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Review
. 2016 Aug;95(35):e4587.
doi: 10.1097/MD.0000000000004587.

Cryptococcal meningitis complicating sarcoidosis

Sonja E Leonhard  1 , Daan Fritz, Diederik van de Beek, Matthijs C Brouwer
Affiliations
Review

Cryptococcal meningitis complicating sarcoidosis

Sonja E Leonhard et al. Medicine (Baltimore). 2016 Aug.

Abstract

Background: Cryptococcal meningitis is an uncommon but severe complication of sarcoidosis.

Methods: We present 2 patients with cryptococcal meningitis complicating sarcoidosis and compared findings with 38 cases reported in the literature.

Results: When analyzing our patients and 38 cases reported in the literature, we found that median age of sarcoidosis patients with cryptococcal meningitis was 39 years (range 30-48); 27 of 33 reported cases (82%) had a history of sarcoidosis. Only 16 of 40 patients (40%) received immunomodulating therapy at the time of diagnosis of cryptococcal meningitis. The diagnosis of cryptococcal meningitis was delayed in 17 of 40 patients (43%), mainly because of the initial suspicion of neurosarcoidosis. Cerebrospinal fluid (CSF) examination showed mildly elevated white blood cell count (range 23-129/mm). Twenty-nine of 32 cases (91%) had a positive CSF culture for Cryptococcus neoformans and 25 of 27 cases (93%) had a positive CSF C neoformans antigen test. CD4 counts were low in all patients in whom counts were performed (84-228/mL). Twelve patients had an unfavorable outcome (32%), of which 7 died (19%) and 24 patients (65%) had a favorable outcome. The rate of unfavorable outcome in patients with a delayed diagnosis was 7 of 17 (41%) compared to 5 of 28 (21%) in patients in whom diagnosis was not delayed.

Conclusion: Cryptococcal meningitis is a rare but life-threatening complication of sarcoidosis. Patients were often initially misdiagnosed as neurosarcoidosis, which resulted in considerable treatment delay and worse outcome. CSF cryptococcal antigen tests are advised in patients with sarcoidosis and meningitis.

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Conflict of interest statement

Funding: financial support and conflict of interest disclosure: MCB is supported by a grant from the Netherlands Organisation for Health Research and Development (ZonMw; NWO-Veni grant 2012 [916.13.078]), DvdB is supported by grants from the Netherlands Organisation for Health Research and Development (ZonMw; NWO-Vidi grant 2010 [016.116.358]) and the European Research Council (ERC Starting Grant 281156). The authors have no conflicts of interest to disclose.

Figures

Figure 1
Figure 1
Cranial CT of case 1 showing cerebellar hemorrhage (A) and hydrocephalus (B). CT = computed tomography.
Figure 2
Figure 2
PET-CT scan of case 1 showing hilair and mediastinal lymphadenopathy. PET-CT = Positron emission tomography–computed tomography.
Figure 3
Figure 3
Flowchart of reviewed articles for cryptococcal meningitis in sarcoidosis patients.
See this image and copyright information in PMC

References

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