Back to Search
Start Over
Juvenile Dermatomyositis: New Clues to Diagnosis and Therapy
- Source :
- Current treatment options in rheumatology
- Publication Year :
- 2021
-
Abstract
- Purpose of reviewTo identify clues to disease activity and discuss therapy options.Recent findingsThe diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine phosphokinase, LDH, and SGOT—which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash—involving the eyelids, hands, knees, face, and upper chest—is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavailability of other orally administered medications should also be considered. At diagnosis, at least 3 days of intravenous methyl prednisolone may help control the HLA-restricted and type 1/2 interferon–driven inflammatory process. The requirement for avoidance of ultraviolet light exposure mandates vitamin D supplementation.SummaryThis often chronic illness targets the cardiovascular system; mortality has decreased from 30 to 1–2% with corticosteroids. New serological biomarkers indicate occult inflammation: ↑CXCL-10 predicts a longer disease course. Some biologic therapies appear promising.
- Subjects :
- 0301 basic medicine
medicine.medical_specialty
Myositis-specific antibodies
Inflammation
Disease
Gastroenterology
Article
03 medical and health sciences
0302 clinical medicine
Prednisone
Calcinosis
Internal medicine
medicine
Juvenile dermatomyositis
030203 arthritis & rheumatology
biology
business.industry
Myositis-associated antibodies
General Medicine
medicine.disease
HLA specificity
Occult
Rheumatology
030104 developmental biology
biology.protein
Nailfold capillaroscopy
Creatine kinase
Therapy
medicine.symptom
business
Infection
Biomarkers
medicine.drug
Subjects
Details
- Language :
- English
- ISSN :
- 21986002
- Volume :
- 7
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Current treatment options in rheumatology
- Accession number :
- edsair.doi.dedup.....30c4806b8dacf4ed7e98bc8d7e9e1fab