[Severe pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV)].
Pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV) or Mac Duffie syndrome is extremely rare with a poor prognosis. We report the case of a 55-year-old female patient treated for HUV over a period of 20 years. The diagnosis was confirmed on the basis of urticarial lesions, ocular inflammation, positive C1q-p test by immunodiffusion, with low rate of C1q. The patient was treated with cycles of cyclophosphamide, corticoids and rituximab as she developed class III dyspnea (NYHA classification ). The clinico-radiological and functional assessment showed thoracic distension and severe obstructive pulmonary disease which found no significant improvement with systemic treatment Aerosol therapy was started and the patient had a marked clinical improvement. Pulmonary involvement in Mac Duffie hypocomplementemic urticarial vasculitis worsens the patient short-term vital prognosis. The knowledge of the different types of pulmonary involvement opens new therapeutic prospects.
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Mohammed
M
Raoufi
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Mustapha
M
Laine
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Hicham Naji
HN
Amrani
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Hicham
H
Souhi
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Hicham
H
Janah
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Hanane
H
Elouazzani
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Ismail Abderrahmane
IA
Rhorfi
Service de Pneumologie, Hôpital Militaire Mohamed V, Rabat, Maroc.
Ahmed
A
Abid
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use; D015922:Complement C1q / Q000172:deficiency; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008171:Lung Diseases / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008875:Middle Aged; D000069283:Rituximab / Q000008:administration & dosage / Q000627:therapeutic use; D013577:Syndrome; D014581:Urticaria / Q000188:drug therapy / Q000473:pathology*; D014657:Vasculitis / Q000188:drug therapy / Q000503:physiopathology*
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D000069283:Rituximab; D015922:Complement C1q; D003520:Cyclophosphamide
Mac Duffie; dyspnea; hypocomplementemia