Juvenile idiopathic inflammatory myopathies: A clinicopathological study with emphasis on muscle histology.
Adolescent
Adrenal Cortex Hormones
/ therapeutic use
Autoimmune Diseases
/ physiopathology
Biopsy
Child
Child, Preschool
Dermatomyositis
/ physiopathology
Female
Humans
Immunohistochemistry
Male
Methotrexate
/ therapeutic use
Muscles
/ pathology
Myositis
/ classification
Platelet Endothelial Cell Adhesion Molecule-1
/ metabolism
Retrospective Studies
Scleroderma, Systemic
/ physiopathology
Vasculitis
/ physiopathology
European Neuromuscular Center criteria
juvenile dermatomyositis
juvenile idiopathic inflammatory myopathy
juvenile overlap myositis
muscle biopsy
Journal
Indian journal of pathology & microbiology
ISSN: 0974-5130
Titre abrégé: Indian J Pathol Microbiol
Pays: India
ID NLM: 7605904
Informations de publication
Date de publication:
Historique:
entrez:
2
2
2019
pubmed:
2
2
2019
medline:
22
5
2019
Statut:
ppublish
Résumé
Juvenile idiopathic inflammatory myopathies (JIIM) are rare and heterogeneous. Subtype identification is important for treatment. Patients below 18 years diagnosed as idiopathic inflammatory myopathy (IIM) according to the Bohan and Peter criteria between January 2010 and May 2015 were evaluated with muscle biopsy in the four domains: muscle fiber, inflammation, connective tissue, and vascular, with basic panel of histochemical stains as per recommendations of the European Neuromuscular center (ENMC) workshop 2015. Immunohistochemistry with CD 31 was done to assess capillary density. JIIM constituted 15.25% of IIM with juvenile dermatomyositis (JDM) being the most common subgroup (24/27) followed by juvenile overlap myositis (JOM) (3/27) in association with systemic lupus erythematosus (2) and systemic sclerosis (1). Muscle biopsy in JDM was characterized by perifascicular atrophy, necrosis, degeneration, and regeneration in all and the other features included perivascular inflammation (21), lymphoid aggregates (2), mitochondrial abnormalities (9), sarcoplasmic vacuoles (6), capillary dropout (5), capillary dilatation (6), and perimysial fibrosis (14). JOM was characterized by auto-antibodies and perivascular inflammation. JIIMs were rare and JDM was the most common subtype. Muscle biopsy evaluation as per ENMC criteria characterized the subgroups.
Sections du résumé
BACKGROUND
BACKGROUND
Juvenile idiopathic inflammatory myopathies (JIIM) are rare and heterogeneous. Subtype identification is important for treatment.
MATERIALS AND METHODS
METHODS
Patients below 18 years diagnosed as idiopathic inflammatory myopathy (IIM) according to the Bohan and Peter criteria between January 2010 and May 2015 were evaluated with muscle biopsy in the four domains: muscle fiber, inflammation, connective tissue, and vascular, with basic panel of histochemical stains as per recommendations of the European Neuromuscular center (ENMC) workshop 2015. Immunohistochemistry with CD 31 was done to assess capillary density.
RESULTS
RESULTS
JIIM constituted 15.25% of IIM with juvenile dermatomyositis (JDM) being the most common subgroup (24/27) followed by juvenile overlap myositis (JOM) (3/27) in association with systemic lupus erythematosus (2) and systemic sclerosis (1). Muscle biopsy in JDM was characterized by perifascicular atrophy, necrosis, degeneration, and regeneration in all and the other features included perivascular inflammation (21), lymphoid aggregates (2), mitochondrial abnormalities (9), sarcoplasmic vacuoles (6), capillary dropout (5), capillary dilatation (6), and perimysial fibrosis (14). JOM was characterized by auto-antibodies and perivascular inflammation.
CONCLUSION
CONCLUSIONS
JIIMs were rare and JDM was the most common subtype. Muscle biopsy evaluation as per ENMC criteria characterized the subgroups.
Identifiants
pubmed: 30706861
pii: IndianJPatholMicrobiol_2019_62_1_61_251251
doi: 10.4103/IJPM.IJPM_387_17
doi:
Substances chimiques
Adrenal Cortex Hormones
0
Platelet Endothelial Cell Adhesion Molecule-1
0
Methotrexate
YL5FZ2Y5U1
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
61-66Déclaration de conflit d'intérêts
None