Complement system is not activated in primary biliary cirrhosis.

[benign recurrent intrahepatic cholestasis]

There is controversial evidence suggesting that the classical pathway of complement system is chronically activated in primary biliary cirrhosis (PBC ) and that complement activation may be important in development of bile duct injury . We have reevaluated this issue by measuring by -products of complement activation such as C 4 a , C 3 a , Bb , and terminal complement complexes (SC 5 b -9 ) in plasma of 44 PBC patients with sensitive methods not previously used to detect complement activation in this disease . Age-matched healthy women and patients with chronic hepatitis of different etiology were studied as controls . We found that PBC patients have normal C 4 a concentrations . This finding argues strongly against chronic classical pathway activation . Although a minor increase of C 3 a levels was observed in a minority of PBC patients , the C 3 a /C 3 ratio , an index used to evaluate the extent of native protein conversion , was remarkably similar in all groups . Potentially lytic terminal complement complexes were not increased . PBC patients had normal Bb plasma levels , indicating that the alternative pathway is also not activated . C 3 concentration was higher in PBC patients than in healthy subjects and in chronic hepatitis patients , particularly in the early stages of the disease . C 3 and C 4 concentrations became lower in PBC and chronic hepatitis with the progression of the disease . The increase of C 3 concentration in PBC does not reflect liver inflammation , since serum levels of C-reactive protein are normal . We found high serum C 3 levels in patients with rare chronic cholestatic syndromes without superimposed infections and observed that serum C 3 levels paralleled those of bilirubin in a patient with benign recurrent intrahepatic cholestasis . In conclusion , our data indicate that complement is not activated in PBC and that the increase of serum C 3 levels is related to cholestasis .

Diseases
Validation

Diseases presenting "high serum" symptom

benign recurrent intrahepatic cholestasis

classical phenylketonuria

familial hypocalciuric hypercalcemia

omenn syndrome

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