Nervous system manifestations in Sjögren's syndrome

2009 ◽  
Vol 81 (2) ◽  
pp. 144-152 ◽  
Author(s):  
A. Hietaharju ◽  
U. Yli-Kerttula ◽  
V. Häkkinen ◽  
H. Frey
Keyword(s):  
Nervous System ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

scholarly journals Sjögren’s syndrome with nervous system injury combined with pulmonary and osseous cryptococcosis: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Liping Xu ◽  
Xinwei Song ◽  
Yan Zhang ◽  
Na Lin ◽  
Ji-An Wang
Keyword(s):  
Computed Tomography ◽  
Nervous System ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Resonance Imaging ◽  
Computed Tomography Scan ◽  
Tomography Scan ◽  
Sjögren‘S Syndrome

Abstract Background Sjögren’s syndrome is a common autoimmune disease that can involve the nervous system, but rarely both the central and peripheral. Long-term use of high-dose corticosteroids and immunosuppressants are the main risk factors for Cryptococcus infection in patients with Sjögren’s syndrome, of which pulmonary infection is the most common, while multiple bone infections are rare. Case presentation A 46-year-old Chinese woman with a 2-year history of Sjögren’s syndrome presented to our hospital with numbness of limbs, shortness of breath, and weakness. Blood immunochemistry showed that antinuclear antibody (1:640), anti-Sjögren’s syndrome-A antibodies, and anti-centromere antibodies were strongly positive. Cranial magnetic resonance imaging revealed multiple demyelinating lesions in the white matter of bilateral cerebral hemispheres. Electromyography indicated serious peripheral nerve injury, especially in lower limbs. Computed tomography scan of lumbar vertebral displayed multiple high-density shadows, and the corresponding vertebrae on magnetic resonance imaging showed abnormal low signal intensity on T1 and T2 sequences. Positron emission tomography–computed tomography showed multiple lesions with high 18F-fluorodeoxyglucose uptake in lung and vertebral bodies. Both lung and bone biopsies suggested Cryptococcus infection, with the diagnosis of Sjögren’s syndrome with nervous system injury combined pulmonary and osseous cryptococcosis. She took a reduced dose of prednisone about 10 mg/day, terminated mycophenolate mofetil, and began to take immunoglobulin of 0.4 g/kg/day intravenously for 5 days, fluconazole (400 mg/day) for 6 months. Within 3 weeks, her chest radiography showed a marked improvement, and 3 months later, the pulmonary lesions disappeared on her computed tomography scan. Conclusions This case exhibits an extremely rare condition of neural involvement in Sjögren’s syndrome combined with pulmonary and osseous cryptococcosis. This report also highlights the crucial role of detailed clinical examination, serologic markers, and biopsy in avoiding misdiagnosis. Currently, there is no guideline for this situation; in this case, we controlled the disease successfully with antifungal drugs and adequate gamma globulin, followed by an appropriate dose of corticosteroids.

Diagnosis and management of central nervous system Sjögren's syndrome

2020 ◽  
pp. 189-209
Author(s):  
Pantelis P. Pavlakis ◽  
Theresa Lawrence–Ford ◽  
Shalini Mahajan ◽  
Janet Lewis ◽  
Arun Varadhachary ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Diagnosis And Management ◽  
Sjögren‘S Syndrome

scholarly journals Primary peripheral T-cell central nervous system lymphoma

2021 ◽  
Vol 12 ◽  
pp. 465
Author(s):  
Cylaina E. Bird ◽  
Jeffrey I. Traylor ◽  
Jenna Thomas ◽  
James P. Caruso ◽  
Benjamin Kafka ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
T Cell ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Central Nervous System Lymphoma ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
History Of ◽  
Sjögren‘S Syndrome

Background: Primary peripheral T-cell central nervous system lymphoma (PCNSL) is a rare, aggressive tumor that arises in the craniospinal axis and has an increased risk in individuals who are immunocompromised. This lesion often mimics other benign and malignant processes on radiographic imaging, leading to misdiagnosis and delays in treatment. We present a case of a patient with a history of Sjögren’s syndrome and progressive neurologic symptoms who underwent craniotomy for diagnosis. Case Description: A 61-year-old woman with a history of Sjögren’s syndrome, progressive aphasia, left facial droop, and right-sided paresthesias for 4 months presented for evaluation and management. An enhancing, infiltrative lesion in the left frontal lobe with underlying vasogenic edema was appreciated and suggestive of a primary or metastatic neoplasm. The patient underwent an open biopsy for further evaluation of the lesion. Extensive histopathologic evaluation revealed a diagnosis of T-cell PCNSL. The patient was started on induction methotrexate and temozolomide followed by consolidative radiotherapy. Conclusion: Autoimmune conditions are a risk factor for T-cell PCNSL development. T-cell PCNSL has radiographic and gross histologic features that are consistent with a broad differential, including gliomas and inflammatory processes. Prompt diagnosis and extensive histopathological evaluation is essential to ensure appropriate treatment.

Clinical features and outcomes from the Singapore Sjögren's syndrome study

Lupus ◽  
2020 ◽  
pp. 096120332097693
Author(s):  
Jeggrey Kay-Wee Kam ◽  
Natasha Charan ◽  
Rachel Wei-Li Leong ◽  
Zhi-Wen Loh ◽  
Bernard Yu-Hor Thong
Keyword(s):  
Nervous System ◽  
Peripheral Neuropathy ◽  
Sjögren’S Syndrome ◽  
Clinical Features ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Mononeuritis Multiplex ◽  
Glandular Enlargement ◽  
Sjögren‘S Syndrome

Objective To study the clinical features, treatment and outcomes of primary Sjögren's Syndrome (pSS) in a Singapore cohort from an outpatient rheumatology clinic. Methods Computerised Physician Order entry records of patients who fulfilled the 2016 ACR-EULAR classification criteria for pSS between 1993 and 2013 were retrospectively analysed. Results There were 102 patients, of which 96 (94.1%) were females, and 91 (89.2%) Chinese. Mean age at diagnosis was 49.3 ± 11.8 years, mean disease duration was 9.0 ± 4.6 years. The most common manifestations were keratoconjunctivitis sicca (99.0%), xerostomia (96.1%), arthralgia/arthritis (56.9%). Exocrine glandular enlargement comprised parotidomegaly (28, 27.5%), with concurrent submandibular and lacrimal gland enlargement in one. The nervous system (15.7%) was the most commonly affected internal organ, with peripheral nervous system (peripheral neuropathy, mononeuritis multiplex) involvement more common than central. Hydroxychloroquine was most frequently used (88.2%), followed by methotrexate (7.8%) and azathioprine (6.9%). Pulsed intravenous (IV) methylprednisolone 500 mg/day for 3 days was used in 5 patients followed by oral (4) or IV cyclophosphamide (1) for cardiomyopathy and interstitial lung disease (1), and neurological involvement (4). These comprised neuromyelitis optica, transverse myelopathy, cranial neuropathy, mononeuritis multiplex and/or peripheral neuropathy alone or in combination. Intravenous immunoglobulins (2.0%) was used for sensory neuropathy and mononeuritis multiplex; rituximab (1.0%) in 1 patient for treatment of non-Hodgkin’s B-cell lymphoma. There were no deaths. Conclusion Musculoskeletal manifestations were common, with the nervous system (peripheral more than central) the most common internal organ involved. Lymphoma was uncommon despite up to one-third of the cohort developing glandular enlargement.

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