Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases
Primary Sjögren syndrome (SS) is an autoimmune disease mainly affecting the exocrine glands causing a sicca syndrome. Neurological manifestations are rarely seen in SS although they are debilitating. Peripheral neuropathies namely sensory axonal neuropathy and painful small fiber neuropathy are the...
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description | Primary Sjögren syndrome (SS) is an autoimmune disease mainly affecting the exocrine glands causing a sicca syndrome. Neurological manifestations are rarely seen in SS although they are debilitating. Peripheral neuropathies namely sensory axonal neuropathy and painful small fiber neuropathy are the most frequent neurological manifestations. Sensory neuronopathy (SN) is less frequently seen although leading to more severe handicap.The aim of the study was to analyze the clinical presentation and treatment efficacy in a series of SS-related SN.We retrospectively studied patients with SS fulfilling the American-European Classification Criteria and SN according to recent criteria. Studied variables were neurological findings, associated autoimmune diseases, biological profiles, nerve conduction and sensory/motor amplitudes study, treatments received, and outcomes. Handicap scores were studied at beginning and end of each treatment using the modified Rankin Scale (mRS).Thirteen patients were included (12 women, 1 man; median age 55 years at SN diagnosis) presenting with SN with a median follow-up of 3 years (range 2-17). In 11 patients, SN preceded or coincided with SS diagnosis. Most common neurological findings were ataxia and areflexia followed by paresthesia and pain. Lower limbs were more affected than upper limbs, neurological deficits were often symmetric and cranial nerves were affected in 3 patients. Seven patients were treated with corticosteroids, 7 with mycophenolate mofetil, 6 with hydroxychloroquine, 5 with intravenous immunoglobulins, 4 with cyclophosphamide, and 2 patients received other immunosuppressive drugs. At the beginning and at the end of follow-up, average mRS was 2.15 (median 2) and 2.38 (median 2), respectively.SS-related SN progression is heterogeneous but tends to be chronic, insidious, and debilitating despite treatment. From these data concerning a small number of patients, treatment strategies with corticosteroids in association with immunosuppressive drugs, namely mycophenolate mofetil, had positive results. In contrast, intravenous immunoglobulins had disappointing results. |
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Ricardo ; Viala, Karine ; Maisonobe, Thierry ; Haroche, Julien ; Mathian, Alexis ; Hié, Miguel ; Amoura, Zahir ; Cohen Aubart, Fleur</creator><creatorcontrib>Pereira, P. Ricardo ; Viala, Karine ; Maisonobe, Thierry ; Haroche, Julien ; Mathian, Alexis ; Hié, Miguel ; Amoura, Zahir ; Cohen Aubart, Fleur</creatorcontrib><description>Primary Sjögren syndrome (SS) is an autoimmune disease mainly affecting the exocrine glands causing a sicca syndrome. Neurological manifestations are rarely seen in SS although they are debilitating. Peripheral neuropathies namely sensory axonal neuropathy and painful small fiber neuropathy are the most frequent neurological manifestations. Sensory neuronopathy (SN) is less frequently seen although leading to more severe handicap.The aim of the study was to analyze the clinical presentation and treatment efficacy in a series of SS-related SN.We retrospectively studied patients with SS fulfilling the American-European Classification Criteria and SN according to recent criteria. Studied variables were neurological findings, associated autoimmune diseases, biological profiles, nerve conduction and sensory/motor amplitudes study, treatments received, and outcomes. Handicap scores were studied at beginning and end of each treatment using the modified Rankin Scale (mRS).Thirteen patients were included (12 women, 1 man; median age 55 years at SN diagnosis) presenting with SN with a median follow-up of 3 years (range 2-17). In 11 patients, SN preceded or coincided with SS diagnosis. Most common neurological findings were ataxia and areflexia followed by paresthesia and pain. Lower limbs were more affected than upper limbs, neurological deficits were often symmetric and cranial nerves were affected in 3 patients. Seven patients were treated with corticosteroids, 7 with mycophenolate mofetil, 6 with hydroxychloroquine, 5 with intravenous immunoglobulins, 4 with cyclophosphamide, and 2 patients received other immunosuppressive drugs. At the beginning and at the end of follow-up, average mRS was 2.15 (median 2) and 2.38 (median 2), respectively.SS-related SN progression is heterogeneous but tends to be chronic, insidious, and debilitating despite treatment. From these data concerning a small number of patients, treatment strategies with corticosteroids in association with immunosuppressive drugs, namely mycophenolate mofetil, had positive results. In contrast, intravenous immunoglobulins had disappointing results.</description><identifier>ISSN: 0025-7974</identifier><identifier>EISSN: 1536-5964</identifier><identifier>DOI: 10.1097/MD.0000000000003632</identifier><identifier>PMID: 27175675</identifier><language>eng</language><publisher>United States: The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved</publisher><subject>Adrenal Cortex Hormones - therapeutic use ; Adult ; Aged ; Cyclophosphamide - therapeutic use ; Disability Evaluation ; Enzyme Inhibitors - therapeutic use ; Female ; Follow-Up Studies ; Humans ; Hydroxychloroquine - therapeutic use ; Immunoglobulins, Intravenous - therapeutic use ; Immunologic Factors - therapeutic use ; Male ; Middle Aged ; Mycophenolic Acid - therapeutic use ; Observational Study ; Peripheral Nervous System Diseases - drug therapy ; Peripheral Nervous System Diseases - etiology ; Retrospective Studies ; Sensory Receptor Cells ; Sjogren's Syndrome - complications ; Treatment Outcome ; Young Adult</subject><ispartof>Medicine (Baltimore), 2016-05, Vol.95 (19), p.e3632-e3632</ispartof><rights>The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.</rights><rights>Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. 2016</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c3554-fe524c01ff439044461420bdb9e73f1601b15f4d9e074b0c6c0f3703a28bbdda3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4902517/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4902517/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,864,885,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27175675$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Pereira, P. Ricardo</creatorcontrib><creatorcontrib>Viala, Karine</creatorcontrib><creatorcontrib>Maisonobe, Thierry</creatorcontrib><creatorcontrib>Haroche, Julien</creatorcontrib><creatorcontrib>Mathian, Alexis</creatorcontrib><creatorcontrib>Hié, Miguel</creatorcontrib><creatorcontrib>Amoura, Zahir</creatorcontrib><creatorcontrib>Cohen Aubart, Fleur</creatorcontrib><title>Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases</title><title>Medicine (Baltimore)</title><addtitle>Medicine (Baltimore)</addtitle><description>Primary Sjögren syndrome (SS) is an autoimmune disease mainly affecting the exocrine glands causing a sicca syndrome. Neurological manifestations are rarely seen in SS although they are debilitating. Peripheral neuropathies namely sensory axonal neuropathy and painful small fiber neuropathy are the most frequent neurological manifestations. Sensory neuronopathy (SN) is less frequently seen although leading to more severe handicap.The aim of the study was to analyze the clinical presentation and treatment efficacy in a series of SS-related SN.We retrospectively studied patients with SS fulfilling the American-European Classification Criteria and SN according to recent criteria. Studied variables were neurological findings, associated autoimmune diseases, biological profiles, nerve conduction and sensory/motor amplitudes study, treatments received, and outcomes. Handicap scores were studied at beginning and end of each treatment using the modified Rankin Scale (mRS).Thirteen patients were included (12 women, 1 man; median age 55 years at SN diagnosis) presenting with SN with a median follow-up of 3 years (range 2-17). In 11 patients, SN preceded or coincided with SS diagnosis. Most common neurological findings were ataxia and areflexia followed by paresthesia and pain. Lower limbs were more affected than upper limbs, neurological deficits were often symmetric and cranial nerves were affected in 3 patients. Seven patients were treated with corticosteroids, 7 with mycophenolate mofetil, 6 with hydroxychloroquine, 5 with intravenous immunoglobulins, 4 with cyclophosphamide, and 2 patients received other immunosuppressive drugs. At the beginning and at the end of follow-up, average mRS was 2.15 (median 2) and 2.38 (median 2), respectively.SS-related SN progression is heterogeneous but tends to be chronic, insidious, and debilitating despite treatment. From these data concerning a small number of patients, treatment strategies with corticosteroids in association with immunosuppressive drugs, namely mycophenolate mofetil, had positive results. In contrast, intravenous immunoglobulins had disappointing results.</description><subject>Adrenal Cortex Hormones - therapeutic use</subject><subject>Adult</subject><subject>Aged</subject><subject>Cyclophosphamide - therapeutic use</subject><subject>Disability Evaluation</subject><subject>Enzyme Inhibitors - therapeutic use</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Humans</subject><subject>Hydroxychloroquine - therapeutic use</subject><subject>Immunoglobulins, Intravenous - therapeutic use</subject><subject>Immunologic Factors - therapeutic use</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Mycophenolic Acid - therapeutic use</subject><subject>Observational Study</subject><subject>Peripheral Nervous System Diseases - drug therapy</subject><subject>Peripheral Nervous System Diseases - etiology</subject><subject>Retrospective Studies</subject><subject>Sensory Receptor Cells</subject><subject>Sjogren's Syndrome - complications</subject><subject>Treatment Outcome</subject><subject>Young Adult</subject><issn>0025-7974</issn><issn>1536-5964</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2016</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkd1O3DAQha0KVJafJ6hU-ZJeBMZ_MelFpWqhUGkpEizXlpOMdwOJvbKzoH0xXqAvRuiWFeAbSz5zvhmfIeQLgyMGhT6-PD2CN0fkgn8iI6ZEnqkil1tkBMBVpgstd8huSncATGguP5MdrplWuVYjsrq5-_s0i-jpDfoU4or-wWUMPixsP1_Rw418bv2sbV6Fb9_pJPhZNsXY0atlX4UOqfU1nUa0fYe-p9eYFsEnpI2ndqDHBhMNjjJBxzZh2ifbzrYJD_7fe-T219l0fJFNrs5_j39OskooJTOHissKmHNSFCClzJnkUNZlgVo4lgMrmXKyLhC0LKHKK3BCg7D8pCzr2oo98mPNXSzLDutqmC3a1ixi09m4MsE25r3im7mZhQcjiyE-pgeAWAOqGFKK6DZeBuZlE-by1HzcxOD6-rbtxvMa_VAg1wWPoe0xpvt2-YjRzNG2_fwfT-mCZxyGPyoGkL28SPEMTGaWeg</recordid><startdate>20160501</startdate><enddate>20160501</enddate><creator>Pereira, P. Ricardo</creator><creator>Viala, Karine</creator><creator>Maisonobe, Thierry</creator><creator>Haroche, Julien</creator><creator>Mathian, Alexis</creator><creator>Hié, Miguel</creator><creator>Amoura, Zahir</creator><creator>Cohen Aubart, Fleur</creator><general>The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved</general><general>Wolters Kluwer Health</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>5PM</scope></search><sort><creationdate>20160501</creationdate><title>Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases</title><author>Pereira, P. Ricardo ; Viala, Karine ; Maisonobe, Thierry ; Haroche, Julien ; Mathian, Alexis ; Hié, Miguel ; Amoura, Zahir ; Cohen Aubart, Fleur</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3554-fe524c01ff439044461420bdb9e73f1601b15f4d9e074b0c6c0f3703a28bbdda3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2016</creationdate><topic>Adrenal Cortex Hormones - therapeutic use</topic><topic>Adult</topic><topic>Aged</topic><topic>Cyclophosphamide - therapeutic use</topic><topic>Disability Evaluation</topic><topic>Enzyme Inhibitors - therapeutic use</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Humans</topic><topic>Hydroxychloroquine - therapeutic use</topic><topic>Immunoglobulins, Intravenous - therapeutic use</topic><topic>Immunologic Factors - therapeutic use</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Mycophenolic Acid - therapeutic use</topic><topic>Observational Study</topic><topic>Peripheral Nervous System Diseases - drug therapy</topic><topic>Peripheral Nervous System Diseases - etiology</topic><topic>Retrospective Studies</topic><topic>Sensory Receptor Cells</topic><topic>Sjogren's Syndrome - complications</topic><topic>Treatment Outcome</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Pereira, P. Ricardo</creatorcontrib><creatorcontrib>Viala, Karine</creatorcontrib><creatorcontrib>Maisonobe, Thierry</creatorcontrib><creatorcontrib>Haroche, Julien</creatorcontrib><creatorcontrib>Mathian, Alexis</creatorcontrib><creatorcontrib>Hié, Miguel</creatorcontrib><creatorcontrib>Amoura, Zahir</creatorcontrib><creatorcontrib>Cohen Aubart, Fleur</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Medicine (Baltimore)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Pereira, P. Ricardo</au><au>Viala, Karine</au><au>Maisonobe, Thierry</au><au>Haroche, Julien</au><au>Mathian, Alexis</au><au>Hié, Miguel</au><au>Amoura, Zahir</au><au>Cohen Aubart, Fleur</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases</atitle><jtitle>Medicine (Baltimore)</jtitle><addtitle>Medicine (Baltimore)</addtitle><date>2016-05-01</date><risdate>2016</risdate><volume>95</volume><issue>19</issue><spage>e3632</spage><epage>e3632</epage><pages>e3632-e3632</pages><issn>0025-7974</issn><eissn>1536-5964</eissn><abstract>Primary Sjögren syndrome (SS) is an autoimmune disease mainly affecting the exocrine glands causing a sicca syndrome. Neurological manifestations are rarely seen in SS although they are debilitating. Peripheral neuropathies namely sensory axonal neuropathy and painful small fiber neuropathy are the most frequent neurological manifestations. Sensory neuronopathy (SN) is less frequently seen although leading to more severe handicap.The aim of the study was to analyze the clinical presentation and treatment efficacy in a series of SS-related SN.We retrospectively studied patients with SS fulfilling the American-European Classification Criteria and SN according to recent criteria. Studied variables were neurological findings, associated autoimmune diseases, biological profiles, nerve conduction and sensory/motor amplitudes study, treatments received, and outcomes. Handicap scores were studied at beginning and end of each treatment using the modified Rankin Scale (mRS).Thirteen patients were included (12 women, 1 man; median age 55 years at SN diagnosis) presenting with SN with a median follow-up of 3 years (range 2-17). In 11 patients, SN preceded or coincided with SS diagnosis. Most common neurological findings were ataxia and areflexia followed by paresthesia and pain. Lower limbs were more affected than upper limbs, neurological deficits were often symmetric and cranial nerves were affected in 3 patients. Seven patients were treated with corticosteroids, 7 with mycophenolate mofetil, 6 with hydroxychloroquine, 5 with intravenous immunoglobulins, 4 with cyclophosphamide, and 2 patients received other immunosuppressive drugs. At the beginning and at the end of follow-up, average mRS was 2.15 (median 2) and 2.38 (median 2), respectively.SS-related SN progression is heterogeneous but tends to be chronic, insidious, and debilitating despite treatment. From these data concerning a small number of patients, treatment strategies with corticosteroids in association with immunosuppressive drugs, namely mycophenolate mofetil, had positive results. In contrast, intravenous immunoglobulins had disappointing results.</abstract><cop>United States</cop><pub>The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved</pub><pmid>27175675</pmid><doi>10.1097/MD.0000000000003632</doi><oa>free_for_read</oa></addata></record> |
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subjects | Adrenal Cortex Hormones - therapeutic use Adult Aged Cyclophosphamide - therapeutic use Disability Evaluation Enzyme Inhibitors - therapeutic use Female Follow-Up Studies Humans Hydroxychloroquine - therapeutic use Immunoglobulins, Intravenous - therapeutic use Immunologic Factors - therapeutic use Male Middle Aged Mycophenolic Acid - therapeutic use Observational Study Peripheral Nervous System Diseases - drug therapy Peripheral Nervous System Diseases - etiology Retrospective Studies Sensory Receptor Cells Sjogren's Syndrome - complications Treatment Outcome Young Adult |
title | Sjögren Sensory Neuronopathy (Sjögren Ganglionopathy): Long-Term Outcome and Treatment Response in a Series of 13 Cases |
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