Polymyositis and dermatomyositis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]
Overview
The exact pathogenesis of polymyositis and dermatomyositis is not fully understood.
Pathophysiology
Pathogenesis
The exact pathogenesis of polymyositis and dermatomyositis is not fully understood. However, it is understood that polymyositis and dermatomyositis are the result of autoimmune attack but triggering factors of are not well-known.
Polymyositis
- Polymyositis is caused by inflammation and degeneration of the muscles.
- In polymyositis, CD8-positive cytotoxic T cells invade muscle fibres that express MHC class I antigens which may leads to fibre necrosis via the perforin pathway.
Dermatomyositis
- Dermatomyositis is caused by skin inflammation.[1][2][3][4]
- In dermatomyositis, activation and deposition of complements may lead to lysis of endomysial capillaries and muscle ischemia.[5]
Antisynthetase syndrome
Amyopathic dermatomyositis
Genetics
- [Disease name] is transmitted in [mode of genetic transmission] pattern.
- Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
- The development of [disease name] is the result of multiple genetic mutations.
- Genes might be associated with development of polymyositis and dermatomyositis, especially in familial cases, which include:[6]
- HLA DRB1*0301 alleles for polymyositis and inclusion-body myositis
- HLA DQA1*0501 for juvenile dermatomyositis,
- Tumour necrosis factor 308A polymorphism for photosensitivity in dermatomyositis
Associated Conditions
Different conditions associated with polymyositis and dermatomyositis include:
- Interstitial lung disease
- Conduction abnormalities and arrhythmia
- Myocardial infarction
- Malignancy in patients with dermatomyositis (5-7 fold more risks than the general population) include:[7]
- Lung
- Ovarian
- Breast
- Colorectal
- Cervical
- Bladder
- Nasopharyngeal
- Esophageal
- Pancreatic
- Kidney
- Malignancy in patients with polymyositis include:
- Lung
- Kidney
- Breast
- Bladder
- Endometrial
- Cervical
- Thyroid
- Lymphoma
- Myeloma
- Brain
- Other connective tissue diseases like
Gross Pathology
- On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
- On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
- ↑ Bodoki L, Nagy-Vincze M, Griger Z, Betteridge Z, Szöllősi L, Dankó K (December 2014). "Four dermatomyositis-specific autoantibodies-anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5-in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort". Autoimmun Rev. 13 (12): 1211–9. doi:10.1016/j.autrev.2014.08.011. PMID 25182203.
- ↑ Tiniakou E, Mammen AL (February 2017). "Idiopathic Inflammatory Myopathies and Malignancy: a Comprehensive Review". Clin Rev Allergy Immunol. 52 (1): 20–33. doi:10.1007/s12016-015-8511-x. PMID 26429706.
- ↑ Bohan, Anthony; Peter, James B. (1975). "Polymyositis and Dermatomyositis". New England Journal of Medicine. 292 (8): 403–407. doi:10.1056/NEJM197502202920807. ISSN 0028-4793.
- ↑ Adler BL, Christopher-Stine L (February 2018). "Triggers of inflammatory myopathy: insights into pathogenesis". Discov Med. 25 (136): 75–83. PMID 29579414.
- ↑ Dalakas, Marinos C; Hohlfeld, Reinhard (2003). "Polymyositis and dermatomyositis". The Lancet. 362 (9388): 971–982. doi:10.1016/S0140-6736(03)14368-1. ISSN 0140-6736.
- ↑ Dalakas, Marinos C; Hohlfeld, Reinhard (2003). "Polymyositis and dermatomyositis". The Lancet. 362 (9388): 971–982. doi:10.1016/S0140-6736(03)14368-1. ISSN 0140-6736.
- ↑ Tiniakou, Eleni; Mammen, Andrew L. (2015). "Idiopathic Inflammatory Myopathies and Malignancy: a Comprehensive Review". Clinical Reviews in Allergy & Immunology. 52 (1): 20–33. doi:10.1007/s12016-015-8511-x. ISSN 1080-0549.