Studies in primary biliary cirrhosis
Taal, B.G.
Netherlands Journal of Medicine 24(Suppl): 1-30
1981
ISSN/ISBN: 0300-2977 PMID: 7322249 Document Number: 172480
The clinical features of PBC (primary biliary cirrhosis) have changed since the disease is more frequently diagnosed in the noncirrhotic stage. The female predisposition is still unmistakable, but nonspecific symptoms such as fatigue and pain in the region of the liver have replaced pruritus and jaundice as major symptoms. The internationally accepted diagnostic criteria for PBC lack specificity or sensitivity; the early and the end-stage of the disease are thus not readily recognizable. The newly proposed system, based on major and minor critera, distinguishes 2 levels of accuracy: definite and probable PBC. This simple system combined clinically acceptable specificity with sensitivity; its ultimate value can only be determined in other populations of patients with PBC and chronic active hepatitis. Serum IgM is a sensitive and simple screening test for PBC in patients with cholestasis. Methods to quantitate serum IgM urgently require standardization. The presence of 7S IgM in PBC is probably an expression of an accelerated immune response related to the activity of the disease. Cryoglobulins are nearly always present in PBC and consist mainly of IgM. Cryoglobulin levels correlate with some biochemical (alkaline phosphatase) and histological (liver fibrosis) markers of the disease. The finding of significantly higher serum alkaline phosphatase concentrations in 7S IgM-positive and strongly cryoglobulin positive patients with PBC supports the postulate that bile duct damage, which is the key to the PBC syndrome, may be related to the presence of immune complexes. D-penicillamine can be administered to patients with PBC with a low incidence of side effects if the go-slow-go-low dose schedule is used. A relatively high dosage is required for a beneficial effect. Combined therapy with small doses of D-penicillamine and prednisone produced marked subjective and significant objective improvement. The combination was superior to D-penicillamine alone.