The Clinical Spectrum and Therapeutic Management of Hypocomplementemic Urticarial Vasculitis: Data From a French Nationwide Study of Fifty‐Seven Patients - Université de Montpellier Accéder directement au contenu
Article Dans Une Revue Arthritis & rheumatology Année : 2015

The Clinical Spectrum and Therapeutic Management of Hypocomplementemic Urticarial Vasculitis: Data From a French Nationwide Study of Fifty‐Seven Patients

1 Hôpital Cochin [AP-HP]
2 AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP)
3 UPD5 - Université Paris Descartes - Paris 5
4 Hôpital Saint-Louis
5 CHUGA - Centre Hospitalier Universitaire [CHU Grenoble]
6 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
7 Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB)
8 CHR Metz-Thionville - Centre hospitalier régional Metz-Thionville
9 CHRU Brest - Centre Hospitalier Régional Universitaire de Brest
10 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
11 LA CONCEPTION - Hôpital de la Conception [CHU - APHM]
12 CHU Trousseau [Tours]
13 CHU Bordeaux
14 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
15 Université Lille Nord de France (COMUE)
16 CHNO - Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts
17 Service de Dermatologie (CHU de Dijon)
18 CHU Marseille
19 Hôpital Edouard Herriot [CHU - HCL]
20 CHU Angers - Centre Hospitalier Universitaire d'Angers
21 CHRU Tours - Centre Hospitalier Régional Universitaire de Tours
22 AP-HP Hôpital universitaire Robert-Debré [Paris]
23 CHU Limoges
24 Hôpital Jean Verdier [AP-HP]
25 CHU Caen
26 AP-HP - Hopital Saint-Louis [AP-HP]
27 Centre hospitalier de Valence
28 Physiopathologie vasculaire : interactions cellulaires, signalisation et vieillissement
29 HEGP - Hôpital Européen Georges Pompidou [APHP]
30 Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Pascal Godmer
  • Fonction : Auteur
Marie-Sylvie Doutre
  • Fonction : Auteur
Noemie Jourde-Chiche
  • Fonction : Auteur
  • PersonId : 1064169
Clotilde Martel
  • Fonction : Auteur
Cécile Morice
  • Fonction : Auteur
Bernard Simorre
  • Fonction : Auteur
Martine Bagot

Résumé

OBJECTIVE: Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV. METHODS: We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy. We evaluated treatment efficacy by measuring the time to treatment failure. RESULTS: Urticarial lesions were typically more pruritic than painful and were associated with angioedema in 51% of patients, purpura in 35%, and livedo reticularis in 14%. Extracutaneous manifestations included constitutional symptoms (in 56% of patients) as well as musculoskeletal involvement (in 82%), ocular involvement (in 56%), pulmonary involvement (in 19%), gastrointestinal involvement (in 18%), and kidney involvement (in 14%). Patients with HUV typically presented with low C1q levels and normal C1 inhibitor levels, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine or colchicine seemed to be as effective as corticosteroids as first-line therapy. In patients with relapsing and/or refractory disease, rates of cutaneous and immunologic response to therapy seemed to be higher with conventional immunosuppressive agents, in particular, azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. Finally, a cutaneous response to therapy was strongly associated with an immunologic response to therapy. CONCLUSION: HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly, musculoskeletal and ocular involvement associated with anti-C1q antibodies, which were found in approximately half of the patients. The best strategy for treating HUV has yet to be defined.

Dates et versions

hal-02189276 , version 1 (19-07-2019)

Identifiants

Citer

Marie Jachiet, Béatrice Flageul, Alban Deroux, Alain Le Quellec, François Maurier, et al.. The Clinical Spectrum and Therapeutic Management of Hypocomplementemic Urticarial Vasculitis: Data From a French Nationwide Study of Fifty‐Seven Patients. Arthritis & rheumatology, 2015, 67 (2), pp.527-534. ⟨10.1002/art.38956⟩. ⟨hal-02189276⟩
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