Inclusion Body Myositis (IBM)

Categories: Bone diseases, Immune diseases, Muscle diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Inclusion Body Myositis

MalaCards integrated aliases for Inclusion Body Myositis:

Name: Inclusion Body Myositis 57 12 20 53 58 36 29 6 15 17 70
Sporadic Inclusion Body Myositis 20 58 54
Ibm 57 20 58
Myositis, Inclusion Body 73 44
Inclusion Body Myopathy, Autosomal Recessive 70
Inclusion Body Myopathy, Autosomal Dominant 70
Inclusion Body Myopathy, Sporadic 70
Inflammatory Myopathies 53
Myositis Inclusion Body 54
Inflammatory Myopathy 20
Nonaka Myopathy 70
Myositis 70
Sibm 58


Orphanet epidemiological data:

inclusion body myositis
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult; Age of death: normal life expectancy;


57 (Updated 05-Apr-2021)
variable phenotype
slowly progressive
most common muscle disease of older persons

isolated cases


inclusion body myositis:
Inheritance autosomal dominant inheritance sporadic
Onset and clinical course slow progression


Orphanet: 58  
Rare neurological diseases
Rare systemic and rhumatological diseases

External Ids:

Disease Ontology 12 DOID:3429
OMIM® 57 147421
KEGG 36 H01505
ICD9CM 34 359.71
MeSH 44 D018979
NCIt 50 C84786
SNOMED-CT 67 72315009
ICD10 32 G72.4 G72.41
ICD10 via Orphanet 33 M60.8
UMLS via Orphanet 71 C0238190 C0751713
Orphanet 58 ORPHA611
MedGen 41 C0238190
UMLS 70 C0027121 C0238190 C0751713 more

Summaries for Inclusion Body Myositis

GARD : 20 Inclusion body myositis (IBM) is a progressive muscle disorder characterized by muscle inflammation, weakness, and atrophy (wasting). It is a type of inflammatory myopathy. IBM develops in adulthood, usually after age 50. The symptoms and rate of progression vary from person to person. The most common symptoms include progressive weakness of the legs, arms, fingers, and wrists. Some people also have weakness of the facial muscles (especially muscles controlling eye closure), or difficulty swallowing ( dysphagia ). Muscle cramping and pain are uncommon, but have been reported in some people. Most people with IBM progress to disability over a period of years. In general, the older a person is when IBM begins, the more rapid the progression of the condition. Most people need assistance with basic daily activities within 15 years, and some people will need to use a wheelchair. Lifespan is thought to be normal, but severe complications (e.g. aspiration pneumonia ) can lead to loss of life. The underlying cause of IBM is poorly understood and likely involves the interaction of genetic, immune-related, and environmental factors. Some people may have a genetic predisposition to developing IBM, but the condition itself typically is not inherited. There is currently no cure for IBM. The primary goal of management is to optimize muscle strength and function. Management may include exercise, fall prevention, physical therapy, occupational therapy, and speech therapy (for dysphagia). There is limited evidence that a small proportion of patients may benefit from drugs that suppress the immune system (particularly those with underlying autoimmune disorders), but this therapy is otherwise typically not recommended.

MalaCards based summary : Inclusion Body Myositis, also known as sporadic inclusion body myositis, is related to myositis and amyloidosis, and has symptoms including muscle weakness, myoclonus and back pain. An important gene associated with Inclusion Body Myositis is GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase), and among its related pathways/superpathways are MicroRNAs in cancer and Alzheimers Disease. The drugs Infliximab and Simvastatin have been mentioned in the context of this disorder. Affiliated tissues include skeletal muscle, eye and t cells, and related phenotypes are skeletal muscle atrophy and autoimmunity

Disease Ontology : 12 A myositis that is characterized by late onset of skeletal muscle inflammation, weakness, and atrophy with cytoplasmic granules and vacuoles in the muscle.

OMIM® : 57 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). (147421) (Updated 05-Apr-2021)

NINDS : 53 Inclusion body myositis (IBM) is one of a group of muscle diseases known as the inflammatory myopathies, which are characterized by chronic, progressive muscle inflammation accompanied by muscle weakness.  The onset of muscle weakness in IBM is generally gradual (over months or years) and affects both proximal (close to the trunk of the body) and distal (further away from the trunk) muscles.  Muscle weakness may affect only one side of the body.  Falling and tripping are usually the first noticeable symptoms of IBM.  For some individuals, the disorder begins with weakness in the wrists and fingers that causes difficulty with pinching, buttoning, and gripping objects.  There may be weakness of the wrist and finger muscles and atrophy (thinning or loss of muscle bulk) of the forearm muscles and quadricep muscles in the legs.  Difficulty swallowing occurs in approximately half of IBM cases.  Symptoms of the disease usually begin after the age of 50, although the disease can occur earlier.  IBM occurs more frequently in men than in women.

KEGG : 36 Inclusion body myositis (IBM) is the most frequent acquired myopathy after age 45. It is distinguished from other inflammatory myopathies by its selective pattern of muscle involvement and slowly progressive course, and by the combination of inflammatory and degenerative muscle pathology and multi-protein deposits in muscle tissue. It typically presents with chronic insidious proximal leg and/or distal arm asymmetric muscle weakness leading to recurrent falls and loss of dexterity. Creatine kinase (CK) is elevated in IBM and needle electromyography (EMG) mostly shows a chronic irritative myopathy. Muscle histopathology demonstrates endomysial inflammatory exudates surrounding and invading non-necrotic muscle fibers often times accompanied by rimmed vacuoles and protein deposits. IBM is refractory to all known immunosuppressive therapies. It has been shown in small short-term trials that individualised exercise programs can lead to improvement or maintenance of muscle strength and aerobic capacity.

Wikipedia : 73 Inclusion body myositis (IBM) (/maɪoʊˈsaɪtɪs/) (sometimes called sporadic inclusion body myositis, sIBM)... more...

Related Diseases for Inclusion Body Myositis

Diseases related to Inclusion Body Myositis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 596)
# Related Disease Score Top Affiliating Genes
2 amyloidosis 31.1 SERPINA3 MAPT APP APOE
3 sarcoidosis 1 30.7 SERPINA3 MIR155 ICOSLG CXCR3 CXCL9
4 motor neuron disease 30.6 SERPINA3 MAPT APP APOE
5 creutzfeldt-jakob disease 30.6 SERPINA3 MAPT APP APOE
6 early-onset, autosomal dominant alzheimer disease 30.5 MAPT APP APOE
7 polymyositis 30.5 MIR34A MIR222 MIR221 MIR214 MIR21 MIR155
8 frontotemporal dementia 30.5 MAPT GNE APP APOE
9 alzheimer disease 30.1 SERPINA3 MIR34A MIR197 MAPT BACE1-AS APP
10 down syndrome 30.1 SERPINA3 MIR155 MAPT APP APOE
11 movement disease 30.0 SERPINA3 MAPT APP APOE
12 bacterial infectious disease 30.0 SERPINA3 MIR155 ICOSLG CXCL9
13 muscular dystrophy, limb-girdle, autosomal recessive 1 29.9 MIR197 MIR155 MIR146B
14 peripheral nervous system disease 29.8 SERPINA3 MIR34A MIR21 MAPT ICOSLG APP
15 muscular disease 29.6 SERPINA3 MIR221 MIR21 ICOSLG GNE
16 skin disease 29.2 SERPINA3 MIR21 MIR155 ICOSLG CXCR3 CXCL9
17 dermatomyositis 29.0 MIR381 MIR34A MIR223 MIR222 MIR221 MIR214
18 primary biliary cholangitis 29.0 MIR223 MIR21 MIR197 MIR155 ICOSLG
19 inherited metabolic disorder 28.9 SERPINA3 MIR34A MIR21 MIR155 APOE
20 acute myocardial infarction 28.8 SERPINA3 MIR34A MIR223 MIR21
21 inflammatory bowel disease 28.7 MIR223 MIR21 MIR155 ICOSLG CXCR3 CXCL9
22 immune deficiency disease 28.7 SERPINA3 MIR34A MIR223 MIR21 MIR155 MIR146B
23 systemic lupus erythematosus 28.6 MIR381 MIR223 MIR21 MIR197 MIR155 ICOSLG
24 hypertension, essential 28.4 SERPINA3 MIR223 MIR222 MIR221 MIR214 MIR21
25 facioscapulohumeral muscular dystrophy 1 28.4 MIR34A MIR222 MIR221 MIR214 MIR21 MIR155
26 connective tissue disease 28.3 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
27 renal cell carcinoma, nonpapillary 28.2 MIR34A MIR221 MIR214 MIR21 MIR155 ICOSLG
28 lipoprotein quantitative trait locus 28.2 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR21
29 myocardial infarction 28.1 SERPINA3 MIR34A MIR223 MIR221 MIR21 MIR155
30 leukemia, chronic lymphocytic 28.1 MIR34A MIR223 MIR221 MIR214 MIR21 MIR155
31 muscular dystrophy, duchenne type 27.9 MIR381 MIR34A MIR222 MIR221 MIR214 MIR21
32 central nervous system disease 27.5 SERPINA3 MIR34A MIR223 MIR221 MIR21 MIR155
33 gastrointestinal system disease 27.4 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
34 arteries, anomalies of 27.3 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
35 nervous system disease 26.3 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
36 myopathy 11.5
37 welander distal myopathy 11.2
38 inflammatory myopathy with abundant macrophages 11.1
39 juvenile idiopathic inflammatory myopathy 11.1
40 necrotizing autoimmune myopathy 11.0
41 camptocormism 11.0
42 juvenile polymyositis 11.0
43 distal myopathy with vocal cord weakness 10.9
44 dysphagia 10.9
45 neuromuscular disease 10.7
46 muscular dystrophy 10.7
47 muscular atrophy 10.6
48 amyotrophic lateral sclerosis 1 10.6
49 lateral sclerosis 10.6
50 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.5

Graphical network of the top 20 diseases related to Inclusion Body Myositis:

Diseases related to Inclusion Body Myositis

Symptoms & Phenotypes for Inclusion Body Myositis

Human phenotypes related to Inclusion Body Myositis:

58 31 (show all 16)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 skeletal muscle atrophy 58 31 hallmark (90%) Very frequent (99-80%) HP:0003202
2 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
3 elevated serum creatine kinase 58 31 hallmark (90%) Very frequent (99-80%) HP:0003236
4 emg abnormality 58 31 hallmark (90%) Very frequent (99-80%) HP:0003457
5 rimmed vacuoles 58 31 hallmark (90%) Very frequent (99-80%) HP:0003805
6 ragged-red muscle fibers 58 31 hallmark (90%) Very frequent (99-80%) HP:0003200
7 inflammatory myopathy 58 31 hallmark (90%) Very frequent (99-80%) HP:0009071
8 quadriceps muscle weakness 58 31 hallmark (90%) Very frequent (99-80%) HP:0003731
9 feeding difficulties in infancy 58 31 frequent (33%) Frequent (79-30%) HP:0008872
10 reduced tendon reflexes 58 31 frequent (33%) Frequent (79-30%) HP:0001315
11 myalgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003326
12 proximal muscle weakness 58 31 Very frequent (99-80%) HP:0003701
13 dysphagia 31 HP:0002015
14 hyporeflexia 31 HP:0001265
15 abnormality of muscle fibers 58 Very frequent (99-80%)
16 distal muscle weakness 31 HP:0002460

Symptoms via clinical synopsis from OMIM®:

57 (Updated 05-Apr-2021)
Abdomen Gastrointestinal:

Muscle Soft Tissue:
rimmed vacuoles
muscle weakness, proximal
muscle atrophy, especially quadriceps and forearm muscles
muscle weakness, distal
muscle biopsy shows inflammation with t cells

Clinical features from OMIM®:

147421 (Updated 05-Apr-2021)

UMLS symptoms related to Inclusion Body Myositis:

muscle weakness; myoclonus; back pain; ophthalmoplegia; myalgia; torticollis; sciatica; muscle cramp; muscle rigidity; muscle spasticity

Drugs & Therapeutics for Inclusion Body Myositis

Drugs for Inclusion Body Myositis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 134)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Infliximab Approved Phase 2, Phase 3 170277-31-3
Simvastatin Approved Phase 2, Phase 3 79902-63-9 54454
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
Tacrolimus Approved, Investigational Phase 3 104987-11-3 6473866 445643 439492
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
Azathioprine Approved Phase 3 446-86-6 2265
Abatacept Approved Phase 3 332348-12-6 10237
belimumab Approved Phase 2, Phase 3 356547-88-1 5957 10451420
Sodium citrate Approved, Investigational Phase 3 68-04-2
Clotrimazole Approved, Vet_approved Phase 3 23593-75-1 2812
Miconazole Approved, Investigational, Vet_approved Phase 3 22916-47-8 4189
Sirolimus Approved, Investigational Phase 3 53123-88-9 6436030 5284616
Zoledronic Acid Approved Phase 3 118072-93-8 68740
Denosumab Approved Phase 3 615258-40-7
Risedronate Approved, Investigational Phase 3 105462-24-6 5245
rituximab Approved Phase 2, Phase 3 174722-31-7 10201696
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
Coenzyme Q10 Approved, Investigational, Nutraceutical Phase 2, Phase 3 303-98-0 5281915
Tretinoin Approved, Investigational, Nutraceutical Phase 3 302-79-4 444795 5538
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
26 Pharmaceutical Solutions Phase 2, Phase 3
27 Interleukin 1 Receptor Antagonist Protein Phase 2, Phase 3
28 Trace Elements Phase 2, Phase 3
29 Nutrients Phase 2, Phase 3
30 Vitamins Phase 2, Phase 3
31 Ubiquinone Phase 2, Phase 3
32 Micronutrients Phase 2, Phase 3
33 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 2, Phase 3
34 Calcineurin Inhibitors Phase 3
35 Methylprednisolone Acetate Phase 3
36 Antimetabolites Phase 3
37 Citrate Phase 3
38 Antibiotics, Antitubercular Phase 3
39 Anti-Infective Agents Phase 3
40 Anti-Bacterial Agents Phase 3
41 Antifungal Agents Phase 3
42 Immunoglobulins Phase 3
43 Antibodies Phase 3
44 Antibodies, Monoclonal Phase 3
45 Diphosphonates Phase 3
46 Antirheumatic Agents Phase 2, Phase 3
47 Antineoplastic Agents, Immunological Phase 2, Phase 3
48 Immunologic Factors Phase 2, Phase 3
49 Immunosuppressive Agents Phase 2, Phase 3
50 Alkylating Agents Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 122)
# Name Status NCT ID Phase Drugs
1 Abatacept for the Treatment of Refractory Juvenile Dermatomyositis Completed NCT02594735 Phase 4 Abatacept
2 Safety and Tolerability Trial of Arimoclomol for Sporadic Inclusion Body Myositis Completed NCT00769860 Phase 2, Phase 3 Arimoclomol
3 An Open Trial With TNF Blockade With Infliximab (Remicade®), in Patients With Chronic Inflammatory Myopathies Completed NCT00443222 Phase 2, Phase 3 Infliximab
4 Six Month Clinical Research Study for Patients With Moderate or Severe Dry Eye Syndrome Completed NCT00025818 Phase 3 Ophthalmic Emulsion
5 An Open-label, Long-term Study to Evaluate the Safety and Tolerability of BYM338 in Patients With Sporadic Inclusion Body Myositis Completed NCT02250443 Phase 2, Phase 3 BYM338 (Bimagrumab)
6 A Randomized, Double-blind, Placebo-controlled, Multicenter, Parallel Group, Dose-finding, Pivotal, Phase 2b/3 Study to Evaluate the Efficacy, Safety, and Tolerability of Intravenous BYM338 at 52 Weeks on Physical Function, Muscle Strength, and Mobility and Additional Long Term Safety up to 2 Years in Patients With Sporadic Inclusion Body Myositis Completed NCT01925209 Phase 2, Phase 3 BYM338/bimagrumab;Placebo
7 Extension of the CBYM338B2203 Phase IIb/III Study to Evaluate the Long-term Efficacy, Safety and Tolerability of Intravenous BYM338 in Patients With Sporadic Inclusion Body Myositis Completed NCT02573467 Phase 3 Bimagrumab;Placebo
8 Anakinra in Patients With Refractory Idiopathic Inflammatory Myopathies Completed NCT01165008 Phase 2, Phase 3 Anakinra
9 Étude de l'Effet de la Rapamycine Sur la Force Musculaire et la réponse Immunitaire au Cours de la Myosite à Inclusions: étude RAPAMI" Completed NCT02481453 Phase 2, Phase 3 Rapamycin;Placebo
10 Clinical Trial of CoQ10 for Mild-to-Moderate Statin-Associated Muscle Symptoms Completed NCT01032993 Phase 2, Phase 3
11 Cyclophosphamide and Azathioprine vs Tacrolimus in Antisynthetase Syndrome-related Interstitial Lung Disease : Multicentric Randomized Phase III Trial Recruiting NCT03770663 Phase 3 Cyclophosphamide and azathioprine;Tacrolimus
12 A Phase 3, Randomized, Double-Blind Clinical Trial to Evaluate the Efficacy and Safety of Abatacept SC With Standard Treatment Compared to Standard Treatment Alone in Improving Disease Activity in Adults With Active Idiopathic Inflammatory Myopathy (IIM) Active, not recruiting NCT02971683 Phase 3 Abatacept subcutaneous;Placebo
13 Belimumab for Maintenance Therapy in Idiopathic Inflammatory Myositis Active, not recruiting NCT02347891 Phase 2, Phase 3 Belimumab;Placebo
14 A Phase 3, Efficacy and Safety Study of Oral Palovarotene for the Treatment of Fibrodysplasia Ossificans Progressiva (FOP) Active, not recruiting NCT03312634 Phase 3 Palovarotene
15 An Open-label, Non-randomized Trial to Investigate the Efficacy and Safety of Early Versus Delayed Start of Arimoclomol in Patients With Sporadic Inclusion Body Myositis Who Have Completed the IBM4809 Trial Enrolling by invitation NCT04049097 Phase 3 Arimoclomol
16 A Double-Blind Randomised Controlled Trial (dbRCT) Phase III Trial Investigating the Effect of Sirolimus on Disease Progression in Patients With Inclusion Body Myositis (IBM) as Measured by the IBM Functional Rating Scale (IBM-FRS) Not yet recruiting NCT04789070 Phase 3 Sirolimus;Placebo
17 Efficacy of Denosumab and Zoledronic Acid in the Treatment of Idiopathic Inflammatory Myopathies Related Reduced Bone Mineral Density: a Prospective Controlled Trial Not yet recruiting NCT04034199 Phase 3 Denosumab;Zoledronic Acid
18 Evaluation of Efficacy and Safety of Garetosmab in Japanese Adult Patients With Fibrodysplasia Ossificans Progressiva Not yet recruiting NCT04577820 Phase 3 garetosmab
19 A Randomized, Double Blind Controlled Trial Comparing Rituximab Against Intravenous Cyclophosphamide in Connective Tissue Disease Associated Interstitial Lung Disease Terminated NCT01862926 Phase 2, Phase 3 Rituximab;Cyclophosphamide
20 Rituximab Therapy in Refractory Adult and Juvenile Idiopathic Inflammatory Myopathy (IIM) Completed NCT00106184 Phase 2 Rituximab;Placebo
21 A Double-blind, Placebo-controlled Study to Evaluate the Safety, Tolerability, Pharmacokinetics, Pharmacodynamics and Preliminary Efficacy of BYM338 in Patients With Sporadic Inclusion Body Myositis Completed NCT01423110 Phase 2
22 Phase II Study of Arimoclomol for the Treatment of Sporadic Inclusion Body Myositis (IBM) Completed NCT02753530 Phase 2 Arimoclomol
23 The Efficacy of High-Dose Intravenous Immunoglobulin in Patients With Inflammatory Myopathies: A Three Month Randomized Trial With Option for Cross-Over Completed NCT00001261 Phase 2 Gamma Globulin
24 Effects of a T Cell-Depleting Monoclonal Antibody, Alemtuzumab, in Patients With Inclusion Body Myositis: A Pilot Clinicopathological Study Completed NCT00079768 Phase 2 Alemtuzumab (Campath)
25 FORCE: Rituximab (CD 20+-B Cell-depleting Monoclonal Antibody) for the Treatment of Refractory Inflammatory Myopathies With Specific Antibodies and Refractory Myasthenia Gravis Completed NCT00774462 Phase 2 Rituximab
26 A Phase 2 Open-label Study to Evaluate the Long-term Safety of Sifalimumab in Adult Subjects With Systemic Lupus Erythematosus or Myositis Completed NCT00979654 Phase 2 Sifalimumab
27 The Effects of High Dose Fish Oil Supplementation on Delayed Onset Muscle Soreness and Inflammatory Markers Completed NCT00805870 Phase 2 Lovaza (omega-3-acid ethyl esters);Wheat Germ Oil
28 A Phase 2 Randomized, Double-Blind, Placebo-Controlled Efficacy and Safety Study of a RARγ-Specific Agonist (Palovarotene) in the Treatment of Preosseous Flare-ups in Subjects With Fibrodysplasia Ossificans Progressiva (FOP) Completed NCT02190747 Phase 2 Palovarotene;Placebo
29 Evaluation of Novel PET Radioligands as Inflammatory Biomarkers in Rheumatoid Arthritis and Myositis Recruiting NCT03912428 Phase 1, Phase 2 Celecoxib
30 A Randomized, Controlled Pilot Trial to Evaluate the Efficacy and Safety of Subcutaneous Abatacept in Treating Interstitial Lung Disease Associated With the Anti-synthetase Syndrome Recruiting NCT03215927 Phase 2 Abatacept
31 An Open-label Study of Sodium Thiosulfate for Treatment of Calcinosis Associated With Juvenile and Adult Dermatomyositis Recruiting NCT03267277 Phase 2 Sodium Thiosulfate
32 Saracatinib Trial TO Prevent FOP Recruiting NCT04307953 Phase 2 AZD0530 Difumarate;Matching placebo
33 A Randomised, Controlled, Double-blind, Double-dummy, Clinical Trial Comparing Sacubitril-Valsartan Versus Valsartan on Left Atrial Volume Index in Asymptomatic, Stage A/B HFpEF Patients With Elevated Natriuretic Peptide and Abnormal LAVI. Active, not recruiting NCT04687111 Phase 2 Sacubitril-Valsartan;Valsartan
34 A Phase 2, Open-Label Extension, Efficacy and Safety Study of a RARγ Specific Agonist (Palovarotene) in the Treatment of Preosseous Flare-ups in Subjects With Fibrodysplasia Ossificans Progressiva (FOP) Active, not recruiting NCT02279095 Phase 2 Palovarotene dose level 1;Palovarotene dose level 2;Palovarotene dose level 3;Palovarotene dose level 4
35 A Randomized, Placebo-controlled Study to Assess the Safety, Tolerability, Pharmacokinetics, and Effects on Heterotopic Bone Formation of REGN2477 in Patients With Fibrodysplasia Ossificans Progressiva Active, not recruiting NCT03188666 Phase 2 REGN2477;Matching placebo
36 A Phase 2, Open-Label Extension, Efficacy and Safety Study of a RARγ-Specific Agonist (Palovarotene) in the Treatment of Preosseous Flare-ups in Subjects With Fibrodysplasia Ossificans Progressiva (FOP) Active, not recruiting NCT02979769 Phase 2 Palovarotene dose level 1;Palovarotene dose level 2
37 A Randomised, Phase IIa Treatment Delayed-start Trial of the Oral JAK 1/2 Inhibitor, Baricitinib, in the Treatment of Adult Idiopathic Inflammatory Myopathy Not yet recruiting NCT04208464 Phase 2 Baricitinib;Baricitinib
38 A Randomized Controlled Trial to Evaluate the Efficacy and Safety of Low-dose Interleukin-2 in Combination With Standard Therapy Compared to Standard Therapy Alone in Adults With Active Idiopathic Inflammatory Myopathy Not yet recruiting NCT04237987 Phase 2 Interleukin-2;ciclosporin and corticosteroid
39 Low-dose Interleukin-2 Treatment on Idiopathic Inflammatory Myopathy Not yet recruiting NCT04062019 Phase 2 Interleukin-2
40 A Multi-centre Double-blind, Placebo Controlled, Proof of Concept Study to Evaluate the Efficacy and Tolerability of BAF312 in Patients With Polymyositis Terminated NCT01801917 Phase 2 Placebo;BAF312
41 A Phase 2, In-Home, Safety and Efficacy Evaluation of Episodic Administration of Open-Label Palovarotene in Subjects With Fibrodysplasia Ossificans Progressiva (FOP) Terminated NCT02521792 Phase 2 Palovarotene
42 A Phase 2 Randomized, Double-Blind, Placebo-Controlled Study of the Safety and Efficacy of Intravenously Administered REGN2477+REGN1033 in Patients With Sporadic Inclusion Body Myositis Withdrawn NCT03710941 Phase 2 REGN2477+REGN1033;Matching placebo
43 Pilot Study of Natalizumab in the Treatment of Patients With Inclusion Body Myositis Unknown status NCT02483845 Phase 1 Natalizumab
44 Phase I Clinical Intramuscular Gene Transfer of rAAV1.CMV.huFollistatin344 Trial to Patients With Becker Muscular Dystrophy and Sporadic Inclusion Body Myositis. Completed NCT01519349 Phase 1
45 An Open-Label Pilot Study of Pioglitazone in Sporadic Inclusion Body Myositis Completed NCT03440034 Phase 1 Pioglitazone
46 Pharmacokinetic Study on N-acetylneuraminic Acid in Patients With Distal Myopathy With Rimmed Vacuoles (DMRV) - Hereditary Inclusion Body Myopathy (hIBM) Completed NCT01236898 Phase 1 NPC-09
47 Kinesio Taping Effects Applied With Different Directions and Tensions on Electromyography, Electroencephalography, Local Temperature and Muscle Strength Completed NCT02501915 Phase 1
48 An Open-Label Study Evaluating the Effect of Food on the Pharmacokinetics of Palovarotene and the Effect of Palovarotene on the Pharmacokinetics of the CYP3A4 Substrate Midazolam in Two Cohorts of Healthy Adult Subjects Completed NCT04829773 Phase 1 Palovarotene;midazolam
49 Safety and Tolerability of Phenylbutyrate in Inclusion Body Myositis Active, not recruiting NCT04421677 Phase 1 Phenylbutyrate Oral Tablet
50 A Phase 1, Open-Label, Ascending Dose Study of ABC008 in Adult Patients With Inclusion Not yet recruiting NCT04659031 Phase 1 ABC008

Search NIH Clinical Center for Inclusion Body Myositis

Inferred drug relations via UMLS 70 / NDF-RT 51 :

chlorphenesin carbamate
Cyclobenzaprine hydrochloride
Orphenadrine Citrate
Orphenadrine Hydrochloride
tizanidine hydrochloride

Cochrane evidence based reviews: myositis, inclusion body

Genetic Tests for Inclusion Body Myositis

Genetic tests related to Inclusion Body Myositis:

# Genetic test Affiliating Genes
1 Inclusion Body Myositis 29

Anatomical Context for Inclusion Body Myositis

MalaCards organs/tissues related to Inclusion Body Myositis:

Skeletal Muscle, Eye, T Cells, Bone, Brain, Endothelial, Heart

Publications for Inclusion Body Myositis

Articles related to Inclusion Body Myositis:

(show top 50) (show all 1635)
# Title Authors PMID Year
Inflammation induces tau pathology in inclusion body myositis model via glycogen synthase kinase-3beta. 57 61
18318434 2008
Inclusion-body myositis: a myodegenerative conformational disorder associated with Abeta, protein misfolding, and proteasome inhibition. 57 61
16432144 2006
Pilot trial of etanercept in the treatment of inclusion-body myositis. 61 57
16432140 2006
Mutant ubiquitin UBB+1 is accumulated in sporadic inclusion-body myositis muscle fibers. 61 57
15452314 2004
Transthyretin Val122Ile, accumulated Abeta, and inclusion-body myositis aspects in cultured muscle. 57 61
12874414 2003
Inclusion body myositis-like phenotype induced by transgenic overexpression of beta APP in skeletal muscle. 61 57
11972038 2002
Minimal expansion of the GCG repeat in the PABP2 gene does not predispose to sporadic inclusion body myositis. 57 61
10025815 1999
Apolipoprotein E epsilon 4 in inclusion body myositis. 57 61
8526471 1995
Distinctive patterns of microRNA expression in primary muscular disorders. 61 47
17942673 2007
Casein kinase 1 alpha associates with the tau-bearing lesions of inclusion body myositis. 61 54
18191026 2008
Raised troponin T in inclusion body myositis is common and serum levels are persistent over time. 61 54
16920359 2006
Parkin protects against mitochondrial toxins and beta-amyloid accumulation in skeletal muscle cells. 54 61
16517603 2006
Neprilysin participates in skeletal muscle regeneration and is accumulated in abnormal muscle fibres of inclusion body myositis. 61 54
16405511 2006
Difference in adhesion molecule expression (ICAM-1 and VCAM-1) in juvenile and adult dermatomyositis, polymyositis and inclusion body myositis. 61 54
16431335 2006
Age- and region-dependent alterations in Abeta-degrading enzymes: implications for Abeta-induced disorders. 54 61
15708439 2005
Proteasomal expression, induction of immunoproteasome subunits, and local MHC class I presentation in myofibrillar myopathy and inclusion body myositis. 54 61
15198127 2004
Apolipoprotein E and alpha-1-antichymotrypsin polymorphisms in sporadic inclusion body myositis. 61 54
15159602 2004
Expression of IFN-gamma-inducible chemokines in inclusion body myositis. 61 54
12965263 2003
Inclusion body myositis: morphological clues to correct diagnosis. 61 54
12398837 2002
[Expression of adhesion molecules in idiopathic inflammatory myopathies. Immunohistochemical study of 17 cases]. 54 61
10320905 1999
Molecular immunology and genetics of inflammatory muscle diseases. 54 61
9865793 1998
Human muscle cells express a functional costimulatory molecule distinct from B7.1 (CD80) and B7.2 (CD86) in vitro and in inflammatory lesions. 54 61
9834075 1998
The expression of co-stimulatory and accessory molecules on cultured human muscle cells is not dependent on stimulus by pro-inflammatory cytokines: relevance for the pathogenesis of inflammatory myopathy. 54 61
9626997 1998
Ubiquitin immunostaining and inclusion body myositis: study of 30 patients with inclusion body myositis. 61 54
9269823 1997
Apolipoprotein E and apolipoprotein E messenger RNA in muscle of inclusion body myositis and myopathies. 61 54
9007091 1996
Apolipoprotein E allele frequencies in sporadic inclusion body myositis. 61 54
8941276 1996
Cytochrome c oxidase deficiencies in the muscle of patients with inflammatory myopathies. 54 61
8740235 1996
Tau protein immunoreactivity in muscle fibers with rimmed vacuoles differs from that in regenerating muscle fibers. 61 54
8560979 1995
Twisted tubulofilaments of inclusion body myositis muscle resemble paired helical filaments of Alzheimer brain and contain hyperphosphorylated tau. 54 61
8291607 1994
MHC class I, MHC class II and intercellular adhesion molecule-1 (ICAM-1) expression in inflammatory myopathies. 61 54
7507012 1994
Ubiquitin expression in inclusion body myositis. An immunohistochemical study. 54 61
8393651 1993
Ubiquitin and beta-amyloid-protein in inclusion body myositis (IBM), familial IBM-like disorder and oculopharyngeal muscular dystrophy: an immunocytochemical study. 54 61
8268725 1993
beta-Amyloid precursor protein mRNA is increased in inclusion-body myositis muscle. 54 61
8394158 1993
Mitochondrial DNA deletions in inclusion body myositis. 61 54
8384916 1993
Rimmed vacuoles of inclusion body myositis and oculopharyngeal muscular dystrophy contain amyloid precursor protein and lysosomal markers. 54 61
8461987 1993
Immunocytochemical localization of ubiquitin in inclusion body myositis allows its light-microscopic distinction from polymyositis. 61 54
1310532 1992
Immunolocalization of ubiquitin in muscle biopsies of patients with inclusion body myositis and oculopharyngeal muscular dystrophy. 54 61
1660975 1991
The pathophysiological effects of exercise in the management of idiopathic inflammatory myopathies: A scoping review. 61
33793075 2021
Regarding Cricopharyngeal Myotomy in Inclusion Body Myositis: Comparison of Endoscopic and Transcervical Approaches. 61
33792927 2021
Anti-cN1A antibodies do not correlate with specific clinical, electromyographic, or pathological findings in sporadic inclusion body myositis. 61
33373040 2021
Determinants and functional impacts of diaphragmatic involvement in patients with inclusion body myositis. 61
33432607 2021
Challenges for Treatment Trials of Inclusion Body Myositis. 61
33597288 2021
Cricopharyngeal bar on videofluoroscopy: high specificity for inclusion body myositis. 61
32980980 2021
[Which treatment attempts should be undertaken for inclusion body myositis?] 61
33709167 2021
More prominent fibrosis of the cricopharyngeal muscle in inclusion body myositis. 61
33529855 2021
Efficacy and Safety of Bimagrumab in Sporadic Inclusion Body Myositis: Long-term Extension of RESILIENT. 61
33597289 2021
Clinical utility of anti-cytosolic 5'-nucleotidase 1A antibody in idiopathic inflammatory myopathies. 61
33556224 2021
[Clinicopathological Features of Myositis and Necrotizing Myopathy: How to Distinguish between Myositis and Muscular Dystrophy on Muscle Pathology]. 61
33561829 2021
Skeletal Muscle Magnetic Resonance Biomarkers in GNE Myopathy. 61
33219145 2021
Role of MRI in idiopathic inflammatory myopathies: a review article. 61
33554607 2021

Variations for Inclusion Body Myositis

ClinVar genetic disease variations for Inclusion Body Myositis:

# Gene Name Type Significance ClinVarId dbSNP ID Position
1 GNE Deletion Pathogenic 438624 GRCh37: 9:36259402-36266483
GRCh38: 9:36259405-36266486

Expression for Inclusion Body Myositis

Search GEO for disease gene expression data for Inclusion Body Myositis.

Pathways for Inclusion Body Myositis

Pathways related to Inclusion Body Myositis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.66 MIR34A MIR223 MIR222 MIR221 MIR214 MIR21
2 11.53 MIR381 MIR21 MAPT APP APOE
4 10.91 MIR222 MIR221 MIR146B

GO Terms for Inclusion Body Myositis

Cellular components related to Inclusion Body Myositis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.91 SERPINA3 MIR34A MIR223 MIR21 MIR197 MIR155
2 extracellular vesicle GO:1903561 9.43 MIR34A MIR222 MIR221 MIR214 MIR21 APOE
3 extracellular space GO:0005615 9.4 SERPINA3 MIR381 MIR223 MIR222 MIR221 MIR21
4 main axon GO:0044304 9.16 MAPT APP

Biological processes related to Inclusion Body Myositis according to GeneCards Suite gene sharing:

(show all 38)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 10.06 MIR34A MIR223 MIR21 MIR155 APP
2 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.92 MIR222 MIR221 MIR21 APP APOE
3 miRNA mediated inhibition of translation GO:0035278 9.86 MIR222 MIR221 MIR21 MIR155
4 cholesterol homeostasis GO:0042632 9.85 MIR34A MIR155 APOE
5 positive regulation of inflammatory response GO:0050729 9.85 MIR21 MIR155 APP
6 negative regulation of cell migration involved in sprouting angiogenesis GO:0090051 9.81 MIR221 MIR155 MIR146B
7 positive regulation of epithelial to mesenchymal transition GO:0010718 9.8 MIR222 MIR221 MIR21
8 negative regulation of vascular smooth muscle cell proliferation GO:1904706 9.8 MIR34A MIR223 MIR214
9 negative regulation of inflammatory response GO:0050728 9.8 MIR223 MIR222 MIR221 MIR155 APOE
10 positive regulation of G1/S transition of mitotic cell cycle GO:1900087 9.79 MIR222 MIR221 MIR214
11 negative regulation of endothelial cell proliferation GO:0001937 9.78 MIR21 CXCR3 APOE
12 negative regulation of cytokine production involved in inflammatory response GO:1900016 9.77 MIR222 MIR221 MIR155
13 positive regulation of activated T cell proliferation GO:0042104 9.74 MIR21 MIR155 ICOSLG
14 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.73 MIR222 MIR221 MIR214 MIR21
15 negative regulation of angiogenesis GO:0016525 9.72 MIR34A MIR222 MIR214 MIR21 CXCR3
16 negative regulation of gene expression GO:0010629 9.7 MIR34A MIR214 MIR21 MIR155 MAPT APP
17 negative regulation of necroptotic process GO:0060546 9.69 MIR221 MIR214 MIR155
18 astrocyte activation GO:0048143 9.68 MAPT APP
19 negative regulation of long-term synaptic potentiation GO:1900272 9.67 APP APOE
20 negative regulation of cell adhesion molecule production GO:0060354 9.67 MIR222 MIR221 MIR155
21 amyloid fibril formation GO:1990000 9.66 MAPT APP
22 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.65 MIR214 MIR155
23 positive regulation of axon regeneration GO:0048680 9.65 MIR222 MIR221
24 negative regulation of vascular wound healing GO:0061044 9.65 MIR34A MIR155
25 positive regulation of connective tissue replacement GO:1905205 9.65 MIR34A MIR214 MIR155
26 negative regulation of regulatory T cell differentiation GO:0045590 9.64 MIR21 MIR155
27 negative regulation of necrotic cell death GO:0060547 9.63 MIR223 MIR155
28 negative regulation by host of viral genome replication GO:0044828 9.63 MIR222 MIR221 MIR155
29 positive regulation of amyloid fibril formation GO:1905908 9.61 APP APOE
30 positive regulation of vascular smooth muscle cell dedifferentiation GO:1905176 9.59 MIR221 MIR214
31 negative regulation of TRAIL-activated apoptotic signaling pathway GO:1903122 9.58 MIR222 MIR221
32 positive regulation of Schwann cell migration GO:1900149 9.58 MIR222 MIR221
33 positive regulation of Schwann cell proliferation involved in axon regeneration GO:1905046 9.55 MIR222 MIR221
34 negative regulation of hematopoietic stem cell proliferation GO:1902034 9.54 MIR222 MIR221
35 negative regulation of leukocyte adhesion to vascular endothelial cell GO:1904995 9.54 MIR222 MIR221 MIR155
36 negative regulation of interleukin-21 production GO:0032705 9.43 MIR222 MIR221 MIR21
37 gene silencing by miRNA GO:0035195 9.32 MIR381 MIR34A MIR223 MIR222 MIR221 MIR214
38 negative regulation of vascular associated smooth muscle cell migration GO:1904753 9.26 MIR34A MIR223 MIR214 MIR21

Molecular functions related to Inclusion Body Myositis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.23 MIR34A MIR223 MIR222 MIR221 MIR214 MIR21
2 lipoprotein particle binding GO:0071813 8.96 MAPT APOE

Sources for Inclusion Body Myositis

9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
31 HPO
32 ICD10
33 ICD10 via Orphanet
37 LifeMap
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
69 Tocris
71 UMLS via Orphanet
Loading form....