Abstract
Granulomatosis with polyangiitis (GPA), necrotizing vasculitis of small and medium-sized vessels, is traditionally believed to mainly affect respiratory tract with additional focal kidney involvements as its primary manifestations with a relatively rare annual incidence rate of 20-50 cases per million. Six percent of the affected cases have cardiac involvements; among which, aneurysms comprise the lowest penetrance. By this paper, we aim to cast light on clinical diagnostic and treatment methods of a rare case presentation, a 10-year-old male GPA patient, diagnosed with massive thrombosis at his coronary artery aneurysm. GPA should be considered as differential diagnosis of prolong fever and coronary aneurysms in adolescents.
Highlights
Granulomatosis with polyangiitis (GPA) or Wegener’s granulomatosis as a necrotizing vasculitis of small and mediumsized vessels is traditionally believed to mainly affect respiratory tract with additional focal kidney involvements as its primary manifestations with a relatively rare annual incidence rate of 20-50 cases per million
Definitive granulomatosis with polyangiitis (GPA) diagnosis lies on fulfilling three out of six following criteria: compatible histopathology with granulomatosis inflammation within the wall of an artery or in the perivascular region, oral or nasal chronic involvement, laryngo-tracheobronchial stenosis, radiographic findings suggestive for pulmonary lesions, positive enzyme-linked immunosorbent assay (ELISA) test for antineutrophil cytoplasmic antibodies (ANCAs), and renal involvement [4]
GPA is a rare rheumatologic disorder in Iranian children with prevalence less than 1 per million children [8]. But it is usually associated with antineutrophil cytoplasmic antibody (ANCA) that was detected in predominantly cytoplasmic form
Summary
Granulomatosis with polyangiitis (GPA) or Wegener’s granulomatosis as a necrotizing vasculitis of small and mediumsized vessels is traditionally believed to mainly affect respiratory tract with additional focal kidney involvements as its primary manifestations with a relatively rare annual incidence rate of 20-50 cases per million. Definitive granulomatosis with polyangiitis (GPA) diagnosis lies on fulfilling three out of six following criteria: compatible histopathology with granulomatosis inflammation within the wall of an artery or in the perivascular region, oral or nasal chronic involvement, laryngo-tracheobronchial stenosis, radiographic findings suggestive for pulmonary lesions, positive enzyme-linked immunosorbent assay (ELISA) test for antineutrophil cytoplasmic antibodies (ANCAs), and renal involvement [4]. Cardiac complications of GPA include pericarditis, coronary arteritis, aortic regurgitation, aortic valvular lesions simulating endocarditis, heart block, and aneurysms [6]. Year-old patient, diagnosed with massive thrombosis at his coronary artery aneurysm
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