[Hypocomplementemic urticarial vasculitis].

La Presse medicale Pub Date : 2020-02-10 DOI:10.32388/epf8z8
V. Boulay, D. Lauque, F. Reynaud, P. Carles, J. Pourrat
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引用次数: 9

Abstract

BACKGROUND Low-complement urticarial vasculitis is an uncommon condition associating urticaria, glomerulonephritis, obstructive ventilatory disorders, and anti-Ciq antibodies. CASE REPORT We report a case in a 34-year-old woman who developed urticaria with purpura, membranoproliferative glomerulonephritis (creatinine 238 mumol/l) and bronchial obstruction with bronchectasia. Total complement and the C3 fraction were low. Anti-C1q antibodies were found in the serum and anti-DNA antibodies were negative. Aggravation of the respiratory and renal failure progressed despite corticosteroid therapy, leading to death at 4 months. DISCUSSION Bronchial obstruction in low-complement urticarial vasculitis results from emphysema and is often life-threatening. Our case exhibited an unusual feature due to the lack of radiodetectable emphysema, the presence of bronchectasia and the rapid degradation of the respiratory function.
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[补乏性荨麻疹血管炎]。
背景:低补体荨麻疹血管炎是一种罕见的疾病,与荨麻疹、肾小球肾炎、阻塞性通气障碍和抗ciq抗体有关。病例报告:我们报告一例34岁女性,并发荨麻疹合并紫癜、膜增生性肾小球肾炎(肌酐238 μ mol/l)和支气管梗阻合并支气管增生。总补体和C3分数低。血清中检测到抗c1q抗体,抗dna抗体阴性。尽管皮质类固醇治疗,呼吸和肾功能衰竭仍加重,导致4个月时死亡。低补体荨麻疹血管炎的支气管阻塞是由肺气肿引起的,通常危及生命。我们的病例表现出一个不寻常的特征,由于缺乏可放射检测到的肺气肿,支气管增生和呼吸功能的快速退化。
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[Pyoderma gangrenosum]. [Hypocomplementemic urticarial vasculitis]. [Trisomy 13]. [Osteogenesis imperfecta]. [Hypersomnia].
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