Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose: Hepatitis C virus infection (HCV) is the main cause of mixed cryoglobulinemia vasculitis (CryoVas). Recent data are lacking regarding demographical, clinical and biological features of patients with infectious mixed CryoVas in the absence of HCV infection.
Objectives. To analyze the features of patients with infectious mixed CryoVas in the absence of HCV infection included in the French CryoVas survey. The objective of this survey is to describe the presentation and to evaluate efficacy and tolerance of treatments in patients with CryoVas.
Methods: Eighty-one French centers of Internal Medicine, Nephrology, Rheumatology, Hematology, Dermatology and Neurology from Universitary and general hospitals have included 260 patients with non-HCV mixed CryoVas diagnosed between January, 1995 and July, 2010. Among them, 18 patients presented with infectious mixed CryoVas. Demographical, clinical and biological data, as well as therapy and outcome, were assessed.
Results: 11 women and 7 men (sex ratio F/M 1.3), mean age 57.9±13.5 years, were analyzed. Infectious causes were : virus infection in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients (ascaridiasis and leishmaniosis in one case each), and leprosy and candidiasis in one case each.
Baseline manifestations were: purpura (78%), glomerulonephritis (28%), arthralgia/arthritis (28%), peripheral neuropathy (22%), necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). No gastrointestinal, central nervous system or pulmonary involvement was observed. Cryoglobulinemia was type II in 12 patients (67%) and type III in 6 (33%). Histological confirmation of vasculitis was available in 72%.
As first-line therapy, 6 patients received corticosteroids, 1 cyclophosphamide and none rituximab, but 14 patients received anti-infectious specific therapy. Among the latters, 10 were in sustained remission of the disease, 2 died of the underlying infectious disease (bacterial septicemia and Candida pneumonia), and 2 had refractory or relapsing disease related to HBV infection treated with rituximab in addition to antiviral therapy, leading to complete remission. The 4 remaining patients who did not receive specific therapy had cytomegalovirus, Epstein Barr virus, parvovirus B19 and HBV infection, and remained in remission of the CryoVas.
Conclusion: In patients with infectious mixed cryoglobulinemia vasculitis in the absence of HCV infection, virus and pyogenic bacterial infections represent the main causes. Anti-infectious specific therapy is most frequently associated with sustained remission of the disease. Thus, immunosuppressive agents should be considered only in second-line in patients with refractory and/or life-threatening vasculitis.
Disclosure:
B. Terrier,
None;
I. Marie,
None;
A. Lacraz,
None;
P. Belenotti,
None;
F. Bonnet,
None;
L. Chiche Sr.,
None;
B. Graffin Sr.,
None;
A. Hot,
None;
J. E. Kahn,
None;
T. Quemeneur,
None;
O. Hermine Sr.,
None;
J. M. Léger,
None;
P. Senet,
None;
E. Plaisier,
None;
X. Mariette,
None;
P. Cacoub Sr.,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/characteristics-of-patients-with-infectious-cryoglobulinemia-vasculitis-in-the-absence-of-hcv-infection-results-from-the-french-nationwide-cryovas-survey/