Coronary artery dilation associated with anti-synthetase syndrome in an adolescent

Karim Asi, Anand Gourishankar, Ankur A Kamdar

Research output: Contribution to journalArticle

Abstract

Background: Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimmune disorders primarily affecting skeletal muscle. Pediatric coronary artery dilation is frequently discussed in Kawasaki disease. However, it has yet to be reported in the IIMs or antisynthetase syndrome. We report a unique case of a patient with IIM, antisynthetase syndrome and coronary artery dilation. Case presentation: We report an adolescent presenting with joint symptoms, fever, and eye swelling with a clinical diagnosis of Juvenile Dermatomyositis. He subsequently developed diastolic hypotension with evidence of coronary artery dilation. He received steroids and immunoglobulin and followed by immunosuppressants with mild improvement in his symptoms. The adolescent later developed dyspnea and cough with CT lungs evident for cystic changes; lung biopsy showed interstitial fibrosis and inflammation, and muscle biopsy was abnormal as well. The anti-pl-12 antibody was positive. Following several weeks of treatment, an echocardiogram showed improvement in coronary artery dilation. His joint symptoms, muscle strength and respiratory symptoms have also improved. Conclusions: Coronary artery dilation is not well understood in IIMs or antisynthetase syndrome. Pathobiology of coronary artery involvement, its treatment and prognosis, and association with IIM and antisynthetase syndrome needs further exploration.

LanguageEnglish
Article number3
JournalPediatric Rheumatology
Volume17
Issue number1
DOIs
StatePublished - Jan 10 2019

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Ligases
Dilatation
Coronary Vessels
Myositis
Joints
Biopsy
Lung
Mucocutaneous Lymph Node Syndrome
Muscle Strength
Immunosuppressive Agents
Cough
Dyspnea
Hypotension
Immunoglobulins
Skeletal Muscle
Fibrosis
Fever
Steroids
Pediatrics
Inflammation

Keywords

  • Antisynthetase syndrome
  • Coronary
  • Idiopathic inflammatory myopathy
  • Juvenile dermatomyositis
  • PL-12

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Rheumatology
  • Immunology and Allergy

Cite this

Coronary artery dilation associated with anti-synthetase syndrome in an adolescent. / Asi, Karim; Gourishankar, Anand; Kamdar, Ankur A.

In: Pediatric Rheumatology, Vol. 17, No. 1, 3, 10.01.2019.

Research output: Contribution to journalArticle

Asi, Karim ; Gourishankar, Anand ; Kamdar, Ankur A. / Coronary artery dilation associated with anti-synthetase syndrome in an adolescent. In: Pediatric Rheumatology. 2019 ; Vol. 17, No. 1.
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