Clinical Subgroups and Factors Associated With Progression in Patients With Inclusion Body Myositis.

Male Female Humans Myositis, Inclusion Body / pathology Myositis Immunosuppressive Agents Muscle Strength Inflammation

Journal

Neurology
ISSN: 1526-632X
Titre abrégé: Neurology
Pays: United States
ID NLM: 0401060

Informations de publication

Date de publication:
28 03 2023
Historique:
received: 09 03 2022
accepted: 18 11 2022
medline: 29 3 2023
pubmed: 24 1 2023
entrez: 23 1 2023
Statut: ppublish

Résumé

Sporadic inclusion body myositis (IBM) is the most common acquired myopathy in individuals older than 50 years. The disorder is slowly progressive, and although many therapies have been investigated, response has generally been poor. Clinical heterogeneity may influence treatment responsiveness; however, data regarding heterogeneity in IBM are limited and often conflicting. We aim to identify clinically distinct subgroups within a large IBM cohort and prognostic factors for disease progression. Clinical, histologic, radiologic, and electrophysiologic data were analyzed for all patients with IBM and other forms of myositis enrolled in a longitudinal cohort from The Johns Hopkins Myositis Center from 2003 to 2018. Patients with IBM were included if they met at least one of the following criteria: Griggs possible, European Neuromuscular Centre 2011 probable, or Lloyd-Greenberg data-derived criteria for IBM. Univariate, multivariate, and graphical analyses were used to identify prognostic factors in patients with IBM. Thus, linear and logistic regressions were used to adjust for potential confounding variables. The evolution of creatine kinase and muscle strength was studied using multilevel linear regression models. Nonmodifiable risk factors (sex, race, disease duration, and age at the onset of first symptoms) were used as adjusting covariates for the regression analyses. Among the 335 patients meeting the inclusion criteria for IBM, 64% were male with an average age of disease onset of 58.7 years and delay to diagnosis of 5.2 years. Initial misdiagnosis (52%) and immunosuppressant treatment (42%) were common. Less than half (43%) of muscle biopsies demonstrated all 3 pathologic hallmarks: endomysial inflammation, mononuclear cell invasion, and rimmed vacuoles. Black patients had significantly weaker arm abductors, hip flexors, and knee flexors compared with non-Black patients. Female patients had stronger finger flexors and knee extensors compared with their male counterparts. Younger age (<50 years) at onset was not associated with increased weakness. Our study demonstrates that female and Black patients have distinct clinical phenotypes and trajectories within the overarching IBM clinical phenotype. These subgroups may have different responses to therapies, which may influence the design of future clinical trials in IBM.

Sections du résumé

BACKGROUND AND OBJECTIVES
Sporadic inclusion body myositis (IBM) is the most common acquired myopathy in individuals older than 50 years. The disorder is slowly progressive, and although many therapies have been investigated, response has generally been poor. Clinical heterogeneity may influence treatment responsiveness; however, data regarding heterogeneity in IBM are limited and often conflicting. We aim to identify clinically distinct subgroups within a large IBM cohort and prognostic factors for disease progression.
METHODS
Clinical, histologic, radiologic, and electrophysiologic data were analyzed for all patients with IBM and other forms of myositis enrolled in a longitudinal cohort from The Johns Hopkins Myositis Center from 2003 to 2018. Patients with IBM were included if they met at least one of the following criteria: Griggs possible, European Neuromuscular Centre 2011 probable, or Lloyd-Greenberg data-derived criteria for IBM. Univariate, multivariate, and graphical analyses were used to identify prognostic factors in patients with IBM. Thus, linear and logistic regressions were used to adjust for potential confounding variables. The evolution of creatine kinase and muscle strength was studied using multilevel linear regression models. Nonmodifiable risk factors (sex, race, disease duration, and age at the onset of first symptoms) were used as adjusting covariates for the regression analyses.
RESULTS
Among the 335 patients meeting the inclusion criteria for IBM, 64% were male with an average age of disease onset of 58.7 years and delay to diagnosis of 5.2 years. Initial misdiagnosis (52%) and immunosuppressant treatment (42%) were common. Less than half (43%) of muscle biopsies demonstrated all 3 pathologic hallmarks: endomysial inflammation, mononuclear cell invasion, and rimmed vacuoles. Black patients had significantly weaker arm abductors, hip flexors, and knee flexors compared with non-Black patients. Female patients had stronger finger flexors and knee extensors compared with their male counterparts. Younger age (<50 years) at onset was not associated with increased weakness.
DISCUSSION
Our study demonstrates that female and Black patients have distinct clinical phenotypes and trajectories within the overarching IBM clinical phenotype. These subgroups may have different responses to therapies, which may influence the design of future clinical trials in IBM.

Identifiants

DOI: 10.1212/WNL.0000000000206777 PMID: 36690456 PMC: PMC10065210
pubmed: 36690456
pii: WNL.0000000000206777
doi: 10.1212/WNL.0000000000206777
pmc: PMC10065210
doi:

Substances chimiques

Immunosuppressive Agents 0

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e1406-e1417

Subventions

Organisme : NIAMS NIH HHS
ID : K08 AR077732
Pays : United States
Organisme : NIAMS NIH HHS
ID : K23 AR073927
Pays : United States
Organisme : NIAMS NIH HHS
ID : R01 AR076390
Pays : United States

Commentaires et corrections

Type : CommentIn
Type : CommentIn

Informations de copyright

© 2023 American Academy of Neurology.

Références

Neuromuscul Disord. 2013 Dec;23(12):1044-55
pubmed: 24268584
Ann Neurol. 2002 Mar;51(3):369-72
pubmed: 11891832
Muscle Nerve. 2016 Sep;54(3):371-7
pubmed: 27238640
Brain. 2011 Nov;134(Pt 11):3176-84
pubmed: 21994327
Neurology. 2000 Jul 25;55(2):296-8
pubmed: 10908910
Arthritis Rheumatol. 2022 Mar;74(3):508-517
pubmed: 34480833
Neurology. 2021 May 25;96(21):e2653-e2661
pubmed: 33879596
Ann Clin Transl Neurol. 2021 Mar;8(3):571-578
pubmed: 33556224
Neurology. 2004 Aug 24;63(4):718-20
pubmed: 15326251
Rheumatology (Oxford). 2022 May 5;61(5):2016-2024
pubmed: 34534271
Neuromuscul Disord. 2013 May;23(5):404-12
pubmed: 23489664
Ann Rheum Dis. 2017 May;76(5):862-868
pubmed: 28122761
Medicine (Baltimore). 2001 Sep;80(5):320-7
pubmed: 11552086
Neurology. 2014 Jul 29;83(5):426-33
pubmed: 24975859
Muscle Nerve. 2020 Jul;62(1):76-82
pubmed: 32134516
Neurology. 2001 Feb 13;56(3):323-7
pubmed: 11171896
Brain. 2016 May;139(Pt 5):1348-60
pubmed: 26920676
J Neurol Neurosurg Psychiatry. 2021 Apr 13;:
pubmed: 33849999
Brain. 2011 Nov;134(Pt 11):3167-75
pubmed: 21908393
Rheumatology (Oxford). 2017 Jun 1;56(6):999-1007
pubmed: 28339994
Rheumatology (Oxford). 2017 May 1;56(5):787-794
pubmed: 28096458
Muscle Nerve. 2015 Oct;52(4):527-33
pubmed: 25557419
Neurol Clin. 2014 Aug;32(3):629-46, vii
pubmed: 25037082
Acta Neuropathol. 2015 May;129(5):611-24
pubmed: 25579751
Acta Neurol Scand. 1994 Feb;89(2):123-31
pubmed: 8191875
Neurology. 2006 Jan 24;66(2 Suppl 1):S123-4
pubmed: 16432140
J Neuromuscul Dis. 2016 Mar 3;3(1):67-75
pubmed: 27854208
Clin Neurophysiol. 2020 Nov;131(11):2766-2776
pubmed: 32928695
J Neurol. 2005 Dec;252(12):1448-54
pubmed: 15942703
Clin Investig. 1993 May;71(5):351-61
pubmed: 8389626
Muscle Nerve. 2021 Apr;63(4):490-496
pubmed: 33373040
J Pediatr. 2017 May;184:38-44.e1
pubmed: 28410093
Ann Neurol. 1995 Nov;38(5):705-13
pubmed: 7486861
J Neurol Sci. 2014 Nov 15;346(1-2):133-7
pubmed: 25152387
Arthritis Res Ther. 2012 Jan 27;14(1):R22
pubmed: 22284862
Brain. 2009 Jun;132(Pt 6):1536-44
pubmed: 19454532
Brain. 1989 Jun;112 ( Pt 3):727-47
pubmed: 2543478
Neurology. 2001 Aug 14;57(3):548-50
pubmed: 11502935
Arch Neurol. 1987 Nov;44(11):1154-7
pubmed: 2823752
Ann Neurol. 1996 Oct;40(4):581-6
pubmed: 8871577
Muscle Nerve. 1990 Oct;13(10):949-51
pubmed: 2172812

Auteurs

Elizabeth Harlan Michelle (EH)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Iago Pinal-Fernandez (I)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Maria Casal-Dominguez (M)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Jemima Albayda (J)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Julie J Paik (JJ)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Eleni Tiniakou (E)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Brittany Adler (B)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Christopher A Mecoli (CA)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Sonye K Danoff (SK)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Lisa Christopher-Stine (L)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Andrew L Mammen (AL)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain.

Thomas E Lloyd (TE)

From the Departments of Neurology (E.H.M., I.P.-F., M.C.-D., A.L.M., T.E.L.), and Medicine (J.A., J.J.P., E.T., B.A., C.A.M., S.K.D., L.C.-S.), Johns Hopkins University School of Medicine, Baltimore, MD; Muscle Disease Unit (I.P.-F., M.C.-D., A.L.M.), Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; and Faculty of Health Sciences and Faculty of Computer Science (I.P.-F.), Multimedia and Telecommunications, Universitat Oberta de Catalunya, Barcelona, Spain. tlloyd4@jhmi.edu.

Articles similaires

[Redispensing of expensive oral anticancer medicines: a practical application].

Lisanne N van Merendonk, Kübra Akgöl, Bastiaan Nuijen
1.00
Humans Antineoplastic Agents Administration, Oral Drug Costs Counterfeit Drugs

Smoking Cessation and Incident Cardiovascular Disease.

Jun Hwan Cho, Seung Yong Shin, Hoseob Kim et al.
1.00
Humans Male Smoking Cessation Cardiovascular Diseases Female

Evaluation of Low-Value Services Across Major Medicare Advantage Insurers and Traditional Medicare.

Ciara Duggan, Adam L Beckman, Ishani Ganguli et al.
1.00
Humans United States Aged Cross-Sectional Studies Medicare Part C

Effectiveness of Virtual Yoga for Chronic Low Back Pain: A Randomized Clinical Trial.

Hallie Tankha, Devyn Gaskins, Amanda Shallcross et al.
1.00
Humans Yoga Low Back Pain Female Male

Classifications MeSH

questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Clinical Subgroups and Factors Associated With...
questionsmedicales.fr › Maladies du système nerveux › Maladies neuromusculaires › Maladies musculaires › Myosite › Myosite à inclusions › Clinical Subgroups and Factors Associated With...
questionsmedicales.fr › Maladies du système nerveux › Maladies neuromusculaires › Maladies musculaires › Myosite › Clinical Subgroups and Factors Associated With...
questionsmedicales.fr › Actions chimiques et utilisations › Actions pharmacologiques › Effets physiologiques des médicaments › Facteurs immunologiques › Immunosuppresseurs › Clinical Subgroups and Factors Associated With...
questionsmedicales.fr › Phénomènes physiologiques de l'appareil locomoteur et du système nerveux › Phénomènes physiologiques du système locomoteur › Force musculaire › Clinical Subgroups and Factors Associated With...
questionsmedicales.fr › États, signes et symptômes pathologiques › Processus pathologiques › Inflammation › Clinical Subgroups and Factors Associated With...