Cryoglobulins in primary biliary cirrhosis: prevalence and modulation by immunosuppressive therapy.
Cryoglobulins were measured in 25 patients with PBC and, for comparison purposes, in 25 age- and sex-matched normal individuals as well as 25 patients with chronic active hepatitis (CAH). Cryoglobulins were present in all patients with PBC (median protein content 18 mg/l, range 8-233) and consisted predominantly of IgM, while none of the normal controls and only 20% of the patients with CAH had cryoglobulins. In PBC, a statistically significant correlation was found between cryoglobulin-IgM concentration and other immunological measurements, such as the serum IgM level (p = 0.003) and Clq binding (p less than 0.001). Cryoglobulin-IgM also correlated significantly with alkaline phosphatase (p = 0.002) and liver fibrosis (p = 0.013), but only in a larger group of patients with PBC. In a longitudinal study of patients with PBC, no changes in the cryoglobulin concentration were found following treatment with D-penicillamine alone or placebo, but the cryoglobulin-IgM level decreased significantly during low-dose combination therapy of D-penicillamine and prednisone (median 15,4 mg/l); this was accompanied by a statistically significant decrease in serum alkaline phosphatase. The relation between cryoglobulin-IgM, serum alkaline phosphatase and liver fibrosis is discussed with regard to the pathogenesis of PBC.
D000328:Adult; D000368:Aged; D000469:Alkaline Phosphatase / Q000097:blood; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D002986:Clinical Trials as Topic; D003450:Cryoglobulins / Q000378:metabolism*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000276:immunology; D010396:Penicillamine / Q000627:therapeutic use*; D011241:Prednisone / Q000627:therapeutic use*; D011897:Random Allocation
D001323:Autoantibodies; D003450:Cryoglobulins; D007075:Immunoglobulin M; D000469:Alkaline Phosphatase; D010396:Penicillamine; D011241:Prednisone