Sjögren′s syndrome secondary to cryptococcal meningoencephalitis: a case report and literature review

doi:10.16150/j.1671-2870.2021.05.006

Abstract

Abstract:

Objective: To investigate a case of cryptococcal meningoencephalitis (CM) secondary to Sjögren′s syndrome (SS), and to analyze the clinical, pathological features, diagnosis and treatment of the diseases combined with literature review. Methods: Clinical data of 1 case of CM secondary to SS patient were collected, and literatures online were reviewed. Results: The main clinical manifestation consisted of headache, fever and intracranial hypertension. CM was confirmed by cerebrospinal fluid ink stain smear, but the patient still died of complication of amphotericin B treatment. Literature review indicated 9%-18% CM may had a history of autoimmune diseases. Headache because of increased cerebrospinal fluid pressure ocuured in 38%-100%. The efficacy of amphotericin B combined with fluorocytosine in recommended treatment was not satisfactory, and mortality was high (5%-42%). Conclusions: The clinical manifestation of CM secondary to autoimmune diseases is not typical, the abnormal increase of cerebrospinal fluid pressure is its characteristic. The efficacy of treatment is not satisfactory, and the mortality rate is high.

Key words: Sjögren′s syndrome, Autoimmune diseases, Cryptococcus neoformans, Meningoencephalitis

CLC Number:

R593.2

LU Hongyu, GU Jun, WANG Jing, CAO Yafeng, SONG Luxi, FAN Jun, CHEN Mei. Sjögren′s syndrome secondary to cryptococcal meningoencephalitis: a case report and literature review[J]. Journal of Diagnostics Concepts & Practice, 2021, 20(05): 456-461.

Figures/Tables 2

References 19

[1]	Thorne I, Sutcliffe N. Sjögren′s syndrome[J]. Br J Hosp Med (Lond), 2017,78(8):438-442. doi: 10.12968/hmed.2017.78.8.438 URL
[2]	Margaretten M. Neurologic Manifestations of Primary Sjögren Syndrome[J]. Rheum Dis Clin North Am, 2017,43(4):519-529. doi: 10.1016/j.rdc.2017.06.002 URL
[3]	Moosa MY, Coovadia YM. Cryptococcal meningitis in Durban, South Africa: a comparison of clinical features, laboratory findings, and outcome for human immunodeficiency virus (HIV)-positive and HIV-negative patients[J]. Clin Infect Dis, 1997,24(2):131-134. pmid: 9114135
[4]	Pappas PG, Perfect JR, Cloud GA, et al. Cryptococcosis in human immunodeficiency virus-negative patients in the era of effective azole therapy[J]. Clin Infect Dis, 2001, 33(5):690-699. pmid: 11477526
[5]	Chau TT, Mai NH, Phu NH, et al. A prospective descriptive study of cryptococcal meningitis in HIV uninfected patients in Vietnam - high prevalence of Cryptococcus neoformans var grubii in the absence of underlying di-sease[J]. BMC Infect Dis, 2010,10:199. doi: 10.1186/1471-2334-10-199 URL
[6]	Lee YC, Wang JT, Sun HY, et al. Comparisons of clinical features and mortality of cryptococcal meningitis between patients with and without human immunodeficiency virus infection[J]. J Microbiol Immunol Infect, 201144(5):338-345.
[7]	Zheng H, Chen Q, Xie Z, et al. A retrospective research of HIV-negative cryptococcal meningoencephalitis patients with acute/subacute onset[J]. Eur J Clin Microbiol Infect Dis, 2016,35(2):299-303. doi: 10.1007/s10096-015-2545-0 URL
[8]	Li M, Chen Z, Xu L, et al. A Comparison of the clinical characteristics and outcomes of cryptococcal meningitis in HIV-negative individuals with and without immunosuppression[J]. Neurologist, 2019,24(1):1-5. doi: 10.1097/NRL.0000000000000221 URL
[9]	Marr KA, Sun Y, Spec A, et al. A multicenter, longitu-dinal cohort study of cryptococcosis in human immuno-deficiency virus-negative people in the United States[J]. Clin Infect Dis, 2020,70(2):252-261. doi: 10.1093/cid/ciz193 URL
[10]	常艳宇, 胡学强. 新型隐球菌性脑膜炎和(或)脑炎102例诊断与治疗经验[J]. 中国现代神经疾病杂志, 2014,14(8):687-692.
[11]	Beardsley J, Hoang NLT, Kibengo FM, et al. Do intrace-rebral cytokine responses explain the harmful effects of dexamethasone in human immunodeficiency virus-associated cryptococcal meningitis?[J]. Clin Infect Dis, 2019,68(9):1494-1501. doi: 10.1093/cid/ciy725 pmid: 30169607
[12]	Boulware DR, Rolfes MA, Rajasingham R, et al. Multisite validation of cryptococcal antigen lateral flow assay and quantification by laser thermal contrast[J]. Emerg Infect Dis, 2014,20(1):45-53. doi: 10.3201/eid2001.130906 pmid: 24378231
[13]	Perfect JR, Dismukes WE, Dromer F, et al. Clinical practice guidelines for the management of cryptococcal disease: 2010 update by the infectious diseases society of america[J]. Clin Infect Dis, 2010,50(3):291-322. doi: 10.1086/649858 pmid: 20047480
[14]	刘正印, 王贵强, 朱利平, 等. 隐球菌性脑膜炎诊治专家共识[J]. 中华内科杂志, 2018,57(5):317-323.
[15]	Tseng HK, Liu CP, Ho MW, et al. Microbiological, epidemiological, and clinical characteristics and outcomes of patients with cryptococcosis in Taiwan, 1997-2010[J]. PLoS One,2013, 8(4):e61921. doi: 10.1371/journal.pone.0061921 URL
[16]	Pappas PG. Cryptococcal infections in non-HIV-infected patients[J]. Trans Am Clin Climatol Assoc, 2013,124:61-79. pmid: 23874010
[17]	Fang W, Chen M, Liu J, et al. Cryptococcal meningitis in systemic lupus erythematosus patients: pooled analysis and systematic review[J]. Emerg Microbes Infect, 2016, 5(9):e95.
[18]	Dixon WG, Abrahamowicz M, Beauchamp ME, et al. Immediate and delayed impact of oral glucocorticoid therapy on risk of serious infection in older patients with rheumatoid arthritis: a nested case-control analysis[J]. Ann Rheum Dis, 2012,71(7):1128-1133. doi: 10.1136/annrheumdis-2011-200702 URL
[19]	Liao TL, Chen YM, Chen DY. Risk factors for cryptococcal infection among patients with rheumatoid arthritis receiving different immunosuppressive medications[J]. Clin Microbiol Infect, 2016,22(9):815,e1.