NAM
MCID: NCR015
MIFTS: 32

Necrotizing Autoimmune Myopathy (NAM)

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

Aliases & Classifications for Necrotizing Autoimmune Myopathy

MalaCards integrated aliases for Necrotizing Autoimmune Myopathy:

Name: Necrotizing Autoimmune Myopathy 20
Immune Myopathy with Myocyte Necrosis 20 58
Immune-Mediated Necrotizing Myopathy 20 58
Autoimmune Necrotizing Myositis 20 58
Anti-Hmg-Coa Myopathy 20 58
Anti-Srp Myopathy 20 58
Imnm 20 58
Nam 20 58

Characteristics:

Orphanet epidemiological data:

58
immune-mediated necrotizing myopathy
Inheritance: Not applicable; Age of onset: Adult,Elderly;

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare systemic and rhumatological diseases


External Ids:

ICD10 via Orphanet 33 G72.4
UMLS via Orphanet 71 C3267047
Orphanet 58 ORPHA206569

Summaries for Necrotizing Autoimmune Myopathy

GARD : 20 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 206569 Definition Necrotizing autoimmune myopathy (NAM) is a rare form of idiopathic inflammatory myopathy characterized clinically by acute or subacute proximal muscle weakness, and histopathologically by myocyte necrosis and regeneration without significant inflammation. Epidemiology The prevalence and annual incidence of NAM are not known but the disorder is very rare. About 300 cases have been reported to date. Clinical description Age of onset ranges from 30 to 70 years of age in reported cases. The main presenting feature of NAM is subacute severe symmetrical proximal myopathy, with a markedly elevated creatine kinase (CK) level. Its presentation is similar to that of polymyositis (see this term) with upper and lower limb weakness causing difficulty in moving from a sitting position, climbing stairs, or lifting objects The neck flexor, pharyngeal, and respiratorymuscles may also be involved. Other manifestations include fatigue, weight loss dysphagia and dyspnea. Interstitial lung disease (see this term) and cardiac involvement have also been reported. The course is often severe but may be self-limiting and recovery may occur within weeks to months of discontinuing the causative agent, if identified. Etiology The disease is thought to be related to an immune response possibly triggered by drug therapy (statins), connective tissue diseases, or cancer. The exact mechanism underling the disorder is not known but some autoantibodies appear to be a likely cause. Malignancy may be involved. Diagnostic methods Diagnosis is based on the clinical picture and on muscle biopsy showing minimal or no inflammatory infiltrates and marked muscle necrosis, unlike other inflammatory myopathies. Electromyography (EMG) shows myopathic findings. Creatine kinase (CK) levels are often more than 10 times above the upper limit of normal at the time of onset of muscle weakness. Magnetic resonance imaging (MRI) may show diffuse or patchy edema within muscles. Anti-SRP and anti-HMGCoAR autoantibodies are frequently associated with this condition. Currently, seronegative NAM represents 20-30% of the cases. Management and treatment Treatment of the underlying cause, if identified, is essential (statin discontinuation, or malignancy). NAM patients generally respond well to multiple-agent, long-term immunosuppressive therapies starting by high dose corticosteroids. Intravenous immunoglobulin (IVIg) appears to be effective. Rituximab has also shown beneficial effects. Response to therapy should be assessed clinically on the basis of muscle strength and biologically on CK levels.

MalaCards based summary : Necrotizing Autoimmune Myopathy, also known as immune myopathy with myocyte necrosis, is related to myopathy, congenital, bailey-bloch and stac3 disorder. The drugs Immunologic Factors and Pharmaceutical Solutions have been mentioned in the context of this disorder. Affiliated tissues include heart, lung and skeletal muscle, and related phenotypes are dysphagia and myopathy

Related Diseases for Necrotizing Autoimmune Myopathy

Diseases related to Necrotizing Autoimmune Myopathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 213)
# Related Disease Score Top Affiliating Genes
1 myopathy, congenital, bailey-bloch 11.5
2 stac3 disorder 11.0
3 myopathy 10.8
4 myositis 10.4
5 mycobacterium tuberculosis 1 10.4
6 avian influenza 10.3
7 cleft lip 10.3
8 cleft lip/palate 10.3
9 connective tissue disease 10.3
10 metabolic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration 10.3
11 encephalopathy, progressive, early-onset, with episodic rhabdomyolysis 10.3
12 dermatomyositis 10.3
13 influenza 10.3
14 polymyositis 10.2
15 systemic scleroderma 10.2
16 overlap myositis 10.2
17 japanese encephalitis 10.2
18 encephalitis 10.2
19 trachoma 10.2
20 malaria 10.1
21 mouth disease 10.1
22 autoimmune disease 10.1
23 hypercholesterolemia, familial, 1 10.1
24 exanthem 10.1
25 respiratory failure 10.1
26 interstitial lung disease 10.1
27 lung disease 10.1
28 dysphagia 10.1
29 disease by infectious agent 10.1
30 typhoid fever 10.1
31 hepatitis b 10.1
32 tonsillitis 10.1
33 iga glomerulonephritis 10.1
34 vasculitis 10.1
35 raynaud phenomenon 10.1
36 spondyloarthropathy 1 10.1
37 sarcoidosis 1 10.1
38 scleroderma, familial progressive 10.1
39 multiple acyl-coa dehydrogenase deficiency 10.1
40 leukemia, acute myeloid 10.1
41 myoglobinuria 10.1
42 antisynthetase syndrome 10.1
43 erythema nodosum 10.1
44 inflammatory spondylopathy 10.1
45 leukemia 10.1
46 congestive heart failure 10.1
47 spondylitis 10.1
48 mitochondrial myopathy 10.1
49 b-cell lymphoma 10.1
50 collagen disease 10.1

Graphical network of the top 20 diseases related to Necrotizing Autoimmune Myopathy:



Diseases related to Necrotizing Autoimmune Myopathy

Symptoms & Phenotypes for Necrotizing Autoimmune Myopathy

Human phenotypes related to Necrotizing Autoimmune Myopathy:

58 (show all 22)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 dysphagia 58 Frequent (79-30%)
2 myopathy 58 Very frequent (99-80%)
3 skeletal muscle atrophy 58 Frequent (79-30%)
4 congestive heart failure 58 Very rare (<4-1%)
5 elevated serum creatine kinase 58 Very frequent (99-80%)
6 emg: myopathic abnormalities 58 Very frequent (99-80%)
7 scapular winging 58 Occasional (29-5%)
8 proximal muscle weakness in lower limbs 58 Frequent (79-30%)
9 arthralgia 58 Very rare (<4-1%)
10 myalgia 58 Frequent (79-30%)
11 neoplasm 58 Occasional (29-5%)
12 myocarditis 58 Occasional (29-5%)
13 chest pain 58 Occasional (29-5%)
14 skin rash 58 Very rare (<4-1%)
15 interstitial pulmonary abnormality 58 Occasional (29-5%)
16 myositis 58 Frequent (79-30%)
17 raynaud phenomenon 58 Very rare (<4-1%)
18 axial muscle weakness 58 Occasional (29-5%)
19 palpitations 58 Occasional (29-5%)
20 autoimmune antibody positivity 58 Very frequent (99-80%)
21 muscle fiber necrosis 58 Very frequent (99-80%)
22 fatiguable weakness of proximal limb muscles 58 Very frequent (99-80%)

Drugs & Therapeutics for Necrotizing Autoimmune Myopathy

Drugs for Necrotizing Autoimmune Myopathy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunologic Factors Phase 2
2 Pharmaceutical Solutions Phase 2
3 Rho(D) Immune Globulin Phase 2
4 gamma-Globulins Phase 2
5 Immunoglobulins, Intravenous Phase 2
6 Autoantibodies Phase 2
7 Immunoglobulins
8 Antibodies

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Monotherapy IVIG Gamunex-C for HMG-CoA Reductase Auto-Antibody Positive Necrotizing Myopathy Treatment (The MIGHT Trial) Recruiting NCT04450654 Phase 2 Gamunex-C;Albumin
2 A Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety, Tolerability, and Efficacy of Zilucoplan in Subjects With Immune-Mediated Necrotizing Myopathy Active, not recruiting NCT04025632 Phase 2 zilucoplan
3 Clinical, Paraclinical, Functional Characteristics and Evolution of Juvenile Autoimmune Necrotizing Myopathies in a National Retrospective Cohort Not yet recruiting NCT04295785

Search NIH Clinical Center for Necrotizing Autoimmune Myopathy

Genetic Tests for Necrotizing Autoimmune Myopathy

Anatomical Context for Necrotizing Autoimmune Myopathy

MalaCards organs/tissues related to Necrotizing Autoimmune Myopathy:

40
Heart, Lung, Skeletal Muscle, Myeloid, Kidney, T Cells, Brain

Publications for Necrotizing Autoimmune Myopathy

Articles related to Necrotizing Autoimmune Myopathy:

(show top 50) (show all 204)
# Title Authors PMID Year
1
Clinical utility of anti-cytosolic 5'-nucleotidase 1A antibody in idiopathic inflammatory myopathies. 61
33556224 2021
2
Marked Respiratory Failure in an Ambulant Patient with Immune-mediated Necrotizing Myopathy and Anti-Kv1.4 and Anti-titin Antibodies. 61
33642484 2021
3
Influenza A (H3N2) infection followed by anti-signal recognition particle antibody-positive necrotizing myopathy: A case report. 61
33217572 2021
4
[A Case of Immune-Mediated Necrotizing Myopathy Associated with Primary Sjögren Syndrome]. 61
33561833 2021
5
[Clinicopathological Features of Myositis and Necrotizing Myopathy: How to Distinguish between Myositis and Muscular Dystrophy on Muscle Pathology]. 61
33561829 2021
6
Immunotherapy reversed myopathy but not cardiomyopathy in a necrotizing autoimmune myopathy patient with positive anti-SRP and MDA-5 autoantibodies. 61
33579193 2021
7
[Immune-Mediated Necrotizing Myopathy: IMNM]. 61
33561827 2021
8
Asymptomatic necrotizing myositis in a young male with progressive interstitial lung disease. 61
33747761 2021
9
Rituximab in the treatment of immune-mediated necrotizing myopathy: a review of case reports and case series. 61
33786066 2021
10
A case of statin-associated immune-mediated necrotizing myopathy with atypical biopsy features. 61
33372889 2021
11
Subcutaneous immunoglobulin therapy in statin-induced necrotizing autoimmune myopathy. 61
32967573 2021
12
Treatment of Statin-Induced Necrotizing Autoimmune Myopathy With Glucocorticoid Monotherapy. 61
33489504 2020
13
Severe axial and pelvifemoral muscle damage in immune-mediated necrotizing myopathy evaluated by whole-body MRI. 61
32222382 2020
14
Immune-mediated necrotizing myopathy: clinical features and pathogenesis. 61
33093664 2020
15
Severe muscle damage with myofiber necrosis and macrophage infiltrates characterize anti-Mi2 positive dermatomyositis. 61
33249503 2020
16
Fulminant immune-mediated necrotising myopathy (IMNM) mimicking myocardial infarction with non-obstructive coronary arteries (MINOCA). 61
33139359 2020
17
Report of a case of necrotizing autoimmune myopathy with thymoma-associated myasthenia gravis. 61
32075478 2020
18
Global versus individual muscle segmentation to assess quantitative MRI-based fat fraction changes in neuromuscular diseases. 61
33219846 2020
19
Where are we moving in the classification of idiopathic inflammatory myopathies? 61
32852298 2020
20
Relationship between change in physical activity and in clinical status in patients with idiopathic inflammatory myopathy: A prospective cohort study. 61
32920327 2020
21
Immune-mediated necrotising myopathy in asymptomatic patients with high creatine kinase. 61
33033004 2020
22
Statin-induced necrotizing autoimmune myopathy. 61
33456195 2020
23
Cardiac and Respiratory Complications of Necrotizing Autoimmune Myopathy. 61
32807522 2020
24
Accumulation of autophagosome cargo protein p62 is common in idiopathic inflammatory myopathies. 61
32896253 2020
25
Machine learning algorithms reveal unique gene expression profiles in muscle biopsies from patients with different types of myositis. 61
32546599 2020
26
Myopathies featuring early or prominent dysphagia. 61
32510670 2020
27
Utilization of myositis antibody-specific panel for diagnosis, treatment, and evaluation of disease progression. 61
33235684 2020
28
[Muscle weakness and CK elevation: is it myositis?] 61
32462339 2020
29
Treatment experience of Taiwanese patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myopathy. 61
32666706 2020
30
[Classification of idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific antibodies]. 61
32895158 2020
31
What is New in Idiopathic Inflammatory Myopathies: Mechanisms and Therapies. 61
33223661 2020
32
[Classification of idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific antibodies]. 61
32701237 2020
33
Immune mediated necrotizing myopathy: A rare complication of statin therapy. 61
32670535 2020
34
Autoantibody-Negative Immune-Mediated Necrotizing Myopathy Responds to Early and Aggressive Treatment: A Case Report. 61
32467802 2020
35
Anti-HMGCR Antibody-Positive Myopathy Shows Bcl-2-Positive Inflammation and Lymphocytic Accumulations. 61
32100014 2020
36
Prevalence of polymyalgia rheumatica in a cohort of patients with idiopathic inflammatory myopathy. 61
31919772 2020
37
A RARE CASE OF STATIN-INDUCED NECROTIZING AUTOIMMUNE MYOPATHY. 61
32524017 2020
38
Clinical and histological features of immune-mediated necrotising myopathy: A multi-centre South Australian cohort study. 61
32229165 2020
39
Muscle aches and pains: do I have leukemia? 61
31612308 2020
40
Sequestosome-1 (p62) expression reveals chaperone-assisted selective autophagy in immune-mediated necrotizing myopathies. 61
31376301 2020
41
A Rapidly Progressive Case of Statin-induced Necrotizing Autoimmune Myopathy. 61
32211257 2020
42
Myopathies presenting with head drop: Clinical spectrum and treatment outcomes. 61
32005492 2020
43
Immune-Mediated Necrotizing Myopathy Initially Presenting as Erythema Nodosum. 61
32922062 2020
44
Aberrant Expression of High Mobility Group Box Protein 1 in the Idiopathic Inflammatory Myopathies. 61
32363191 2020
45
Statin-Associated Autoimmune Myopathy: Current Perspectives. 61
32581543 2020
46
Statins, myalgia, and rhabdomyolysis. 61
30735805 2020
47
Complete Recovery from COVID-19 Bilateral Pneumonia in an Immunosuppressed Man with Immune-Mediated Necrotizing Myopathy. 61
33133718 2020
48
Soluble IL-2 Receptor in Dermatomyositis: Its Associations with Skin Ulcers and Disease Activity. 61
32774147 2020
49
Initial seronegative immune-mediated necrotising myopathy with subsequent anti-HMGCR antibody development and response to rituximab: case report. 61
32613157 2020
50
Immune-mediated necrotising myopathy: A critical review of current concepts. 61
31109639 2019

Variations for Necrotizing Autoimmune Myopathy

Expression for Necrotizing Autoimmune Myopathy

Search GEO for disease gene expression data for Necrotizing Autoimmune Myopathy.

Pathways for Necrotizing Autoimmune Myopathy

GO Terms for Necrotizing Autoimmune Myopathy

Sources for Necrotizing Autoimmune Myopathy

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61 PubMed
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71 UMLS via Orphanet
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