[Dermatomyositis and juvenile dermatomyositis].

Dressler, Frank; Maurer, Britta (2023). [Dermatomyositis and juvenile dermatomyositis]. Zeitschrift für Rheumatologie, 82(3), pp. 233-245. Springer-Medizin-Verlag 10.1007/s00393-022-01205-5

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Dermatomyositis (DM) is an inflammatory multisystem disease of unknown etiology, which can already occur in children but first onset can also be in older adulthood. Myalgia and muscle weakness can occur later in the course of the disease or even be completely absent in some forms. Classical signs on the skin include heliotrope rash, facial erythema, Gottron's papules and nailfold capillary abnormalities. For the diagnosis, screening for the presence of myositis-specific autoantibodies has become increasingly more relevant. Muscle enzymes may be elevated but not in approximately one third of patients. In the absence of typical clinical or serologic findings, additional examination methods such as nailfold capillaroscopy, magnetic resonance imaging, electromyography, skin or muscle biopsies may help to establish the diagnosis. Depending on the clinical and serological subtype, additional screening for gastrointestinal or cardiopulmonary involvement should be considered. In adults, an age-appropriate tumor screening should also be performed. Apart from corticosteroids as induction therapy, biologics and small molecule inhibitors are gaining in importance in addition to conventional disease-modifying anti-rheumatic drugs and intravenous immunoglobulins. The prognosis for DM and juvenile DM (JDM) has improved. Most patients recover at least to some extent; however, a few patients die and a minority develop persisting muscle atrophy or severe calcinosis.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology, Clinical Immunology and Allergology

UniBE Contributor:

Maurer, Britta

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0340-1855

Publisher:

Springer-Medizin-Verlag

Language:

German

Submitter:

Pubmed Import

Date Deposited:

02 May 2022 08:22

Last Modified:

06 Apr 2023 00:11

Publisher DOI:

10.1007/s00393-022-01205-5

PubMed ID:

35486206

Uncontrolled Keywords:

Biologics Calcinosis Myositis-specific autoantibodies Neoplasms Small molecule inhibitors

BORIS DOI:

10.48350/169639

URI:

https://boris.unibe.ch/id/eprint/169639

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