Background Cryoglobulinemic vasculitis (Vcrio) is characterized by the presence of circulating cryoglobulins usually affecting small blood vessel. The Nomenclature of Chapel Hill 1993 (A&R 1994;37:187) considered all VCrio generically. However, VCrio may be idiopathic (essential) or associated with chronic infections, mainly due to hepatitis C virus (HCV), autoimmune diseases and hematological malignancies. The new Nomenclature of Chapel Hill 2012 (A&R 2013:65:1) considered as Vcrio only the essential, and the remaining cases as Vcrio associated to other processes.
Objectives Our aim was to study the frequency and characteristics of Vcrio according to this new classification in a defined population.
Methods Study of a series of Vcrio from a universitary hospital. Circulating cryoglobulins (cryocrit>1%) and vasculitic involvement of a target organ, especially skin, peripheral nerve and/or kidney was required to make a diagnosis. For this purpose we used the criteria mentioned above (Chapel Hill-2012). According to them, VCrio were divided into primary and associated with other processes. A comparative study between both groups was performed.
Results We studied 34 patients (15 men/19 women) with a mean±SD age of 53±15 years (range, 27-81). The essential Vcrio was observed in 12 cases. In the remaining cases it was associated with one or more of the following: a) HCV (15 cases), b) HBV (2 cases), c) autoimmune disease (6 cases; 4 Sjögren´s syndrome, 1 RA, 1 sarcoidosis, 1 SLE, 1 primary billiary cirrhosis) and d)hematological disorders (4 cases, 2 Hodgkin lymphomas, 1 NHL and 1 MALT lymphoma).
The comparative study between primary Vcrio versus secondary (expressed always in this order) showed that the clinical vasculitic manifestations were similar in both groups: cutaneous (92% vs 86%, p=0.6), joint (75% vs 55%, p=0.2), kidney (33% vs 41%, p=0.6), peripheral neuropathy (25% vs 36%, p=0.5), Raynaud’s phenomenon (17% vs 14%, p=0.8), and digestive (8% vs 18%, p=0.4). The main laboratory findings were elevated ESR (75% vs 95%, p=0.1), leukocytosis (8% vs 5%, p=0.6), anemia (17% vs 50%, p=0.05). Immunological tests were determined in 29 cases being similar in both groups; positive Rheumatoid Factor (82% vs 83%, p=0.9) and decreased C3 and/or C4 (64% vs 56%, p=0.6). Positive Antinuclear Antibodies were more frequently observed in secondary Vcrio (0% vs 35%, p=0.02) and cryocrit in primary VCrio (25.5% vs 3%, p=0.02).
Comparative study of treatment in primary Vcrio vs secondary was: a)NSAIDs (67% vs 20%, p=0.01), b)steroids (50% vs 71%, p=0.2), c)cytotoxic (40% vs 48%, p=0.7) and d)plasmapheresis (0% vs 18%, p=0.1). In 13.3% of patients with HCV-associated Vcrio combined antiviral therapy was used (interferon and ribavirin).
After a median (IQR) of follow-up of 42 (8-99) months, relapses were observed in 45% vs 67%, (p=0.3) and complete recovery in 42% vs 13%,(p=0.9).
Conclusions Overall, most clinical and laboratory manifestations related to vasculitis in the setting of both primary VCrio and that associated-to other conditions are similar. In any case their differentiation following the Nomenclature of Chapel Hill-2012 is useful.
Acknowledgements This study was supported by grants from “Fondo de Investigaciones Sanitarias”PI12/00193 (Spain). This work was also partially supported by RETICS Program, RD08/0075 (RIER) from “Instituto de Salud Carlos III” (ISCIII) (Spain).
Disclosure of Interest None Declared