scholarly journals Intravenous Immunoglobulin and Mycophenolate Mofetil for Long-Standing Sensory Neuronopathy in Sjögren's Syndrome

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Maria Giovanna Danieli ◽  
Lucia Pettinari ◽  
Ramona Morariu ◽  
Fernando Monteforte ◽  
Francesco Logullo
Keyword(s):  
Mycophenolate Mofetil ◽  
Intravenous Immunoglobulin ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Lower Limbs ◽  
High Dose ◽  
Sensory Neuronopathy ◽  
Sjögren‘S Syndrome

Sensory neuronopathy is described in association with the Sjögren's syndrome (SS). We studied a 55-year-old woman with a 4-year history of progressive asymmetric numbness, distal tingling, and burning sensation in upper and lower limbs. In a few months, she developed ataxia with increased hypoanaesthesia. Electrodiagnostic tests revealed undetectable distal and proximal sensory nerve action potential in upper and lower limbs. Cervical spine magnetic resonance showed a signal hyperintensity of posterior columns. Previous treatment with high-dose glucocorticoids and azathioprine was ineffective. A combined treatment with intravenous immunoglobulin and mycophenolate mofetil was followed by a progressive and persistent improvement. This case documented the efficacy and the safety of the coadministration of intravenous immunoglobulin and mycophenolate mofetil in sensory neuronopathy associated with SS refractory to conventional immunosuppressive therapy.

Benefit of IVIg for long-standing ataxic sensory neuronopathy with Sjogren's syndrome

Neurology ◽  
2003 ◽  
Vol 61 (6) ◽  
pp. 873-873 ◽  
Author(s):  
T. M. Burns ◽  
S. Quijano-Roy ◽  
H. R. Jones ◽  
G. I. Wolfe ◽  
S. P. Nations ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sensory Neuronopathy ◽  
Sjögren‘S Syndrome

scholarly journals Sjögren’s syndrome with rapidly progressive motor neuron disease: a case report

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052097446 ◽  
Author(s):  
Huijia Yang ◽  
Xiaozhong Jing ◽  
Jinhua Yan ◽  
Dihui Ma
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Lower Limbs ◽  
Weekly Dose ◽  
Limb Weakness ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome is an autoimmune disease that can affect multiple systems. Sjögren’s syndrome with motor neuron disease is rarely reported. Herein, we describe a patient with rapidly progressive motor neuron disease secondary to Sjögren’s syndrome. A 42-year-old woman was admitted to our hospital with a 2-month history of progressive limb weakness. Neurological assessment revealed fasciculation in the lower limbs and amyotrophy in the bilateral supraspinatus, interosseous, and thenar muscles. Serological examination and labial gland biopsy revealed Sjögren’s syndrome. In addition, electromyography demonstrated neurogenic damage to the upper and lower limbs. The patient received a short course of high-dose corticosteroids, intravenous immunoglobulins, and immunosuppressant treatment, including a weekly dose of 0.4 g cyclophosphamide and a daily dose of 0.2 g hydroxychloroquine. However, the patient’s limb weakness was further aggravated and her respiratory function was compromised. Electromyography re-examination demonstrated extensive neurogenic damage, and she was diagnosed with Sjögren’s syndrome with motor neuron disease. The patient died of respiratory failure after 2 months. We suggest that more effective maintenance treatments should be sought. Further investigation is required to elucidate the association between autoimmune motor neuron disease and Sjögren’s syndrome.

scholarly journals Chronic hepatitis E in a patient treated with rituximab and mycophenolate mofetil for Sjögren’s syndrome

Rheumatology ◽  
2016 ◽  
Vol 55 (12) ◽  
pp. 2275-2277 ◽  
Author(s):  
Paolo Fraticelli ◽  
Patrizia Bagnarelli ◽  
Giuseppe Tarantino ◽  
Giuseppe Pio Martino ◽  
Devis Benfaremo ◽  
...  
Keyword(s):  
Chronic Hepatitis ◽  
Mycophenolate Mofetil ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Hepatitis E ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

Benefit of IVIG for long-standing ataxic sensory neuronopathy with Sjogren's syndrome

Neurology ◽  
2003 ◽  
Vol 60 (3) ◽  
pp. 503-505 ◽  
Author(s):  
Y. Takahashi ◽  
T. Takata ◽  
M. Hoshino ◽  
M. Sakurai ◽  
I. Kanazawa
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sensory Neuronopathy ◽  
Sjögren‘S Syndrome

scholarly journals HTLV-I Associated uveitis, myelopathy, rheumatoid arthritis and Sjögren's syndrome

1995 ◽  
Vol 53 (4) ◽  
pp. 777-781 ◽  
Author(s):  
Sônia Regina A. A. Pinheiro ◽  
Marco Aurélio Lana-Peixoto ◽  
Anna B. Carneiro Proietti ◽  
Fernando Oréfice ◽  
Maria V. Lima-Martins ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Spastic Paraparesis ◽  
Clinical Manifestations ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Lower Limbs ◽  
Type I ◽  
Human T Cell ◽  
Sjögren‘S Syndrome

A 62 year-old white female presented with a 10-year-history of slowly progressive spastic paraparesis, pain and dysesthesia in the lower limbs and sphincter disturbance. A few years after the onset of the neurologic symptoms she developped migratory arthritis with swelling of the knees and pain on palpation of knees and fingers, dry eyes, mouth and skin. Two months before admission she presented bilateral nongranulomatous anterior uveitis. Examination revealed spastic paraparesis with bilateral Babinski sign, a decreased sensation level below L3, decreased vibration sense in the lower extremities, and a postural tremor of the upper limbs. Laboratory work-up disclosed HTLV-I positive tests in the blood and cerebrospinal fluid (CSF), and a mild pleocytosis in the CSF with a normal protein content. Nerve conduction velocity studies were normal. The present case shows the association of uveitis, arthritis and Sjögren's syndrome in a patient with tropical spastic paraparesis / human T-cell lymphotropic virus type I (HTLV-I) associated myelopathy (TSP/HAM), and illustrates the wide spectrum of clinical manifestations which may accompany this infection with this virus.

Rare case of Sjögren’s syndrome antibody (SSA-Ro52kDA)-associated interstitial lung disease and myositis with pipe stem capillaries

2021 ◽  
Vol 14 (10) ◽  
pp. e244133
Author(s):  
Anitha Ramu ◽  
Navneet Kaur ◽  
Ruchi Jain
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Inflammatory Myopathy ◽  
Elevated Serum ◽  
Membrane Attack Complex ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
High Dose ◽  
Mortality And Morbidity ◽  
Sjögren‘S Syndrome

Necrotising myopathy with pipestem capillaries is a distinct form of inflammatory myopathy exhibiting only sparse inflammation on biopsy, with clinical presentation and histopathological profile entirely different from dermatomyositis, polymyositis or inclusion body myositis. A 51-year-old non-diabetic man presents with progressively worsening shortness of breath and myalgias with only mild proximal muscle weakness and elevated serum creatine kinase. Autoimmune workup, ordered after ruling out infectious and cardiac aetiologies, returned positive for Sjögren’s syndrome antibody (SSA/Ro-52). Lung imaging and biopsy were suggestive of cryptogenic organising pneumonia and muscle biopsy showed myositis with pipestem capillaries and abnormal deposition of membrane attack complex with only sparse inflammation. The patient received high-dose steroids, mycophenolate mofetil, intravenous immunoglobulin and rituximab with improvement in muscle symptoms. However, his pulmonary findings progressed, requiring evaluation for a lung transplant. This case emphasises the need for further research to better understand this disease entity and improve mortality and morbidity in these patients.

scholarly journals A case of ataxic sensory neuronopathy associated with Sjögren’s syndrome

2014 ◽  
Vol 54 (5) ◽  
pp. 413-416
Author(s):  
Masanori Sawamura ◽  
Wataru Oishi ◽  
Etsuro Nakanishi ◽  
Shinichiro Maruhama ◽  
Gan Kim ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sensory Neuronopathy ◽  
Sjögren‘S Syndrome
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