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

Abstract

BackgroundIdiopathic 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 presentationWe 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.ConclusionsCoronary 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.

Pulmonary Presentation of Atypical Kawasaki Disease

“…Our index case never followed the sequence and only had coronary artery abnormalities (CAA) without compatible clinical and laboratory feature which was perplexing to us. Occasional cases of mild CAA (2.5-5 Z score) have been reported in non-KD febrile illness but moderate CAA have never been [2][3][4]. The presence of moderate CAA rules out non-KD febrile illness in our case.…”

Cardiovascular Abnormalities in Juvenile Dermatomyositis: A Scoping Review for the Clinical Rheumatologists

“…A study has shown that Raynaud's phenomenon increased the chance of coronary vasospasm (46). One child with JDM was reported to have coronary artery dilatation which was an unusual finding (47).…”

Antisynthetase syndrome: A distinct disease spectrum

“…Other than the classic features mentioned above, calcinosis, panniculitis, coronary artery dilatation, eosinophilic pleural effusions, and myopericarditis have also been reported in small case series. [60][61][62][63][64][65][66][67][68][69]…”