Boulay V, Lauque D, Reynaud F, Carles P, Pourrat J
Service de Médecine interne, CHU Purpan, Toulouse.
Presse Med. 2000 Sep 23;29(27):1507-9.
Low-complement urticarial vasculitis is an uncommon condition associating urticaria, glomerulonephritis, obstructive ventilatory disorders, and anti-Ciq antibodies.
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.
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.
低补体性荨麻疹性血管炎是一种罕见疾病，伴有荨麻疹、肾小球肾炎、阻塞性通气障碍及抗C1q抗体。
我们报告一例34岁女性病例，该患者出现伴有紫癜的荨麻疹、膜增生性肾小球肾炎（肌酐238μmol/L）及伴有支气管扩张的支气管阻塞。总补体及C3成分降低。血清中发现抗C1q抗体，抗DNA抗体阴性。尽管进行了皮质类固醇治疗，呼吸和肾衰竭仍进行性加重，4个月后死亡。
低补体性荨麻疹性血管炎中的支气管阻塞由肺气肿引起，常危及生命。我们的病例表现出不寻常的特征，即缺乏可通过放射检查发现的肺气肿、存在支气管扩张且呼吸功能迅速恶化。
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