A rare case report of primary Sjögren’s syndrome with clinical characteristics similar to those of CLIPPERS

Background Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disease characterized by dryness of the eyes, mouth and other mucous membranes. Patients with pSS can also present with extraglandular manifestations, such as pulmonary, kidney and nervous system involvement. Central nerv...

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Bibliographic Details
Published in:BMC neurology 2022-11, Vol.22 (1), p.1-414, Article 414
Main Authors: Lv, ChenLing, Zhu, FeiYan, Chen, Chao, Wang, YunLing, Guo, DengJun, Zhang, ZhenZhong
Format: Article
Language:English
Subjects:
Antibodies
Ataxia
Autoimmune diseases
Basal ganglia
Biopsy
Blood
Bone marrow
Brain stem
Care and treatment
Case Report
Case reports
Central nervous system
Cerebrospinal fluid
CLIPPERS
Development and progression
Diagnosis
Disease
Exocrine glands
Inflammation
Inflammatory diseases
Leukopenia
Lymphocytes
Lymphoma
Magnetic resonance imaging
Nervous system
Neuroimaging
Neurological complications
Pathogenesis
Pathology
Patients
Primary Sjögren′s syndrome
Rheumatology
Salivary gland
Sjogren's syndrome
Steroid hormones
Steroids
Thrombocytopenia
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Summary:Background Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disease characterized by dryness of the eyes, mouth and other mucous membranes. Patients with pSS can also present with extraglandular manifestations, such as pulmonary, kidney and nervous system involvement. Central nervous system (CNS) manifestations have rarely been described in pSS. Case presentation A 33-year-old man was admitted with a one-month history of dizziness, speech disturbance, and walking instability. His brain enhanced magnetic resonance imaging (MRI) showed symmetrical, enhanced "salt-and-pepper-like" speckled lesions in the brainstem, basal ganglia, and subcortical regions, and his diagnosis was considered possible chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Further examination revealed that anti-SSA antibody was positive, and the Schirmer test and labial salivary gland histopathology were abnormal, which supported the diagnosis of pSS. Conclusion pSS is a chronic systemic autoimmune disease that involves neurological complications. This case suggests that CNS lesions of pSS can present with clinical and MRI findings similar to those of CLIPPERS. Keywords: Primary Sjögren's syndrome, CLIPPERS, Central nervous system, Magnetic resonance imaging
ISSN:1471-2377
1471-2377
DOI:10.1186/s12883-022-02945-2