Figueiredo Rafael, Pires Duro Inês, Marinho António, Mota Conceição, Guedes Margarida, Zilhão Carla
Department of Pediatrics, Centro Materno-Infantil do Norte-Centro Hospitalar Universitário do Porto, Porto, Portugal.
Department of Internal Medicine, Hospital de Santo António-Centro Hospitalar Universitário do Porto, Porto, Portugal.
Case Rep Rheumatol. 2021 Jun 19;2021:6642910. doi: 10.1155/2021/6642910. eCollection 2021.
. Granulomatosis with polyangiitis (GPA) is a rare disease in pediatric age. We report two cases with distinct presentations. . A seventeen-year-old male with prolonged febrile syndrome, cough, and constitutional symptoms. CT-scan showed cavitated lesions of the lung and bronchial biopsy a necrotizing inflammatory process. The remaining investigation revealed hematoproteinuria and positive C-ANCA and anti-PR3. Complications: Bilateral acute pulmonary thromboembolism, splenic infarction, and extensive popliteal and superficial femoral deep vein thrombosis. He was treated with corticosteroids, immunoglobulin, rituximab, and anticoagulation. Rituximab was maintained every six months during the first two years. Control angio-CT was performed with almost complete resolution of previous findings. In a twelve-year-old female with inflammatory signs of the limbs, investigation showed myositis of the thigh and tenosynovitis of the wrist, normocytic normochromic anemia (Hg 9.4 g/dL), mild elevation of inflammatory markers, and high creatine kinase. During hospitalization, she presented an extensive alveolar hemorrhage associated with severe anemia and positive C-ANCA and anti-PR3. Clinical deterioration prompted intravenous methylprednisolone pulses and plasmapheresis. Induction therapy with rituximab and prednisolone showed good results. Rituximab was maintained every six months, for 18 months, with gradual tapering of corticoids. . GPA is a systemic disease with variable clinical presentation and severity. Pediatric patients have similar clinical manifestations to adults but different frequencies of organ involvement; constitutional symptoms are also more common. We highlight the different presentation of these two cases, as well as the need for an individualized approach. Rituximab has been used for both induction-remission and maintenance therapy, with good results, particularly in young patients.
肉芽肿性多血管炎（GPA）在儿童期是一种罕见疾病。我们报告两例表现各异的病例。一名17岁男性，有长期发热综合征、咳嗽和全身症状。CT扫描显示肺部有空洞性病变，支气管活检显示为坏死性炎症过程。其余检查显示血红蛋白尿以及C-ANCA和抗PR3阳性。并发症：双侧急性肺血栓栓塞、脾梗死以及广泛的腘静脉和股浅深静脉血栓形成。他接受了糖皮质激素、免疫球蛋白、利妥昔单抗和抗凝治疗。在最初两年中，每六个月维持使用一次利妥昔单抗。进行了对照血管CT检查，先前的检查结果几乎完全消退。一名12岁女性，有肢体炎症体征，检查显示大腿肌炎和腕部腱鞘炎、正细胞正色素性贫血（血红蛋白9.4g/dL）、炎症标志物轻度升高以及肌酸激酶升高。住院期间，她出现了广泛的肺泡出血，伴有严重贫血以及C-ANCA和抗PR3阳性。临床恶化促使进行静脉注射甲泼尼龙冲击治疗和血浆置换。利妥昔单抗和泼尼松龙的诱导治疗显示出良好效果。利妥昔单抗每六个月维持使用一次，持续18个月，同时逐渐减少皮质类固醇的用量。GPA是一种临床表现和严重程度各异的全身性疾病。儿科患者的临床表现与成人相似，但器官受累的频率不同；全身症状也更为常见。我们强调这两例病例的不同表现，以及采取个体化治疗方法的必要性。利妥昔单抗已用于诱导缓解和维持治疗，效果良好，尤其是在年轻患者中。
