title: Non HCV-related infectious cryoglobulinemia vasculitis: Results from the French nationwide CryoVas survey and systematic review of the literature.
abstract: In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections 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, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis.
authors: Department of Internal Medicine, National Reference Center for Autoimmune Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris (AP-HP), Université Pierre Descartes, Paris 5, Paris, France. Electronic address: benjamin.terrier@cch.aphp.fr.
authors: Benjamin
authors: B
authors: Terrier
authors: Department of Internal Medicine, CHU, Rouen, France.
authors: Isabelle
authors: I
authors: Marie
authors: Department of Nephrology, CH Côte Basque, Bayonne, France.
authors: Adeline
authors: A
authors: Lacraz
authors: Department of Internal Medicine, CHU, Marseille, France.
authors: Pauline
authors: P
authors: Belenotti
authors: Department of Internal Medicine, CHU, Bordeaux, France.
authors: Fabrice
authors: F
authors: Bonnet
authors: Department of Internal Medicine, Hôpital Européen, Marseille, France.
authors: Laurent
authors: L
authors: Chiche
authors: Department of Internal Medicine, CH, Metz, France.
authors: Bruno
authors: B
authors: Graffin
authors: Department of Internal Medicine, CHU Edouard Herriot, Lyon, France.
authors: Arnaud
authors: A
authors: Hot
authors: Department of Internal Medicine, Hôpital Foch, Suresnes, France.
authors: Jean-Emmanuel
authors: JE
authors: Kahn
authors: Department of Dermatology, CH, Mulhouse, France.
authors: Catherine
authors: C
authors: Michel
authors: Department of Internal Medicine, CH, Valenciennes, France.
authors: Thomas
authors: T
authors: Quemeneur
authors: Department of Internal Medicine, CHU, Brest, France.
authors: Luc
authors: L
authors: de Saint-Martin
authors: Department of Hematology, Hôpital Necker-Enfants Malades, Paris, France.
authors: Olivier
authors: O
authors: Hermine
authors: Department of Neurology, Groupe Hospitalier Pitié-Salpetrière, Paris, France.
authors: Jean-Marc
authors: JM
authors: Léger
authors: Department of Rheumatology, AP-HP, Hôpitaux Universitaires Paris-Sud, Université Paris-Sud, INSERM U1184, Le Kremlin-Bicêtre, France.
authors: Xavier
authors: X
authors: Mariette
authors: Department of Dermatology, Tenon Hospital, AP-HP, Université Paris 6, Paris, France.
authors: Patricia
authors: P
authors: Senet
authors: Department of Nephrology and Dialysis, Tenon Hospital, AP-HP, Université Paris 6, Paris, France.
authors: Emmanuelle
authors: E
authors: Plaisier
authors: Sorbonne Universités, UPMC Univ Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), F-75005, Paris, France; INSERM, UMR_S 959, F-75013, Paris, France; CNRS, FRE3632, F-75005, Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, F-75013, Paris, France. Electronic address: patrice.cacoub@gmail.com.
authors: Patrice
authors: P
authors: Cacoub
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000890:Anti-Infective Agents / Q000627:therapeutic use; D001424:Bacterial Infections / Q000150:complications; D003449:Cryoglobulinemia / Q000175:diagnosis / Q000188:drug therapy / Q000382:microbiology; D003586:Cytomegalovirus Infections / Q000150:complications; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D005602:France / Q000453:epidemiology; D006509:Hepatitis B / Q000150:complications; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab / Q000627:therapeutic use; D011795:Surveys and Questionnaires; D056647:Systemic Vasculitis / Q000175:diagnosis / Q000188:drug therapy / Q000382:microbiology; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000890:Anti-Infective Agents; D007166:Immunosuppressive Agents; D000069283:Rituximab
keywords: Bacteria; Mixed cryoglobulinemia; Parasite; Vasculitis; Viruses