{"id":"https://openalex.org/W2800436342","doi":"https://doi.org/10.1212/wnl.88.16_supplement.p5.077","title":"Mitochondrial Skeletal and Cardiac Myopathy Responsive to Aggressive Immunosuppression in Rapidly Progressing Systemic Sclerosis (P5.077)","display_name":"Mitochondrial Skeletal and Cardiac Myopathy Responsive to Aggressive Immunosuppression in Rapidly Progressing Systemic Sclerosis (P5.077)","publication_year":2017,"publication_date":"2017-04-18","ids":{"openalex":"https://openalex.org/W2800436342","doi":"https://doi.org/10.1212/wnl.88.16_supplement.p5.077","mag":"2800436342"},"language":"en","primary_location":{"id":"doi:10.1212/wnl.88.16_supplement.p5.077","doi":"https://doi.org/10.1212/wnl.88.16_supplement.p5.077","is_oa":false,"landing_page_url":"https://doi.org/10.1212/wnl.88.16_supplement.p5.077","pdf_url":null,"source":{"id":"https://openalex.org/S79704628","display_name":"Neurology","issn_l":"0028-3878","issn":["0028-3878","1526-632X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Neurology","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5060973267","display_name":"Crystal Jing Jing Yeo","orcid":"https://orcid.org/0000-0001-5445-5965","observed_orcids":["https://orcid.org/0000-0001-5445-5965"]},"institutions":[{"id":"https://openalex.org/I1295876152","display_name":"Houston Methodist","ror":"https://ror.org/027zt9171","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1295876152"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Crystal Yeo","raw_affiliation_strings":["Methodist Neurological Institute Houston TX United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Methodist Neurological Institute Houston TX United States","institution_ids":["https://openalex.org/I1295876152"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Vivian Bunin","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1295876152","display_name":"Houston Methodist","ror":"https://ror.org/027zt9171","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1295876152"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Vivian Bunin","raw_affiliation_strings":["Rheumatology, Houston Methodist Hospital Houston TX United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Rheumatology, Houston Methodist Hospital Houston TX United States","institution_ids":["https://openalex.org/I1295876152"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5022462108","display_name":"Popovich Jovan","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1295876152","display_name":"Houston Methodist","ror":"https://ror.org/027zt9171","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1295876152"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Popovich Jovan","raw_affiliation_strings":["Rheumatology, Houston Methodist Hospital Houston TX United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Rheumatology, Houston Methodist Hospital Houston TX United States","institution_ids":["https://openalex.org/I1295876152"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5067158932","display_name":"Matthew D. Cykowski","orcid":"https://orcid.org/0000-0002-5973-803X","observed_orcids":["https://orcid.org/0000-0002-5973-803X"]},"institutions":[{"id":"https://openalex.org/I1295876152","display_name":"Houston Methodist","ror":"https://ror.org/027zt9171","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1295876152"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Matthew Cykowski","raw_affiliation_strings":["Pathology, Houston Methodist Hospital Houston TX United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pathology, Houston Methodist Hospital Houston TX United States","institution_ids":["https://openalex.org/I1295876152"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5081273730","display_name":"Robert David Smith","orcid":"https://orcid.org/0000-0002-8497-216X","observed_orcids":["https://orcid.org/0000-0002-8497-216X"]},"institutions":[{"id":"https://openalex.org/I1295876152","display_name":"Houston Methodist","ror":"https://ror.org/027zt9171","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1295876152"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Robert Smith","raw_affiliation_strings":["Methodist Neurological Institute Houston TX United States"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Methodist Neurological Institute Houston TX United States","institution_ids":["https://openalex.org/I1295876152"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":["https://openalex.org/I1295876152"],"apc_list":null,"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.2769993,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"88","issue":"16_supplement","first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11330","display_name":"Systemic Sclerosis and Related Diseases","score":0.9150000214576721,"subfield":{"id":"https://openalex.org/subfields/2734","display_name":"Pathology and Forensic Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T11330","display_name":"Systemic Sclerosis and Related Diseases","score":0.9150000214576721,"subfield":{"id":"https://openalex.org/subfields/2734","display_name":"Pathology and Forensic Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11724","display_name":"Inflammatory Myopathies and Dermatomyositis","score":0.05290000140666962,"subfield":{"id":"https://openalex.org/subfields/2713","display_name":"Epidemiology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10301","display_name":"Mitochondrial Function and Pathology","score":0.01489999983459711,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/systemic-sclerosis","display_name":"systemic sclerosis","score":0.9649999737739563},{"id":"https://openalex.org/keywords/mitochondrial-myopathy","display_name":"mitochondrial myopathy","score":0.6830000281333923},{"id":"https://openalex.org/keywords/cardiomyopathy","display_name":"cardiomyopathy","score":0.8610000014305115},{"id":"https://openalex.org/keywords/skeletal-myopathy","display_name":"skeletal myopathy","score":0.8790000081062317},{"id":"https://openalex.org/keywords/immunosuppressive-therapy","display_name":"immunosuppressive therapy","score":0.7870000004768372},{"id":"https://openalex.org/keywords/perivascular-inflammation","display_name":"perivascular inflammation","score":0.8560000061988831},{"id":"https://openalex.org/keywords/mitochondrial-pathology","display_name":"mitochondrial pathology","score":0.7509999871253967}],"concepts":[{"id":"https://openalex.org/C2780252810","wikidata":"https://www.wikidata.org/wiki/Q1455316","display_name":"Immunosuppression","level":2,"score":0.8354966640472412},{"id":"https://openalex.org/C2777300911","wikidata":"https://www.wikidata.org/wiki/Q692536","display_name":"Myopathy","level":2,"score":0.6912621259689331},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.6601434946060181},{"id":"https://openalex.org/C2775833069","wikidata":"https://www.wikidata.org/wiki/Q6881881","display_name":"Mitochondrial myopathy","level":4,"score":0.43098583817481995},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.38172340393066406},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3423054814338684},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.1110149621963501},{"id":"https://openalex.org/C24586158","wikidata":"https://www.wikidata.org/wiki/Q27075","display_name":"Mitochondrial DNA","level":3,"score":0.06734272837638855},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.039124131202697754},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1212/wnl.88.16_supplement.p5.077","doi":"https://doi.org/10.1212/wnl.88.16_supplement.p5.077","is_oa":false,"landing_page_url":"https://doi.org/10.1212/wnl.88.16_supplement.p5.077","pdf_url":null,"source":{"id":"https://openalex.org/S79704628","display_name":"Neurology","issn_l":"0028-3878","issn":["0028-3878","1526-632X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Neurology","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":1.0}],"sustainable_development_goals_aurora":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.8199999928474426}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W2559832188","https://openalex.org/W2053792722","https://openalex.org/W2032285400","https://openalex.org/W7171877149","https://openalex.org/W7163653328","https://openalex.org/W2410260992","https://openalex.org/W2132151198","https://openalex.org/W1996588877","https://openalex.org/W7165029205","https://openalex.org/W7167892233"],"abstract_inverted_index":{"Objective:":[0],"To":[1],"report":[2,61],"mitochondrial":[3,223,266,295],"pathology":[4],"and":[5,18,49,57,70,78,97,109,122,128,133,153,164,184,190,200,212,215,225,258,271],"perivascular":[6,147],"epimysial":[7,232],"inflammation":[8,233],"without":[9,242],"myositis":[10,183,243],"in":[11,29,159,188,237,248,260,285],"rapidly":[12,46],"progressing":[13,23,47,298],"systemic":[14,30,138,249,261],"sclerosis":[15],"with":[16,66,81,130,264],"cardiac":[17,48,84,272],"skeletal":[19,50,93,270],"myopathy.":[20,296],"Background:":[21],"Rapidly":[22,297],"myopathy":[24,51,176,241,284],"is":[25,37,234],"a":[26,71,144,173],"rare":[27],"presentation":[28,282],"sclerosis,":[31,262],"as":[32,294],"the":[33],"associated":[34],"muscle":[35,68,94,124,141,273],"weakness":[36],"commonly":[38],"indolent.":[39],"Prior":[40],"case":[41],"series":[42],"reported":[43,247],"that":[44,253],"such":[45,293],"has":[52,244,307,313,319,325,331],"histopathological":[53],"features":[54,171],"of":[55,75,92,172,182,283],"polymyositis":[56],"dermatomyositis.":[58],"Design/Methods:":[59],"Case":[60],"Results:":[62],"A":[63],"32-year-old":[64],"man":[65],"exercise-induced":[67],"cramps":[69],"maternal":[72],"family":[73],"history":[74],"depression,":[76],"fibromyalgia":[77],"\u201chypochondria\u201d,":[79],"presented":[80],"fulminant":[82],"non-ischemic":[83],"failure":[85],"necessitating":[86],"AICD,":[87],"followed":[88,104],"by":[89,105,166,204,256],"rapid":[90],"development":[91],"atrophy,":[95],"fasciculations":[96],"weakness,":[98],"rendering":[99],"him":[100],"wheelchair-bound.":[101],"This":[102],"was":[103,137,162],"Raynaud\u2019s":[106],"phenomenon,":[107],"sclerodactyly,":[108],"widespread":[110,185],"salt-and-pepper":[111],"pigmentation.":[112],"Labs":[113],"showed":[114,143,180,195],"ANA":[115],"1:40,":[116],"positive":[117],"anti-RNA":[118],"Polymerase":[119],"III":[120],"Ab":[121],"anti-smooth":[123],"Ab,":[125],"elevated":[126],"sIL-2R":[127],"IL-6,":[129],"negative":[131],"anti-scl70":[132],"anti-Jo-1.":[134],"The":[135],"diagnosis":[136],"sclerosis.":[139,250],"Skeletal":[140],"biopsy":[142,194],"mixed,":[145],"epimysial,":[146],"lymphocytic":[148],"infiltrate,":[149],"comprising":[150],"CD4,":[151],"CD8,":[152],"CD20":[154],"immunoreactive":[155],"cells.":[156],"An":[157,280],"increase":[158],"subsarcolemmal":[160],"mitochondria":[161],"identified":[163],"confirmed":[165],"electron":[167],"microscopy.":[168],"No":[169],"pathologic":[170],"specific":[174],"inflammatory":[175],"were":[177,217],"present.":[178],"EMG":[179],"lack":[181],"myopathic":[186],"units":[187],"limb":[189],"paraspinal":[191],"muscles.":[192],"Cardiac":[193],"interstitial":[196],"fibrosis.":[197],"Muscle":[198],"strength":[199],"endurance":[201],"improved":[202],"significantly":[203],"at":[205],"least":[206],"1":[207],"MRC":[208],"grade":[209],"after":[210],"methylprednisolone":[211],"Rituximab.":[213],"Cyclosporine":[214],"IVIG":[216],"added":[218],"to":[219,275,309,315,321,327,333],"his":[220],"regimen.":[221],"Molecular":[222],"analysis":[224],"repeat":[226],"echocardiogram":[227],"are":[228],"pending.":[229],"Conclusions:":[230],"Perivascular":[231],"rarely":[235],"seen":[236],"any":[238],"disease.":[239],"Mitochondrial":[240],"not":[245],"been":[246],"We":[251],"propose":[252],"ischemia":[254],"caused":[255],"vasculopathy":[257],"fibrosis":[259],"together":[263],"subclinical":[265],"pathology,":[267,292],"severely":[268],"affected":[269],"due":[274],"their":[276],"high":[277],"metabolic":[278],"demands.":[279],"unusual":[281],"autoimmune":[286,299],"disease":[287,300],"suggests":[288],"an":[289],"underlying":[290],"latent":[291],"necessitates":[301],"aggressive":[302],"immunosuppression.":[303],"Disclosure:":[304],"Dr.":[305,311,317,323,329],"Yeo":[306],"nothing":[308,314,320,326,332],"disclose.":[310,316,322,328,334],"Bunin":[312],"Jovan":[318],"Cykowski":[324],"Smith":[330]},"counts_by_year":[],"updated_date":"2026-10-26T11:51:55.731654","created_date":"2018-05-17T00:00:00"}