Department of Paediatric Rheumatology, Medical University of Lodz, Sporna 36/50, 91-738, Lodz, Poland.
Rheumatol Int. 2018 Apr;38(4):683-687. doi: 10.1007/s00296-017-3923-y. Epub 2018 Jan 2.
We herein describe a case of a 6-year-old girl diagnosed with microscopic polyangiitis (MPA) after a long diagnostic process demanding the cooperation of paediatric respiratory medicine specialists, rheumatologists, nephrologists and radiologist. First symptoms of MPA were observed after Mycoplasma pneumoniae infection and included persistent crepitations at the basis of both lungs and mild haematuria. CT imaging showed features of lung fibrosis, renal biopsy was indicative of mesangial proliferative glomerulonephritis, in the skin biopsy features of microscopic polyangiitis were described. In the laboratory tests pANCA antibodies specific for myeloperoxidase (MPO) were present in high titer. MPA with initial features of lung fibrosis is an unusual presentation of this rare disease, usually observed in adult population. This unique case illustrates the diagnostic and therapeutic challenges in paediatric vasculitis patients with an unusual initial presentation in the form of interstitial lung disease. In the manuscript we also discuss the possible aetiology of lung fibrosis in microscopic polyangiitis MPA and provide the review of the current literature on the topic of childhood-onset MPA.
我们在此描述了一例 6 岁女孩的病例，该女孩在经过儿科呼吸医学专家、风湿病学家、肾病学家和放射科医生的合作后，经过长时间的诊断过程被诊断为显微镜下多血管炎（MPA）。MPA 的最初症状是在肺炎支原体感染后观察到的，包括双肺底部持续的爆裂音和轻度血尿。CT 成像显示出肺纤维化的特征，肾活检显示为局灶节段性肾小球肾炎，皮肤活检显示出显微镜下多血管炎的特征。在实验室检查中，针对髓过氧化物酶（MPO）的 pANCA 抗体呈高滴度阳性。以肺纤维化初始特征为表现的 MPA 是一种罕见疾病的不常见表现，通常在成年人群中观察到。这个独特的病例说明了儿科血管炎患者在以间质性肺病为特征的不常见初始表现形式下的诊断和治疗挑战。在本文中，我们还讨论了 MPA 显微镜下多血管炎中肺纤维化的可能病因，并提供了关于儿童发病 MPA 这一主题的当前文献综述。
