Background/Purpose:
Cryoglobulin (CryoGl) and cryofibrinogen (CryoFg) are cryoproteins, both responsible for well-described systemic vasculitis. Nevertheless, cryoglobulinemia may be associated with cryofibrinogenemia. Such mixed cryoprotein vasculitis (MCV) has not been properly described yet. This work aimed at determining whether the phenotype of MCV is different from CryoGl vasculitis.
Methods:
Concomitant CryoGl and CryoFg dosages performed at our University Hospital from January 2011 to December 2012 were extracted and the corresponding medical files reviewed. We included all adult patients with systemic vasculitis associated with CryoGl with or without CryoFg. As in the CryoVas study, systemic vasculitis was defined by purpura or cutaneous ulcers, or other symptoms biopsy-proven associated with a detectable cryoprotein. Qualitative variables were compared with Fisher or chi² tests and quantitative variables with Student t-test or Wilcoxon-Mann-Withney test.
Results:
Among 107 patients with cryoprotein positive dosage, 21 had MCV and 16 CryoGl vasculitis. Male:female sex ratio was respectively 1/2 and 5/3 (p=0.8). Mean age at diagnosis was respectively 58 ± 20 and 59 ± 16 years (p=0.9). Cutaneous manifestations (purpura, skin necrosis or ulceration and acrocyanosis) were similar in both groups as well as kidney (38 vs 31%) and peripheral nerve involvement (28 vs 32%). The frequency of rheumatic symptoms (arthralgia and/or myalgia) or of venous/arterial thrombotic events (43 vs 25%, p=0.4) was not different betweens both groups (51 vs 32%, p=0.2). Estimated glomerular filtration rate and cryoglobulin dosage were similar in both groups. As expected, α1 and α2globulin levels were higher in the MCV group (p<0.05). Decreased values of C3 and C4 were more frequent in the MCV group albeit not significantly (respectively, 29 vs 12% and 53 vs 35%). Regarding associated diseases, neoplasms tended to be 3 times more frequent in the MCV group (43 vs 12%, p=0.07) whereas the frequency of auto-immune disorders was similar in both groups (38% vs 50%, p=0.5). The use of systemic corticosteroids was more frequent in the MCV group (67 vs 31%, p=0.03), as well as exposure to immunosuppressives (47 vs 18%, p=0.07).
Conclusion:
Cryofibrinogemnemia is associated with a more serious disease in cryoglobulinemic patients as they are associated with an increased use of corticosteroids and immunosuppressive drugs. Neoplasms should also be carefully searched in this group.
Disclosure:
M. Michaud,
None;
G. Moulis,
None;
J. Pourrat,
None;
B. Puissant,
None;
A. Blancher,
None;
L. Sailler,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cryoglobulinemia-vasculitis-with-or-without-associated-cryofibrinogenemia-a-different-phenotype/