Peripheral Nervous System Involvement in Sjögren’s Syndrome: Analysis of a Cohort From the Italian Research Group on Sjögren’s Syndrome

PurposeThe prevalence of peripheral nervous system (PNS) involvement in primary Sjögren’s syndrome (pSS) has been reported to range from 2% to over 50%. Bias in study designs, including low number of patients and unclearly defined rheumatological and neurological diagnosis could explain such variability. Consequently, the exact depiction of PNS involvement in pSS is still lacking. This study aimed at analyzing the prevalence and the clinical and laboratory factors associated with PNS involvement in a very large cohort of well-characterized pSS patients with a clearly defined neurological diagnosis.MethodsClinical and serological data of 1,695 pSS patients with specific and accurate information on PNS involvement were analyzed. Comparisons between patients with and without PNS involvement and between patients with distinct subsets of PNS manifestations were performed.ResultsPrevalence of PNS involvement was 3.7%. The most frequent types observed were pure sensory neuropathies and axonal sensorimotor polyneuropathies (SMP). Patients with PNS involvement exhibited a more active disease profile and were more frequently treated with immunosuppressants. Intriguingly, clinical and serological negative prognostic factors, including purpura, extra-glandular manifestations, leukopenia, low complement and cryoglobulinemia, principally characterized patients with SMP, while subjects with pure sensory neuropathy displayed a milder phenotype.ConclusionOur results highlight that PNS involvement is rather rare, but prognostically relevant in pSS. Main adverse prognostic features characterize patients with SMP, while pure sensory neuropathies are usually associated with a mild clinical picture. These findings, useful for patient stratification, may suggest protean pathogenic pathways underlying different types of PNS manifestations in pSS.

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Peripheral nervous system in primary Sjögren’s syndrome

Reumatologia/Rheumatology ◽

10.5114/reum.2013.35771 ◽

2013 ◽

Vol 3 ◽

pp. 202-209

Author(s):

Żaneta Smoleńska ◽

Małgorzata Bilińska ◽

Hanna Kujawska-Danecka ◽

Zbigniew Zdrojewski

Keyword(s):

Nervous System ◽

Peripheral Nervous System ◽

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Primary Sjögren’S Syndrome ◽

Primary Sjogren’S Syndrome ◽

Primary Sjögren's Syndrome ◽

Sjögren‘S Syndrome

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NEUROLOGIC DISEASE IN SJOGREN’S SYNDROME: MONONUCLEAR INFLAMMATORY VASCULOPATHY AFFECTING CENTRAL/PERIPHERAL NERVOUS SYSTEM AND MUSCLE

Rheumatic Disease Clinics of North America ◽

10.1016/s0889-857x(21)00211-8 ◽

1993 ◽

Vol 19 (4) ◽

pp. 869-908

Author(s):

Elaine L. Alexander

Keyword(s):

Nervous System ◽

Peripheral Nervous System ◽

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Neurologic Disease ◽

Sjögren‘S Syndrome

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

Journal of Medical Case Reports ◽

10.1186/s13256-021-02941-z ◽

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.

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