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

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/Tables2

项目	南非^{[ 3 ]}	美国^{[ 4 ]}	越南^{[ 5 ]}	中国（台湾）^{[ 6 ]}	中国^{[ 7 ]}	中国^{[ 8 ]}	美国^{[ 9 ]}	中国^{[ 10 ]}
入组人数	21	291	57	51	106	164	145	102
年龄[岁(范围）]	38（9~72）	55（1~84）	34（15~75）	60（31~88）	37（NR）	39（NR）	57（17~89）	40（12~69）
男性比例（%）	71	61	54	63	69	72	66	71
头痛（%）	62	38	100	64	76	97	51	54
发热（%）	33	44	77	60	63	82	28	35
合并肺部受累（%）	0	36	13	NR	NR	NR	64	28
脑脊液压力> 200 mmH₂0%（n/N）	NR	平均230 mmH₂0	NR	65%（24/51）	82%（87/106）	76%（194/255）	NR	98%（100/102）> 180 mmH₂0
接受免疫抑制治疗（%）	NR	28	12	NR	14	23	47	35
自身免疫病（%）	NR	13	9	18	11	12	16	11
死亡率（%）	9	30	19	33	42	5	NR	5

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