amyopathic dermatomyositis; dermatomyositis-sine-myositis
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Etiology
- cormidities common
- lung disease 19%, 39% of these fatal
- malignancies 14%
- may be triggered by sunlight[3]
Epidemiology
- 20-30% of patients with dermatomyositis
Pathology
- histopathologic myositis may or may not be present
Clinical manifestations
- skin lesions characteristic of dermatomyositis
- delayed or absent muscle involvement
Laboratory
- anti-nuclear antibody present in 63%
- anti Jo-1 antibody uncommon
- serum muscle enzymes may be elevated
- serum creatine kinase
- CKMB index may be elevated by regenerating skeletal muscle
- serum lactate dehydrogenase
- serum aldolase
- serum aspartate transaminase (serum AST)
- serum creatine kinase
- CADM-140 autoantigen (IFIH1)
- patients with anti-CADM-140 antibodies frequently develop life-threatening acute progressive interstitial lung disease[2]
Diagnostic procedures
- electromyography normal or may be abnormal[3]
- pulmonary function testing with DLCO
- evaluate for interstitial lung disease[4]
- evaluate for malignancy
Complications
Management
Notes
see dermatomyositis
More general terms
References
- ↑ Gerami P, Schope JM, McDonald L, Walling HW, Sontheimer RD. A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis sine myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol. 2006 Apr;54(4):597-613. Epub 2006 Jan 23. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16546580
- ↑ 2.0 2.1 UniProt http://www.uniprot.org/uniprot/Q9BYX4.html
- ↑ 3.0 3.1 3.2 Medical Knowledge Self Assessment Program (MKSAP) 16,17 American College of Physicians, Philadelphia 2012,2015
- ↑ 4.0 4.1 Morganroth PA, Kreider ME, Okawa J, Taylor L, Werth VP. Interstitial lung disease in classic and skin-predominant dermatomyositis: a retrospective study with screening recommendations. Arch Dermatol. 2010 Jul;146(7):729-38. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20644033