index of clinical suspicion is needed to differentiate the two. We present a case of previously undiagnosed active sarcoidosis presenting with cryptococcal meningitis.
CASE PRESENTATION: A 42-year-old female presented after awakening with a severe headache. She reported recent worsening dyspnea, cough, weight loss and subjective fevers. She was febrile and tachycardic. On exam she had clear lungs, palpable hepatosplenomegaly, notable photophobia and neck stiffness. Non-contrast head CT was negative. Initial laboratory studies showed serum calcium of 12.2 mg/dL and an elevated ESR. Chest CT demonstrated enlarged mediastinal, retroperitoneal and bilateral hilar lymph nodes along with scattered pulmonary nodules. There was a lytic lesion of the right glenoid associated with an adjacent soft tissue mass. Further workup included an ACE level of 157 units/L. Transbronchial biopsy demonstrated fragments of benign bronchial tissue with non-caseating granulomas. CMV and HSV antigens were negative as was CSF india ink staining. Both blood and CSF cryptococcal antigen titers were positive. Biopsy culture from the glenoid soft tissue mass was also positive for cryptococcal elements.
DISCUSSION: Sarcoid specific sequestration of CD4 T-cells in sarcoid granulomas with consequent reversal of the CD4/CD8 ratio and anergy increases risk for cryptococcosis, even in steroid naïve patients. The most common presentations of cryptococcal disease associated with sarcoidosis are meningitis (42%) followed by osteomyelitis (35%). It can be challenging to differentiate neurosarcoidosis from cryptococcal meningitis as their presentations are similar including meningismus, headache, and CSF profile with modest lymphocytic pleiocytosis and slight increases in protein content. Diagnostic test of choice in HIV-negative patients is a cerebral spinal fluid cryptococcal antigen titer, which is positive in 97% of the cases. India ink preparation is less sensitive and is only positive in about 51% of the cases and less likely to be positive in HIV-negative patients.
CONCLUSIONS: Sarcoidosis is a risk factor for cryptococcal disease, even in apparently immunocompetent individuals. It can be difficult to differentiate neurosarcoidosis from cryptococcal meningitis, however, presentation with fever, meningismus and lack of cranial nerve palsy may suggest cryptococcal disease. A high degree of clinical suspicion is needed as many symptoms overlap and one should always consider cryptococcal meningitis in the setting of a sarcoidosis patient presenting with neurological abnormalities.
1) Ross, J.J. and J.D. Katz, Cryptococcal meningitis and sarcoidosis. Scand J Infect Dis, 2002. 34(12):p.937-9.
DISCLOSURE: The following authors have nothing to disclose: William Hunt, Eric Honig
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