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Inclusion body myositis(IBM)

MedGen UID:
68659
Concept ID:
C0238190
Disease or Syndrome
Synonym: IBM
SNOMED CT: Inclusion body myositis (72315009); IBM - Inclusion body myositis (72315009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0007827
OMIM®: 147421
Orphanet: ORPHA611

Definition

Sporadic inclusion body myositis (IBM) is the most common age-related muscle disease in the elderly that results in severe disability. Although traditionally considered an inflammatory myopathy, it is now considered to be more consistent with a myodegenerative disease (Sugarman et al., 2002; Askanas and Engel, 2006). [from OMIM]

Additional description

From MedlinePlus Genetics
In sporadic inclusion body myositis, the muscles most affected are those of the wrists and fingers and the front of the thigh. Affected individuals may frequently stumble while walking and find it difficult to grasp items. As in dermatomyositis and polymyositis, swallowing can be difficult.

Polymyositis and dermatomyositis have similar symptoms, but dermatomyositis is distinguished by a reddish or purplish rash on the eyelids, elbows, knees, or knuckles. Sometimes, abnormal calcium deposits form hard, painful bumps under the skin (calcinosis).

Polymyositis and dermatomyositis involve weakness of the muscles closest to the center of the body (proximal muscles), such as the muscles of the hips and thighs, upper arms, and neck. People with these forms of idiopathic inflammatory myopathy may find it difficult to climb stairs, get up from a seated position, or lift items above their head. In some cases, muscle weakness may make swallowing or breathing difficult.

There are several forms of idiopathic inflammatory myopathy, including polymyositis, dermatomyositis, and sporadic inclusion body myositis.

The primary symptom of idiopathic inflammatory myopathy is muscle weakness, which develops gradually over a period of weeks to months or even years. Other symptoms include joint pain and general tiredness (fatigue).

Idiopathic inflammatory myopathy is a group of disorders characterized by inflammation of the muscles used for movement (skeletal muscles). Idiopathic inflammatory myopathy usually appears in adults between ages 40 and 60 or in children between ages 5 and 15, though it can occur at any age.  https://medlineplus.gov/genetics/condition/idiopathic-inflammatory-myopathy

Clinical features

From HPO
Dysphagia
MedGen UID:
41440
Concept ID:
C0011168
Disease or Syndrome
Difficulty in swallowing.
Hyporeflexia
MedGen UID:
195967
Concept ID:
C0700078
Finding
Reduction of neurologic reflexes such as the knee-jerk reaction.
Proximal muscle weakness
MedGen UID:
113169
Concept ID:
C0221629
Finding
A lack of strength of the proximal muscles.
Distal muscle weakness
MedGen UID:
140883
Concept ID:
C0427065
Finding
Reduced strength of the musculature of the distal extremities.
Rimmed vacuoles
MedGen UID:
340089
Concept ID:
C1853932
Finding
Presence of abnormal vacuoles (membrane-bound organelles) in the sarcolemma. On histological staining with hematoxylin and eosin, rimmed vacuoles are popcorn-like clear vacuoles with a densely blue rim. The vacuoles are often associated with cytoplasmic and occasionally intranuclear eosinophilic inclusions.
Inflammatory myopathy
MedGen UID:
1611049
Concept ID:
CN971896
Disease or Syndrome
Chronic muscle inflammation accompanied by muscle weakness.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Inclusion body myositis
Follow this link to review classifications for Inclusion body myositis in Orphanet.

Professional guidelines

PubMed

Selva-O'Callaghan A, Pinal-Fernandez I, Trallero-Araguás E, Milisenda JC, Grau-Junyent JM, Mammen AL
Lancet Neurol 2018 Sep;17(9):816-828. doi: 10.1016/S1474-4422(18)30254-0. PMID: 30129477
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460

Recent clinical studies

Etiology

Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Greenberg SA
Nat Rev Rheumatol 2019 May;15(5):257-272. doi: 10.1038/s41584-019-0186-x. PMID: 30837708
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Lundberg IE, de Visser M, Werth VP
Nat Rev Rheumatol 2018 May;14(5):269-278. Epub 2018 Apr 12 doi: 10.1038/nrrheum.2018.41. PMID: 29651121
Dalakas MC, Hohlfeld R
Lancet 2003 Sep 20;362(9388):971-82. doi: 10.1016/S0140-6736(03)14368-1. PMID: 14511932

Diagnosis

Goyal NA
Continuum (Minneap Minn) 2022 Dec 1;28(6):1663-1677. doi: 10.1212/CON.0000000000001204. PMID: 36537974
Ashton C, Paramalingam S, Stevenson B, Brusch A, Needham M
Intern Med J 2021 Jun;51(6):845-852. doi: 10.1111/imj.15358. PMID: 34155760
Greenberg SA
Nat Rev Rheumatol 2019 May;15(5):257-272. doi: 10.1038/s41584-019-0186-x. PMID: 30837708
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317

Therapy

Tavee J, Brannagan TH 3rd, Lenihan MW, Muppidi S, Kellermeyer L, D Donofrio P; AANEM
Muscle Nerve 2023 Oct;68(4):356-374. Epub 2023 Jul 11 doi: 10.1002/mus.27922. PMID: 37432872
Gandiga PC, Ghetie D, Anderson E, Aggrawal R
Curr Rheumatol Rep 2023 Aug;25(8):152-168. Epub 2023 Jun 1 doi: 10.1007/s11926-023-01105-w. PMID: 37261663
Goyal NA
Continuum (Minneap Minn) 2022 Dec 1;28(6):1663-1677. doi: 10.1212/CON.0000000000001204. PMID: 36537974
Sasaki H, Kohsaka H
Mod Rheumatol 2018 Nov;28(6):913-921. Epub 2018 May 9 doi: 10.1080/14397595.2018.1467257. PMID: 29669460
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317

Prognosis

Dalakas MC
Handb Clin Neurol 2023;195:425-460. doi: 10.1016/B978-0-323-98818-6.00023-6. PMID: 37562881
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Schmidt J
J Neuromuscul Dis 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091Free PMC Article
Findlay AR, Goyal NA, Mozaffar T
Muscle Nerve 2015 May;51(5):638-56. doi: 10.1002/mus.24566. PMID: 25641317
Dalakas MC, Hohlfeld R
Lancet 2003 Sep 20;362(9388):971-82. doi: 10.1016/S0140-6736(03)14368-1. PMID: 14511932

Clinical prediction guides

Connolly CM, Paik JJ
Expert Rev Clin Immunol 2023 Jul-Dec;19(7):797-811. Epub 2023 May 18 doi: 10.1080/1744666X.2023.2212162. PMID: 37158055Free PMC Article
Mariampillai K, Granger B, Amelin D, Guiguet M, Hachulla E, Maurier F, Meyer A, Tohmé A, Charuel JL, Musset L, Allenbach Y, Benveniste O
JAMA Neurol 2018 Dec 1;75(12):1528-1537. doi: 10.1001/jamaneurol.2018.2598. PMID: 30208379Free PMC Article
Tieu J, Lundberg IE, Limaye V
Best Pract Res Clin Rheumatol 2016 Feb;30(1):149-68. Epub 2016 May 26 doi: 10.1016/j.berh.2016.04.007. PMID: 27421222
Lahouti AH, Amato AA, Christopher-Stine L
Curr Opin Rheumatol 2014 Nov;26(6):690-6. doi: 10.1097/BOR.0000000000000116. PMID: 25215417
Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, Herbelin L, Barohn R, Isenberg D, Miller FW
Arthritis Care Res (Hoboken) 2011 Nov;63 Suppl 11(0 11):S118-57. doi: 10.1002/acr.20532. PMID: 22588740Free PMC Article

Recent systematic reviews

Ambrocio KR, Garand KLF, Roy B, Bhutada AM, Malandraki GA
Rheumatology (Oxford) 2023 Oct 3;62(10):3227-3244. doi: 10.1093/rheumatology/kead194. PMID: 37115631
Zhen C, Hou Y, Zhao B, Ma X, Dai T, Yan C
Front Immunol 2022;13:1051609. Epub 2022 Dec 12 doi: 10.3389/fimmu.2022.1051609. PMID: 36578492Free PMC Article
Abdelnaby R, Mohamed KA, Elgenidy A, Sonbol YT, Bedewy MM, Aboutaleb AM, Ebrahim MA, Maallem I, Dardeer KT, Heikal HA, Gawish HM, Zschüntzsch J
Cells 2022 Feb 9;11(4) doi: 10.3390/cells11040600. PMID: 35203250Free PMC Article
Baker BS, Stannard MS, Duren DL, Cook JL, Stannard JP
Clin Orthop Relat Res 2020 Mar;478(3):593-606. doi: 10.1097/CORR.0000000000001090. PMID: 31860546Free PMC Article
Hughes L, Paton B, Rosenblatt B, Gissane C, Patterson SD
Br J Sports Med 2017 Jul;51(13):1003-1011. Epub 2017 Mar 4 doi: 10.1136/bjsports-2016-097071. PMID: 28259850

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