Background/Purpose
Hypocomplementemic urticarial vasculitis (HUV), an uncommon vasculitis of unknown etiology, is rarely reported. It is also called anti-C1q vasculitis in the 2012 revised International Chapel Hill Consensus Conference Nomenclature for Vasculitis. Information on its presentation and therapeutic management is scarce.
Methods
To analyze the clinical spectrum and therapeutic management of HUV patients, we conducted a French nationwide retrospective and transdisciplinary study on behalf of the FVSG that included 57 patients with chronic urticaria, histological leukocytoclastic vasculitis and hypocomplementemia as inclusion criteria.
Results
The 57 identified patients had a median age at diagnosis of 45 (range 15–83) years, and 42 (74%) were women (sex ratio 2.8). HUV was isolated in 43 (75%) patients, while the remaining 14 (25%) were associated with systemic lupus erythematosus (n=10), primary Sjögren’s syndrome (n=2), systemic sclerosis and lung cancer (n=1 each). Urticarial lesions were typically erythematous papules, more pruritic than painful, associated with angioedema (51%), purpura (35%) and/or livedo reticularis (14%). Extracutaneous manifestations included constitutional symptoms (56%), musculoskeletal (82%), ocular (56%), pulmonary (19%), gastrointestinal (18%) and/or kidney involvement (14%). HUV patients typically had low C1q-complement and normal C1-inhibitor levels, with 55% of them also having anti-C1q antibodies. Patients with anti-C1q antibodies had more frequent systemic HUV, angioedema, livedo reticularis, ocular, musculoskeletal and/or kidney involvement(s), and less frequent pulmonary and/or gastrointestinal involvement(s). Hydroxychloroquine (HCQ) or colchicine seemed to be as effective as corticosteroids as first-line therapy. For patients with relapsing and/or refractory HUV, higher cutaneous and immunological response rates were obtained with immunosuppressants, particularly azathioprine (AZA), mycophenolate mofetil (MMF), cyclophosphamide or rituximab (RTX)-based regimens, with the latter apparently more effective. Finally, cutaneous and immunological responses were strongly associated.
Conclusion
HUV is an uncommon systemic and relapsing vasculitis with various manifestations, mainly musculoskeletal and ocular. Half of the patients have anti-C1q antibodies. HCQ and colchicine should be the first-line therapy, whereas corticosteroids alone or combined with an immunosuppressant, preferentially AZA, MMF or RTX, could be alternative therapeutic options for relapsing and/or refractory disease.
Disclosure:
M. Jachiet,
None;
A. Le Quellec,
None;
A. Deroux,
None;
P. Godmer,
None;
M. Ebbo,
None;
L. Astudillo,
None;
B. Flageul,
None;
N. Dupin,
None;
S. Aractingi,
None;
L. Guillevin for the French Vasculitis Study Group,
None;
L. Mouthon,
None;
B. Terrier,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-biological-spectrum-and-therapeutic-management-of-hypocomplementemic-urticarial-vasculitis-data-from-a-french-nationwide-study-on-57-patients/