Refractory myasthenia gravis: Characteristics of a portuguese cohort
ABSTRACT Introduction: Some myasthenia gravis (MG) patients are refractory to conventional treatments. Methods: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA‐DRB1 alleles, a cohort study of 114 consecutive MG patients was perfor...
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| Veröffentlicht in: | Muscle & nerve 2019-08, Vol.60 (2), p.188-191 |
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| creator | Santos, Ernestina Bettencourt, Andreia Duarte, Sara Gabriel, Denis Oliveira, Vanessa da Silva, Ana Martins Costa, Paulo Pinho Lopes, Carlos Gonçalves, Guilherme da Silva, Berta Martins Leite, Maria Isabel |
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Introduction: Some myasthenia gravis (MG) patients are refractory to conventional treatments. Methods: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA‐DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria. Results: Twenty‐two patients were found to have RMG (19.3%). There were no differences between non‐RMG and RMG patients with respect to sex, age of onset, abnormal 3‐Hz repetitive nerve stimulation, anti‐acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA‐DRB1*03 was more frequent in the non‐RMG vs. control population (P = 3 × 10−6). The HLA‐DRB1*13 allele was less frequent in non‐RMG patients compared with controls (P = 0.002), and less frequent in the non‐RMG group compared with the RMG group (P = 0.003). Discussion: HLA‐DRB1*03 was more common in non‐RMG, and the HLA‐DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188–191, 2019 |
| doi_str_mv | 10.1002/mus.26507 |
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Introduction: Some myasthenia gravis (MG) patients are refractory to conventional treatments. Methods: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA‐DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria. Results: Twenty‐two patients were found to have RMG (19.3%). There were no differences between non‐RMG and RMG patients with respect to sex, age of onset, abnormal 3‐Hz repetitive nerve stimulation, anti‐acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA‐DRB1*03 was more frequent in the non‐RMG vs. control population (P = 3 × 10−6). The HLA‐DRB1*13 allele was less frequent in non‐RMG patients compared with controls (P = 0.002), and less frequent in the non‐RMG group compared with the RMG group (P = 0.003). Discussion: HLA‐DRB1*03 was more common in non‐RMG, and the HLA‐DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188–191, 2019</description><identifier>ISSN: 0148-639X</identifier><identifier>EISSN: 1097-4598</identifier><identifier>DOI: 10.1002/mus.26507</identifier><identifier>PMID: 31050006</identifier><language>eng</language><publisher>Hoboken, USA: John Wiley & Sons, Inc</publisher><subject>Alleles ; Antibodies ; Antigens ; Autoimmune diseases ; Drb1 protein ; Histocompatibility antigen HLA ; human leukocyte antigens ; Hyperplasia ; Leukocytes ; Muscles ; Myasthenia ; Myasthenia gravis ; Neuromuscular junctions ; polyautoimmunity ; Population genetics ; refractory ; Thymectomy ; Thymoma ; thymoma MG ; Thymus</subject><ispartof>Muscle & nerve, 2019-08, Vol.60 (2), p.188-191</ispartof><rights>2019 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3817-c4affe99117ee680b6530ad1f9f378a46c84c48d0ad48680531a349d24a598933</citedby><cites>FETCH-LOGICAL-c3817-c4affe99117ee680b6530ad1f9f378a46c84c48d0ad48680531a349d24a598933</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fmus.26507$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fmus.26507$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31050006$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Santos, Ernestina</creatorcontrib><creatorcontrib>Bettencourt, Andreia</creatorcontrib><creatorcontrib>Duarte, Sara</creatorcontrib><creatorcontrib>Gabriel, Denis</creatorcontrib><creatorcontrib>Oliveira, Vanessa</creatorcontrib><creatorcontrib>da Silva, Ana Martins</creatorcontrib><creatorcontrib>Costa, Paulo Pinho</creatorcontrib><creatorcontrib>Lopes, Carlos</creatorcontrib><creatorcontrib>Gonçalves, Guilherme</creatorcontrib><creatorcontrib>da Silva, Berta Martins</creatorcontrib><creatorcontrib>Leite, Maria Isabel</creatorcontrib><title>Refractory myasthenia gravis: Characteristics of a portuguese cohort</title><title>Muscle & nerve</title><addtitle>Muscle Nerve</addtitle><description>ABSTRACT
Introduction: Some myasthenia gravis (MG) patients are refractory to conventional treatments. Methods: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA‐DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria. Results: Twenty‐two patients were found to have RMG (19.3%). There were no differences between non‐RMG and RMG patients with respect to sex, age of onset, abnormal 3‐Hz repetitive nerve stimulation, anti‐acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA‐DRB1*03 was more frequent in the non‐RMG vs. control population (P = 3 × 10−6). The HLA‐DRB1*13 allele was less frequent in non‐RMG patients compared with controls (P = 0.002), and less frequent in the non‐RMG group compared with the RMG group (P = 0.003). Discussion: HLA‐DRB1*03 was more common in non‐RMG, and the HLA‐DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188–191, 2019</description><subject>Alleles</subject><subject>Antibodies</subject><subject>Antigens</subject><subject>Autoimmune diseases</subject><subject>Drb1 protein</subject><subject>Histocompatibility antigen HLA</subject><subject>human leukocyte antigens</subject><subject>Hyperplasia</subject><subject>Leukocytes</subject><subject>Muscles</subject><subject>Myasthenia</subject><subject>Myasthenia gravis</subject><subject>Neuromuscular junctions</subject><subject>polyautoimmunity</subject><subject>Population genetics</subject><subject>refractory</subject><subject>Thymectomy</subject><subject>Thymoma</subject><subject>thymoma MG</subject><subject>Thymus</subject><issn>0148-639X</issn><issn>1097-4598</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><recordid>eNp1kM9LwzAUx4Mobk4P_gNS8KKHbi9N2ibeZP6EiaAOvJUsTbaOdplJq_S_N6XTg-ApvLwPn_feF6FTDGMMEE2qxo2jJIZ0Dw0x8DSkMWf7aAiYsjAh_H2AjpxbAwBmSXqIBgRD7KtkiG5elLZC1sa2QdUKV6_UphDB0orPwl0F05XousoWri6kC4wORLA1tm6WjXIqkGbli2N0oEXp1MnuHaH53e3b9CGcPd8_Tq9noSQMp6GkQmvFOcapUgmDRRITEDnWXJOUCZpIRiVluf-jzPdjggWhPI-o8PdwQkboovdurfnw8-usKpxUZSk2yjQui6KIRyQG1qHnf9C1aezGb-epOAGgwFJPXfaUtMY5q3S2tUUlbJthyLpos6rTdtF69mxnbBaVyn_Jnyw9MOmBr6JU7f-m7Gn-2iu_AQVBgZw</recordid><startdate>201908</startdate><enddate>201908</enddate><creator>Santos, Ernestina</creator><creator>Bettencourt, Andreia</creator><creator>Duarte, Sara</creator><creator>Gabriel, Denis</creator><creator>Oliveira, Vanessa</creator><creator>da Silva, Ana Martins</creator><creator>Costa, Paulo Pinho</creator><creator>Lopes, Carlos</creator><creator>Gonçalves, Guilherme</creator><creator>da Silva, Berta Martins</creator><creator>Leite, Maria Isabel</creator><general>John Wiley & Sons, Inc</general><general>Wiley Subscription Services, Inc</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>7TM</scope><scope>7TS</scope><scope>7U7</scope><scope>7U9</scope><scope>C1K</scope><scope>H94</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>7X8</scope></search><sort><creationdate>201908</creationdate><title>Refractory myasthenia gravis: Characteristics of a portuguese cohort</title><author>Santos, Ernestina ; Bettencourt, Andreia ; Duarte, Sara ; Gabriel, Denis ; Oliveira, Vanessa ; da Silva, Ana Martins ; Costa, Paulo Pinho ; Lopes, Carlos ; Gonçalves, Guilherme ; da Silva, Berta Martins ; Leite, Maria Isabel</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3817-c4affe99117ee680b6530ad1f9f378a46c84c48d0ad48680531a349d24a598933</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2019</creationdate><topic>Alleles</topic><topic>Antibodies</topic><topic>Antigens</topic><topic>Autoimmune diseases</topic><topic>Drb1 protein</topic><topic>Histocompatibility antigen HLA</topic><topic>human leukocyte antigens</topic><topic>Hyperplasia</topic><topic>Leukocytes</topic><topic>Muscles</topic><topic>Myasthenia</topic><topic>Myasthenia gravis</topic><topic>Neuromuscular junctions</topic><topic>polyautoimmunity</topic><topic>Population genetics</topic><topic>refractory</topic><topic>Thymectomy</topic><topic>Thymoma</topic><topic>thymoma MG</topic><topic>Thymus</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Santos, Ernestina</creatorcontrib><creatorcontrib>Bettencourt, Andreia</creatorcontrib><creatorcontrib>Duarte, Sara</creatorcontrib><creatorcontrib>Gabriel, Denis</creatorcontrib><creatorcontrib>Oliveira, Vanessa</creatorcontrib><creatorcontrib>da Silva, Ana Martins</creatorcontrib><creatorcontrib>Costa, Paulo Pinho</creatorcontrib><creatorcontrib>Lopes, Carlos</creatorcontrib><creatorcontrib>Gonçalves, Guilherme</creatorcontrib><creatorcontrib>da Silva, Berta Martins</creatorcontrib><creatorcontrib>Leite, Maria Isabel</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Physical Education Index</collection><collection>Toxicology Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Environmental Sciences and Pollution Management</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>MEDLINE - Academic</collection><jtitle>Muscle & nerve</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Santos, Ernestina</au><au>Bettencourt, Andreia</au><au>Duarte, Sara</au><au>Gabriel, Denis</au><au>Oliveira, Vanessa</au><au>da Silva, Ana Martins</au><au>Costa, Paulo Pinho</au><au>Lopes, Carlos</au><au>Gonçalves, Guilherme</au><au>da Silva, Berta Martins</au><au>Leite, Maria Isabel</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Refractory myasthenia gravis: Characteristics of a portuguese cohort</atitle><jtitle>Muscle & nerve</jtitle><addtitle>Muscle Nerve</addtitle><date>2019-08</date><risdate>2019</risdate><volume>60</volume><issue>2</issue><spage>188</spage><epage>191</epage><pages>188-191</pages><issn>0148-639X</issn><eissn>1097-4598</eissn><abstract>ABSTRACT
Introduction: Some myasthenia gravis (MG) patients are refractory to conventional treatments. Methods: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA‐DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria. Results: Twenty‐two patients were found to have RMG (19.3%). There were no differences between non‐RMG and RMG patients with respect to sex, age of onset, abnormal 3‐Hz repetitive nerve stimulation, anti‐acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA‐DRB1*03 was more frequent in the non‐RMG vs. control population (P = 3 × 10−6). The HLA‐DRB1*13 allele was less frequent in non‐RMG patients compared with controls (P = 0.002), and less frequent in the non‐RMG group compared with the RMG group (P = 0.003). Discussion: HLA‐DRB1*03 was more common in non‐RMG, and the HLA‐DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188–191, 2019</abstract><cop>Hoboken, USA</cop><pub>John Wiley & Sons, Inc</pub><pmid>31050006</pmid><doi>10.1002/mus.26507</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Alleles Antibodies Antigens Autoimmune diseases Drb1 protein Histocompatibility antigen HLA human leukocyte antigens Hyperplasia Leukocytes Muscles Myasthenia Myasthenia gravis Neuromuscular junctions polyautoimmunity Population genetics refractory Thymectomy Thymoma thymoma MG Thymus |
| title | Refractory myasthenia gravis: Characteristics of a portuguese cohort |
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