Novel pediatric granulomatosis with polyangiitis with a marked bloody pericardial effusion and bloody stool: a case report

• DOI: 10.1186/s13223-021-00627-1
• Publication Date: 2021-12-04T18:02:27Z
• Authors: Memi Kato; Keisuke Jimbo; Masumi Nagata; Yoshiko Endo; Kosuke Kashiwagi; Kimiko Maruyama; Natsuki Ito; Kaori Tokushima; Nobuyasu Arai; Reiko Kyodo; Masamichi Sato; Eri Miyata; Kenji Hosoi; Eisuke Inage; Tamaki Ikuse; Hideo Fukunaga; Takahiro Kudo; Toshiaki Shimizu
• Keywords: Hematochezia; Pericardiocentesis; Chills; Bloody; Granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA) is a syndrome of refractory vasculitis involving the upper respiratory tract, lungs, kidneys, and systemic small medium-sized arteries that affects all age groups. No pediatric case bloody pericardial effusion resulting in cardiac tamponade co-existing hematochezia has been reported.A 14-year-old boy was referred for evaluation prolonged fever, chest pain, intermittent hematochezia. Diagnostic imaging showed prominent effusion. Immediately after admission, his systolic blood pressure decreased. Emergent pericardiocentesis resulted aspiration massive amount fluid. This diagnosed as because recovered immediately drainage. The patient had an elevated serine proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA) level on serological examination. Head MRI thickening nasal sinusoidal mucosa cystic mass left sphenoid sinus. After ruling out malignancy based cytology effusion, MRI, gallium scintigraphy, total colonoscopy multiple irregular-shaped aphthae from right transverse colon to cecum contralateral side mesenteric attachments. Biopsy specimens aphthous lesions confirmed necrotizing granulomatous inflammation. A diagnosis GPA made these findings, oral prednisolone (PSL) azathioprine were started. disappeared rapidly, no recurrence seen PSL tapering completed. PR3-ANCA decreased into normal range initial therapy.Pericarditis common complication GPA, but there have reports resultant tamponade. first gastrointestinal complications preceding symptoms such or renal symptoms. also extremely rare. In conclusion, serial measurement ANCA levels important patients persistent fever stool, inflammatory bowel disease, make vasculitic syndrome.