[Hypocomplementemic urticarial vasculitis].
BACKGROUND
Low-complement urticarial vasculitis is an uncommon condition associating urticaria, glomerulonephritis, obstructive ventilatory disorders, and anti-Ciq antibodies.


METHODS
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.


CONCLUSIONS
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.
Boulay
V
V
Service de Médecine interne, CHU Purpan, Toulouse. boulay.v@chu-toulouse.fr
Lauque
D
D
Reynaud
F
F
Carles
P
P
Pourrat
J
J
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001706:Biopsy; D001987:Bronchiectasis / Q000276:immunology / Q000473:pathology; D015922:Complement C1q / Q000276:immunology; D003165:Complement System Proteins / Q000172:deficiency*; D017809:Fatal Outcome; D005260:Female; D005920:Glomerular Mesangium / Q000276:immunology / Q000473:pathology; D005921:Glomerulonephritis / Q000276:immunology / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000276:immunology / Q000473:pathology; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014581:Urticaria / Q000276:immunology* / Q000473:pathology; D014657:Vasculitis / Q000276:immunology* / Q000473:pathology
D001323:Autoantibodies; D015922:Complement C1q; D003165:Complement System Proteins