Spontaneous intracranial hemorrhage as an initial manifestation of primary Sjögren’s syndrome: a case report

Guo-Qiang Wang,Wei-Wei Zhang

doi:10.1186/1471-2377-13-100

Guo-Qiang Wang, Wei-Wei Zhang

Open Access

https://doi.org/10.1186/1471-2377-13-100

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Journal: BMC Neurology	Publication Date: Jul 29, 2013
Citations: 4	License type: cc-by

Affiliation: The Military General Hospital of Beijing PLA

Abstract

BackgroundSjögren’s syndrome can involve the central nervous system; however, spontaneous intracranial hemorrhage has rarely been reported as the initial manifestation.Case presentationWe report a 39-year-old woman with primary Sjögren’s syndrome presenting with intracranial hemorrhage. The diagnosis of primary Sjögren’s syndrome was based on the presence of ocular dryness, salivary gland secretory and excretory dysfunction confirmed with dynamic tracer emission CT, and positive anti-Sjögren’s syndrome A and anti-Sjögren’s syndrome B antibodies.ConclusionPrimary Sjögren’s syndrome can present with variable central nervous system signs, which may precede the classic sicca symptoms. Therefore, Sjögren’s syndrome-associated indicators should be investigated in patients without the common risk factors for stroke who present with spontaneous intracranial hemorrhage.

Highlights

Sjögren’s syndrome can involve the central nervous system; spontaneous intracranial hemorrhage has rarely been reported as the initial manifestation.Case presentation: We report a 39-year-old woman with primary Sjögren’s syndrome presenting with intracranial hemorrhage
SS has a marked female preponderance with a female-to-male ratio of 13:1. It features a later age at onset, with a median age of 54 years for women and 58 years for men at the first diagnosis [3]. This syndrome is classified as primary SS in the absence of other autoimmune diseases, and as secondary SS when it is associated with other autoimmune diseases such as rheumatoid arthritis, scleroderma, systemic lupus erythematosus, AIDS, hepatitis C infection, pre-existing lymphoma sarcoidosis, graft-versus-host disease, or the use of anticholinergic drugs
We report a case of a patient with primary SS (pSS) who presented with intracranial hemorrhage (ICH)

Summary

Conclusion

Five case of pSS-associated ICH have been reported [8,9,10,11,12]. Unlike our case, all of the five reported cases had a clear history of pSS prior to the ICH, and none had ICH as the first manifestation of pSS. The elevated immunoglobulin levels in SS patients contain a number of autoantibodies, including rheumatoid factors, and antinuclear, anti-SS-A and anti-SS-B antibodies [3] The presence of these autoantibodies is closely related to the occurrence of vasculitis [14]. The diagnosis of pSS is based on the following: high titers for anti-SS-A/Ro, SS-B/La, and recombinant Ro-52 antibodies and rheumatoid factor; elevated IgG and IgA; accelerated ESR; decreased exocrine glandular function; no trace of other autoimmune or infectious disease; and the revised classification criteria for Sjögren’s syndrome proposed by the AmericanEuropean Consensus Group [21]. We hypothesized that in our patient, the pSS-associated vasculitis might have contributed to the development of the asymptomatic occlusion of the left terminal portion of the internal carotid artery and the subsequent development of moyamoya vessels, ICH, and multiple lacunar infarctions.

Background

Findings