Inclusion Body Myositis (IBM)

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

Aliases & Classifications for Inclusion Body Myositis

MalaCards integrated aliases for Inclusion Body Myositis:

Name: Inclusion Body Myositis 56 12 52 53 58 36 29 6 15 17 71
Hereditary Inclusion Body Myopathy 12 74 52 25
Nonaka Myopathy 12 52 25 71
Inclusion Body Myopathy, Autosomal Recessive 52 25 71
Distal Myopathy with Rimmed Vacuoles 12 52 25
Sporadic Inclusion Body Myositis 52 58 54
Inclusion Body Myopathy 2 12 52 25
Inclusion Body Myopathy 3 12 52 36
Hibm 12 52 25
Ibm2 12 52 25
Ibm 56 52 58
Inclusion Body Myopathy, Quadriceps-Sparing 52 25
Myositis, Inclusion Body 74 43
Rimmed Vacuole Myopathy 52 25
Dmrv 52 25
Qsm 52 25
Myopathy with Congenital Joint Contractures, Ophthalmoplegia, and Rimmed Vacuoles 52
Hereditary Inclusion Body Myopathy - Joint Contractures - Ophthalmoplegia 52
Inclusion Body Myopathy, Autosomal Dominant 71
Inclusion Body Myopathy Autosomal Dominant 52
Hereditary Inclusion Body Myopathy Type 3 52
Inclusion Body Myopathy, Sporadic 71
Myopathy, Inclusion Body, Type 2 39
Myopathy, Inclusion Body, Type 3 39
Distal Myopathy, Nonaka Type 12
Quadriceps Sparing Myopathy 52
Inflammatory Myopathies 53
Myositis Inclusion Body 54
Inflammatory Myopathy 52
Gne Myopathy 52
Myositis 71
Ibm-3 12
Hibm3 52
Ibm3 52
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;


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 56 147421
ICD9CM 34 359.71
MeSH 43 D018979
NCIt 49 C84786
SNOMED-CT 67 72315009
ICD10 32 G72.4 G72.41
ICD10 via Orphanet 33 M60.8
UMLS via Orphanet 72 C0238190 C0751713
Orphanet 58 ORPHA611
MedGen 41 C0238190
UMLS 71 C0027121 C0238190 C0751713 more

Summaries for Inclusion Body Myositis

Genetics Home Reference : 25 Inclusion body myopathy 2 is a condition that primarily affects skeletal muscles, which are muscles that the body uses for movement. This disorder causes muscle weakness that appears in late adolescence or early adulthood and worsens over time. The first sign of inclusion body myopathy 2 is weakness of a muscle in the lower leg called the tibialis anterior. This muscle helps control up-and-down movement of the foot. Weakness in the tibialis anterior alters the way a person walks and makes it difficult to run and climb stairs. As the disorder progresses, weakness also develops in muscles of the upper legs, hips, shoulders, and hands. Unlike most forms of myopathy, inclusion body myopathy 2 usually does not affect the quadriceps, which are a group of large muscles at the front of the thigh. This condition also does not affect muscles of the eye or heart, and it does not cause neurological problems. Weakness in leg muscles makes walking increasingly difficult, and most people with inclusion body myopathy 2 require wheelchair assistance within 20 years after signs and symptoms appear. People with the characteristic features of inclusion body myopathy 2 have been described in several different populations. When the condition was first reported in Japanese families, researchers called it distal myopathy with rimmed vacuoles (DMRV) or Nonaka myopathy. When a similar disorder was discovered in Iranian Jewish families, researchers called it rimmed vacuole myopathy or hereditary inclusion body myopathy (HIBM). It has since become clear that these conditions are variations of a single disorder caused by mutations in the same gene.

MalaCards based summary : Inclusion Body Myositis, also known as hereditary inclusion body myopathy, is related to myositis and polymyositis, and has symptoms including muscle weakness, myoclonus and myalgia. 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 Tight junction and MicroRNAs in cancer. The drugs Oseltamivir and Telbivudine have been mentioned in the context of this disorder. Affiliated tissues include skeletal muscle, t cells and heart, and related phenotypes are elevated serum creatine kinase and emg abnormality

Disease Ontology : 12 A myositis that is characterized by slowly progressive weakness and wasting of both distal and proximal muscles, most apparent in the muscles of the arms and legs that can occur in a sporadic or hereditary forms.

NIH Rare Diseases : 52 Inclusion body myopathy 2 , also known as hereditary inclusion body myopathy (HIBM), GNE -related myopathy, distal myopathy with rimmed vacuoles, and Nonaka myopathy, is an inherited condition that primarily affects the skeletal muscles (the muscles that the body uses to move). This disorder is characterized by muscle weakness that appears in late adolescence or early adulthood and worsens over time. Early symptoms typically develop in the 20s and 30s and may include difficulty running or walking, tripping, weakness in the index finger, and frequent loss of balance. Inclusion body myopathy 2 is caused by mutations in the GNE gene . The condition is inherited in an autosomal recessive manner. Treatment is focused on managing individual symptoms.

OMIM : 56 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)

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 myopathy 3 (IBM3) is an autosomal dominant myopathy associated with a heterozygous missense mutation in the myosin heavy chain (MyHC) IIa gene (MYH2), changing the highly conserved and negatively charged glutamate at position 706 to the positively charged lysine (E706K). This region is important for myosin functioning during muscle contraction, because it undergoes conformational changes during adenosine triphosphate (ATP) hydrolysis. Clinical characteristics include congenital joint contractures, a progressive course in adulthood, and external ophthalmoplegia.

Wikipedia : 74 Hereditary inclusion body myopathies (HIBM) are a group of rare genetic disorders which have different... 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 601)
# Related Disease Score Top Affiliating Genes
2 polymyositis 31.5 MIR222 MIR221 MIR214 MIR21 MIR155 MIR146B
3 amyloidosis 31.2 SERPINA3 MAPT APP APOE
4 prion disease 31.0 SERPINA3 MAPT APP APOE
5 dementia 30.8 SERPINA3 RPS27A MAPT APP APOE
6 creutzfeldt-jakob disease 30.7 SERPINA3 MAPT APP APOE
7 frontotemporal dementia 30.7 RPS27A MAPT GNE APP APOE
8 sarcoidosis 1 30.7 SERPINA3 MIR155 ICOSLG CXCR3 CXCL9
9 peripheral nervous system disease 30.6 SERPINA3 MIR21 ICOSLG APP
10 multiple system atrophy 1 30.3 SERPINA3 RPS27A MAPT
11 down syndrome 30.1 SERPINA3 MIR155 MAPT APP APOE
12 parkinson disease, late-onset 30.0 SERPINA3 RPS27A MIR21 MAPT APP APOE
13 muscular disease 30.0 SERPINA3 MIR221 ICOSLG GNE
14 alzheimer disease 29.9 SERPINA3 RPS27A MIR34A MIR197 MAPT BACE1-AS
15 bacterial infectious disease 29.8 SERPINA3 MIR155 ICOSLG CXCL9
16 rectum cancer 29.4 MIR221 MIR214 MIR21
17 skin disease 29.4 SERPINA3 MIR21 MIR155 ICOSLG CXCR3 CXCL9
18 coronary heart disease 1 29.3 MIR222 MIR221 MIR155 APOE
19 pulmonary fibrosis, idiopathic 29.3 MIR214 MIR21 ICOSLG CXCR3 CXCL9
20 inherited metabolic disorder 28.7 SERPINA3 MIR34A MIR21 MIR155 APOE
21 acute myocardial infarction 28.4 SERPINA3 MIR34A MIR223 MIR21
22 systemic lupus erythematosus 28.1 MIR381 MIR223 MIR21 MIR197 MIR155 ICOSLG
23 inflammatory bowel disease 28.1 MIR223 MIR21 MIR155 ICOSLG CXCR3 CXCL9
24 dermatomyositis 27.8 MIR381 MIR34A MIR223 MIR222 MIR221 MIR214
25 renal cell carcinoma, nonpapillary 27.7 MIR34A MIR221 MIR214 MIR21 MIR155 ICOSLG
26 facioscapulohumeral muscular dystrophy 1 27.7 MIR34A MIR222 MIR221 MIR214 MIR21 MIR155
27 myocardial infarction 27.3 SERPINA3 MIR34A MIR223 MIR221 MIR21 MIR155
28 leukemia, chronic lymphocytic 27.2 MIR34A MIR223 MIR221 MIR214 MIR21 MIR155
29 connective tissue disease 27.0 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
30 muscular dystrophy, duchenne type 27.0 MIR381 MIR34A MIR222 MIR221 MIR214 MIR21
31 central nervous system disease 26.4 SERPINA3 MIR34A MIR223 MIR221 MIR21 MIR155
32 gastrointestinal system disease 26.3 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
33 arteries, anomalies of 26.1 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
34 nervous system disease 25.7 SERPINA3 MIR34A MIR223 MIR222 MIR221 MIR214
35 ovarian cancer 25.6 SERPINA3 MIR381 MIR34A MIR223 MIR222 MIR221
36 hereditary inclusion body myopathy type 4 12.6
37 inflammatory myopathy with abundant macrophages 12.5
38 juvenile idiopathic inflammatory myopathy 12.4
39 myopathy 11.9
40 necrotizing autoimmune myopathy 11.8
41 welander distal myopathy 11.7
42 juvenile polymyositis 11.5
43 myopathy, myofibrillar, 9, with early respiratory failure 11.4
44 hereditary proximal myopathy with early respiratory failure 11.2
45 camptocormism 11.1
46 distal myopathy with vocal cord weakness 11.1
47 dysphagia 10.8
48 residual stage of open angle glaucoma 10.6 APP APOE
49 spinal muscular atrophy type 0 10.6 RPS27A MAPT
50 senile plaque formation 10.6 APP APOE

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 elevated serum creatine kinase 58 31 hallmark (90%) Very frequent (99-80%) HP:0003236
2 emg abnormality 58 31 hallmark (90%) Very frequent (99-80%) HP:0003457
3 skeletal muscle atrophy 58 31 hallmark (90%) Very frequent (99-80%) HP:0003202
4 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
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:

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:


UMLS symptoms related to Inclusion Body Myositis:

muscle weakness, myoclonus, myalgia, back pain, ophthalmoplegia, 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 171)
# Name Status Phase Clinical Trials Cas Number PubChem Id
Oseltamivir Approved Phase 4 204255-11-8, 196618-13-0 65028
Telbivudine Approved, Investigational Phase 4 3424-98-4 159269
Entecavir Approved, Investigational Phase 4 142217-69-4 153941
Simvastatin Approved Phase 4 79902-63-9 54454
Ezetimibe Approved Phase 4 163222-33-1 150311
Nicotinamide Approved, Investigational Phase 4 98-92-0 936
Abatacept Approved Phase 4 332348-12-6 10237
Atorvastatin Approved Phase 4 134523-00-5 60823
Folic acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
Melatonin Approved, Nutraceutical, Vet_approved Phase 4 73-31-4 896
Niacin Approved, Investigational, Nutraceutical Phase 4 59-67-6 938
Tenofovir Experimental, Investigational Phase 4 147127-20-6 464205
13 Anti-Infective Agents Phase 4
14 Anti-HIV Agents Phase 4
15 Antiviral Agents Phase 4
16 Anti-Retroviral Agents Phase 4
17 Reverse Transcriptase Inhibitors Phase 4
18 Vitamin B Complex Phase 4
19 Folate Phase 4
20 Vitamin B9 Phase 4
21 Protective Agents Phase 4
22 Antioxidants Phase 4
23 Trace Elements Phase 4
24 Vitamins Phase 4
25 Nutrients Phase 4
26 Micronutrients Phase 4
27 Ezetimibe, Simvastatin Drug Combination Phase 4
28 Liver Extracts Phase 4
29 Vasodilator Agents Phase 4
30 Nicotinic Acids Phase 4
31 Vitamin B3 Phase 4
32 Antimetabolites Phase 4
33 Hypolipidemic Agents Phase 4
34 Lipid Regulating Agents Phase 4
35 Anticholesteremic Agents Phase 4
36 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
Sirolimus Approved, Investigational Phase 2, Phase 3 53123-88-9 5284616 6436030 46835353
Infliximab Approved Phase 2, Phase 3 170277-31-3
leucovorin Approved Phase 3 58-05-9 6006 143
Methotrexate Approved Phase 3 59-05-2, 1959-05-2 126941
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
Lenograstim Approved, Investigational Phase 3 135968-09-1
Sargramostim Approved, Investigational Phase 3 83869-56-1, 123774-72-1
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
Celecoxib Approved, Investigational Phase 3 169590-42-5 2662
belimumab Approved Phase 2, Phase 3 356547-88-1 10451420 5957
Sodium citrate Approved, Investigational Phase 3 68-04-2
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5

Interventional clinical trials:

(show top 50) (show all 191)
# Name Status NCT ID Phase Drugs
1 Efficacy and Adverse Effect of Simvastatin Compare to Rosuvastatin in SLE Patients With Corticosteroid Therapy and High LDL Cholesterol Level Unknown status NCT00866229 Phase 4 Rosuvastatin;Simvastatin
2 Banlangen Granules Anti-seasonal Influenza Study: a Randomized, Double Blind, Positive and Placebo Controlled,Clinical Study. Unknown status NCT02232945 Phase 4 placebo of oseltamivir phosphate;oseltamivir phosphate;Banlangen (Radix Isatidis) granules;placebo of Banlangen(Radix Isatidis) granules
3 A Randomized, Prospective, Multicenter, Open-label Study to Evaluate the Efficacy of Telbivudine With or Without add-on Tenofovir According to Roadmap Strategy Compare With Entecavir in HBeAg-positive Chronic Hepatitis B Patients Unknown status NCT01588912 Phase 4 Telbivudine;Tenofovir;Entecavir
4 Effects of High Dose Simvastatin vs. Atorvastatin on Baseline Lipoprotein Profiles, Apo-A-1 and C Reactive Protein Unknown status NCT00736463 Phase 4 Simvastatin;Atorvastatin 80 mg
5 Rho-kinase in Patients With Atherosclerosis: Effects of Statins A Randomized Clinical Trial Comparing Ezetimibe/Simvastatin and Simvastatin Completed NCT00560170 Phase 4 Simvastatin (Zocor);10mg/10mg of Ezetimibe/Simvastatin (Vytorin)
6 The Effects of Melatonin on Elevated Liver Enzymes During Statins Treatment Completed NCT03121521 Phase 4 Melatonin 10 mg;Placebo
7 Magnetic Resonance Characterization of Carotid Atherosclerotic Plaque in Vivo: Effect of High Density Lipoprotein Elevation on Plaque Morphology Completed NCT00307307 Phase 4 Niacin/simvastatin compared to simvastatin alone at 2 doses
8 Abatacept for the Treatment of Refractory Juvenile Dermatomyositis Recruiting NCT02594735 Phase 4 Abatacept
9 Double Blind, Prospective Randomized, Crossover Study of Patients With Muscle Complaints on Statin Therapy Terminated NCT00127335 Phase 4 cellulose placebo vs. atorvastatin
10 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
11 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)
12 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
13 A Phase 3 Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Efficacy and Safety of Sialic Acid Extended-Release Tablets in Patients With GNE Myopathy (GNEM) or Hereditary Inclusion Body Myopathy (HIBM) Completed NCT02377921 Phase 3 aceneuramic acid extended-release (Ace-ER);Placebo
14 Safety and Tolerability Trial of Arimoclomol for Sporadic Inclusion Body Myositis Completed NCT00769860 Phase 2, Phase 3 Arimoclomol
15 Anakinra in Patients With Refractory Idiopathic Inflammatory Myopathies Completed NCT01165008 Phase 2, Phase 3 Anakinra
16 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
17 An Open Trial With TNF Blockade With Infliximab (Remicade®), in Patients With Chronic Inflammatory Myopathies Completed NCT00443222 Phase 2, Phase 3 Infliximab
18 A Prospective, Randomised, Assessor-blind, Multicenter Study of Efficacy and Safety of Combined Treatment of Methotrexate + Glucocorticoids Versus Glucocorticoids Alone in Patients With Polymyositis and Dermatomyositis. Completed NCT00651040 Phase 3 Prednisone;Methotrexate
19 Six Month Clinical Research Study for Patients With Moderate or Severe Dry Eye Syndrome Completed NCT00025818 Phase 3 Ophthalmic Emulsion
20 Clinical Trial of CoQ10 for Mild-to-Moderate Statin-Associated Muscle Symptoms Completed NCT01032993 Phase 2, Phase 3
21 Effect of 80-mg Atorvastatin on Myocardial Edema Following Coronary Artery Bypass Surgery in Relation With Follistatin-Like Protein-1 Completed NCT02901379 Phase 3 Atorvastatin 80mg;Atorvastatin 10mg
22 High Dose Cyclophosphamide for Treatment of Systemic Sclerosis (Scleroderma) Completed NCT00501995 Phase 3 IV Cyclophosphamide
23 A Double Blind Randomized Control Trial of Tadalafil in Interstitial Lung Disease of Scleroderma Completed NCT01553981 Phase 3 Tadalafil;Placebo
24 High-dose Simvastatin for Aneurysmal Subarachnoid Haemorrhage: Is it Better? Completed NCT01077206 Phase 2, Phase 3 Simvastatin
25 Evaluation of Novel PET Radioligands as Inflammatory Biomarkers in Rheumatoid Arthritis and Myositis Recruiting NCT03912428 Phase 3 Celecoxib
26 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
27 Belimumab for Maintenance Therapy in Idiopathic Inflammatory Myositis Active, not recruiting NCT02347891 Phase 2, Phase 3 Belimumab;Placebo
28 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
29 A Randomized, Double Blind Controlled Trial Comparing Rituximab Against Intravenous Cyclophosphamide in Connective Tissue Disease Associated Interstitial Lung Disease Active, not recruiting NCT01862926 Phase 2, Phase 3 Rituximab;Cyclophosphamide
30 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
31 Cyclophosphamide and Azathioprine vs Tacrolimus in Antisynthetase Syndrome-related Interstitial Lung Disease : Multicentric Randomized Phase III Trial Not yet recruiting NCT03770663 Phase 3 Cyclophosphamide and azathioprine;Tacrolimus
32 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
33 Intraorbital Injection Versus Oral Steroid in Patients With Anterior Idiopathic Orbital Inflammation: A Randomized Clinical Trial Not yet recruiting NCT03958344 Phase 3 Prednisolone;Triamcinolone + Betamethason
34 Phase 3B Open-Label Extension Study to Evaluate the Safety and Efficacy of Aceneuramic Acid Extended-Release (Ace-ER) Tablets in Patients With GNE Myopathy (GNEM) or Hereditary Inclusion Body Myopathy (HIBM) Terminated NCT02736188 Phase 3 Aceneuramic Acid Extended-Release Tablets
35 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
36 A Phase 2 Randomized, Double-Blind, Placebo-Controlled, Parallel Group Study to Evaluate the Dose and Pharmacodynamic Efficacy of Sialic Acid-Extended Release (SA-ER) Tablets in Patients With GNE Myopathy or Hereditary Inclusion Body Myopathy Completed NCT01517880 Phase 2 Sialic Acid Extended Release (SA-ER);Placebo
37 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)
38 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
39 An Open-Label Phase 2 Study of ManNAc in Subjects With GNE Myopathy Completed NCT02346461 Phase 2 ManNAc;ManNAc
40 An Open-label Phase 2 Extension Study to Evaluate the Long Term Safety and Efficacy of Sialic Acid-Extended Release (SA-ER) Tablets and Sialic Acid-Immediate Release (SA-IR) Capsules in Patients With GNE Myopathy or Hereditary Inclusion Body Myopathy Completed NCT01830972 Phase 2 SA-ER 500 mg;SA-IR 500 mg
41 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
42 A Phase 2 Study to Evaluate the Efficacy of Sialic Acid-Extended Release (SA-ER) Tablets in Patients With GNE Related Thrombocytopenia Completed NCT02845609 Phase 2 Sialic Acid-Extended Release
43 Rituximab Therapy in Refractory Adult and Juvenile Idiopathic Inflammatory Myopathy (IIM) Completed NCT00106184 Phase 2 Rituximab;Placebo
44 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
45 Tocilizumab in the Treatment of Refractory Polymyositis and Dermatomyositis Completed NCT02043548 Phase 2 tocilizumab;placebo
46 Statins and Lupus: Effects of Statins on Clinical Lupus Parameters, Serological Markers and Toll-like Receptors. Completed NCT00519363 Phase 1, Phase 2 atorvastatin
47 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
48 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
49 A Double-blind, Randomized Study to Evaluate the Efficacy and Safety of TAK-475 or Placebo When Coadministered With Fenofibrate in Subjects With Combined Hyperlipidemia Completed NCT00813527 Phase 2 Lapaquistat acetate and fenofibrate;Fenofibrate
50 Novel Drug Delivery of Sodium Thiosulfate for Calcinosis Associated With Adult and Juvenile Dermatomyositis Completed NCT01572844 Phase 2 Sodium thiosulfate

Search NIH Clinical Center for Inclusion Body Myositis

Inferred drug relations via UMLS 71 / NDF-RT 50 :

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, T Cells, Heart, Eye, Lung, Bone, Testes

Publications for Inclusion Body Myositis

Articles related to Inclusion Body Myositis:

(show top 50) (show all 1587)
# Title Authors PMID Year
Inflammation induces tau pathology in inclusion body myositis model via glycogen synthase kinase-3beta. 56 61
18318434 2008
Pilot trial of etanercept in the treatment of inclusion-body myositis. 56 61
16432140 2006
Inclusion-body myositis: a myodegenerative conformational disorder associated with Abeta, protein misfolding, and proteasome inhibition. 56 61
16432144 2006
Mutant ubiquitin UBB+1 is accumulated in sporadic inclusion-body myositis muscle fibers. 61 56
15452314 2004
Transthyretin Val122Ile, accumulated Abeta, and inclusion-body myositis aspects in cultured muscle. 61 56
12874414 2003
Inclusion body myositis-like phenotype induced by transgenic overexpression of beta APP in skeletal muscle. 56 61
11972038 2002
Minimal expansion of the GCG repeat in the PABP2 gene does not predispose to sporadic inclusion body myositis. 61 56
10025815 1999
Apolipoprotein E epsilon 4 in inclusion body myositis. 56 61
8526471 1995
Distinctive patterns of microRNA expression in primary muscular disorders. 61 46
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 allele frequencies in sporadic inclusion body myositis. 61 54
8941276 1996
Apolipoprotein E and apolipoprotein E messenger RNA in muscle of inclusion body myositis and myopathies. 54 61
9007091 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. 54 61
8560979 1995
Twisted tubulofilaments of inclusion body myositis muscle resemble paired helical filaments of Alzheimer brain and contain hyperphosphorylated tau. 61 54
8291607 1994
MHC class I, MHC class II and intercellular adhesion molecule-1 (ICAM-1) expression in inflammatory myopathies. 54 61
7507012 1994
Ubiquitin expression in inclusion body myositis. An immunohistochemical study. 61 54
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. 54 61
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. 54 61
1310532 1992
Immunolocalization of ubiquitin in muscle biopsies of patients with inclusion body myositis and oculopharyngeal muscular dystrophy. 61 54
1660975 1991
Long-term strength and functional status in inclusion body myositis and identification of trajectory subgroups. 61
32134516 2020
Diving into the Heterogeneity of Inclusion Body Myositis. 61
32319105 2020
Ultrasound can differentiate inclusion body myositis from disease mimics. 61
32239702 2020
Bioinformatics analysis of gene expression profiles of Inclusion body myositis. 61
32259312 2020
Myopathies with finger flexor weakness: Not only inclusion-body myositis. 61
32478919 2020
Inclusion body myositis in patients with spinocerebellar ataxia types 3 and 6. 61
32576615 2020
Attitudes Toward Noninterventional Observational Studies in US and Australian Patients With Sporadic Inclusion Body Myositis. 61
32453104 2020
Obstruction-related dysphagia in inclusion body myositis: Cricopharyngeal bar on videofluoroscopy indicates risk of aspiration. 61
32172012 2020
Concurrent anti-PM-Scl antibody-associated systemic sclerosis and inclusion body myositis - report of two cases and review of the literature. 61
31806155 2020
Performance of the new EULAR/ACR classification criteria for idiopathic inflammatory myopathies (IIM) in a large monocentric IIM cohort. 61
32024593 2020
Increased intra-mitochondrial lipofuscin aggregates with spherical dense body formation in mitochondrial myopathy. 61
32272361 2020
Myopathies featuring early or prominent dysphagia. 61
32510670 2020
Juvenile Dermatomyositis and the Inflammatory Myopathies. 61
32252099 2020

Variations for Inclusion Body Myositis

ClinVar genetic disease variations for Inclusion Body Myositis:

6 ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 GNE deletion Pathogenic 438624 9:36259402-36266483 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 KEGG:

# Name Kegg Source Accession
1 Tight junction hsa04530

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 space GO:0005615 9.44 SERPINA3 RPS27A MIR381 MIR223 MIR222 MIR221
2 extracellular vesicle GO:1903561 9.43 MIR34A MIR222 MIR221 MIR214 MIR21 APOE
3 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 10.05 MIR34A MIR223 MIR21 MIR155 APP
2 negative regulation of gene expression GO:0010629 9.95 MIR214 MIR21 MIR155 MAPT APP APOE
3 positive regulation of ERK1 and ERK2 cascade GO:0070374 9.92 MIR222 MIR221 MIR21 APP APOE
4 negative regulation of angiogenesis GO:0016525 9.9 MIR34A MIR222 MIR214 MIR21
5 cholesterol homeostasis GO:0042632 9.85 MIR34A MIR155 APOE
6 miRNA mediated inhibition of translation GO:0035278 9.84 MIR222 MIR221 MIR21 MIR155
7 negative regulation of cell migration involved in sprouting angiogenesis GO:0090051 9.81 MIR221 MIR155 MIR146B
8 positive regulation of G1/S transition of mitotic cell cycle GO:1900087 9.8 MIR222 MIR221 MIR214
9 positive regulation of epithelial to mesenchymal transition GO:0010718 9.8 MIR222 MIR221 MIR21
10 negative regulation of inflammatory response GO:0050728 9.8 MIR223 MIR222 MIR221 MIR155 APOE
11 negative regulation of vascular smooth muscle cell proliferation GO:1904706 9.79 MIR34A MIR223 MIR214
12 negative regulation of cytokine production involved in inflammatory response GO:1900016 9.78 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 necroptotic process GO:0060546 9.69 MIR221 MIR214 MIR155
16 astrocyte activation GO:0048143 9.68 MAPT APP
17 negative regulation of long-term synaptic potentiation GO:1900272 9.67 APP APOE
18 amyloid fibril formation GO:1990000 9.67 MAPT APP
19 positive regulation of connective tissue replacement GO:1905205 9.67 MIR34A MIR214 MIR155
20 virion assembly GO:0019068 9.66 RPS27A APOE
21 positive regulation of cardiac muscle hypertrophy in response to stress GO:1903244 9.66 MIR214 MIR155
22 positive regulation of axon regeneration GO:0048680 9.65 MIR222 MIR221
23 negative regulation of vascular wound healing GO:0061044 9.65 MIR34A MIR155
24 negative regulation of regulatory T cell differentiation GO:0045590 9.64 MIR21 MIR155
25 negative regulation of necrotic cell death GO:0060547 9.63 MIR223 MIR155
26 negative regulation of cell adhesion molecule production GO:0060354 9.63 MIR222 MIR221 MIR155
27 positive regulation of amyloid fibril formation GO:1905908 9.61 APP APOE
28 positive regulation of vascular smooth muscle cell dedifferentiation GO:1905176 9.59 MIR221 MIR214
29 negative regulation of TRAIL-activated apoptotic signaling pathway GO:1903122 9.58 MIR222 MIR221
30 positive regulation of Schwann cell migration GO:1900149 9.58 MIR222 MIR221
31 positive regulation of Schwann cell proliferation involved in axon regeneration GO:1905046 9.56 MIR222 MIR221
32 negative regulation of hematopoietic stem cell proliferation GO:1902034 9.55 MIR222 MIR221
33 negative regulation of leukocyte adhesion to vascular endothelial cell GO:1904995 9.54 MIR222 MIR221 MIR155
34 negative regulation by host of viral genome replication GO:0044828 9.5 MIR222 MIR221 MIR155
35 negative regulation of interleukin-21 biosynthetic process GO:0045391 9.43 MIR222 MIR221 MIR21
36 negative regulation of interleukin-21 production GO:0032705 9.33 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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
54 Novoseek
57 OMIM via Orphanet
61 PubMed
70 Tocris
72 UMLS via Orphanet
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