Guidelines on dermatomyositis--excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology.

Sunderkötter, Cord; Nast, Alexander; Worm, Margitta; Dengler, Reinhard; Dörner, Thomas; Ganter, Horst; Hohlfeld, Reinhard; Melms, Arthur; Melzer, Nico; Rösler, Kai Michael; Schmidt, Jens; Sinnreich, Michael; Walter, Maggi C; Wanschitz, Julia; Wiendl, Heinz (2016). Guidelines on dermatomyositis--excerpt from the interdisciplinary S2k guidelines on myositis syndromes by the German Society of Neurology. Journal der Deutschen Dermatologischen Gesellschaft, 14(3), pp. 321-338. Blackwell 10.1111/ddg.12909

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The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at www.awmf.org). The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30% of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup. Although the cutaneous manifestations in DM are variable, taken together, they represent a characteristic and crucial diagnostic criterion for DM. Approximately 5-20% of individuals exhibit typical skin lesions without any clinically manifest muscle involvement (amyopathic DM). About 30% of adult DM cases are associated with a malignancy. This fact, however, should not delay the treatment of severe myositis. Corticosteroids are the therapy of choice in myositis (1-2 mg/kg). Additional immunosuppressive therapy is frequently required (azathioprine, for children methotrexate). In case of insufficient therapeutic response, the use of intravenous immunoglobulins is justified. The benefit of rituximab has not been conclusively ascertained yet. Acute therapeutic management is usually followed by low-dose maintenance therapy for one to three years. Skin lesions do not always respond sufficiently to myositis therapy. Effective treatment for such cases consists of topical corticosteroids and sometimes also calcineurin inhibitors. Systemic therapies shown to be effective include antimalarial agents (also in combination), methotrexate, and corticosteroids. Intravenous immunoglobulins or rituximab may also be helpful. UV protection is an important prophylactic measure.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DCR Unit Sahli Building > Forschungsgruppe Neurologie
04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology

UniBE Contributor:

Rösler, Kai Michael

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1610-0379

Publisher:

Blackwell

Language:

English

Submitter:

Stefanie Hetzenecker

Date Deposited:

13 Oct 2017 09:17

Last Modified:

13 Oct 2017 09:23

Publisher DOI:

10.1111/ddg.12909

PubMed ID:

26972210

BORIS DOI:

10.7892/boris.104846

URI:

https://boris.unibe.ch/id/eprint/104846

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