Active `juvenile' cirrhosis considered as part of a systemic disease and the effect of corticosteroid therapy

Abstract

EDITORIAL SYNOPSIS This is a clinical study of 81 patients (32 male and 49 female), who are not alcoholics, with jaundice of hepato-cellular type lasting longer than three months. Forty-nine of these patients were under 21 years of age. The natural history of the condition and the response to corticosteroid therapy is described; good symptomatic relief can be achieved with corticosteroids but they do not seem to prolong life. The aetiology is discussed with special reference to the relationship to viral hepatitis, to systemic lupus erythematosus, and to other disturbances of immunity.

Hyperglobulinaemic Renal Tubular Acidosis: A Report of Nine Cases

“…Three patients in our series had a history of recurrent attacks of purpura, and in none was a coagulation defect or a cryoglobulin found. Two of our cases had active chronic hepatitis, and this association has been reported previously (Morris and Fudenberg, 1967;Read et al, 1963;Seedat and Raine, 1965). The concurrence of renal tubular acidosis with primary biliary cirrhosis has been recorded only once (Leeson and Fourman, 1967), and the defect of renal acidification in this patient was thought to be caused by a raised level of serum copper.…”

Hyperglobulinaemic Renal Tubular Acidosis: A Report of Nine Cases

“…Three patients in our series had a history of recurrent attacks of purpura, and in none was a coagulation defect or a cryoglobulin found. Two of our cases had active chronic hepatitis, and this association has been reported previously (Morris and Fudenberg, 1967;Read et al, 1963;Seedat and Raine, 1965). The concurrence of renal tubular acidosis with primary biliary cirrhosis has been recorded only once (Leeson and Fourman, 1967), and the defect of renal acidification in this patient was thought to be caused by a raised level of serum copper.…”

Ulcerative colitis with chronic liver disease, eosinophilia and auto-immune thyroid disease.

“…Although the hepatic disorder in the patient described in this report has run a benign course consistent with the histological finding of chronic persistent hepatitis, it has had some of the serological accompaniments normally associated with chronic aggressive hepatitis-a persistently positive smooth muscle antibody and a substantially raised concentration of serum IgG. Antibodies to thyroglobulin have an increased incidence in women with chronic active hepatitis (Doniach et al, 1966) and there is an increased incidence of thyrotoxicosis and Hashimoto's thyroiditis in these patients (Read, Sherlock and Harrison, 1963;Doniach et al, 1966). This is of particular interest in view of the present patient's myxoedema and the high titre antibody to thyroglobulin in her serum.…”

“…There was also a steep rise in the eosinophil count at the time of her presumed hypersensitivity reaction to sulphasalazine; this was similar to the eosinophilia noted with pulmonary allergy to this drug (Jones and Malone, 1972). A mild blood eosinophilia is not uncommon in patients with ulcerative colitis, the counts raising in relapse and falling in remission (Riisager, 1959;Riis and Anthonisen, 1964;Wright and Truelove, 1966) and a blood eosinophilia is seen, but to a lesser extent, in Crohn's disease (Haeberle and Griffen, 1972). Tissue eosinophilia occurs in 80% of patients with ulcerative colitis, and eosinophils are frequently found on cytological examination of the rectal mucus of such patients (Riis and Anthonisen, 1964;Anthonisen and Riis, 1971).…”

Ulcerative Colitis and Cirrhosis of the Liver [ Abridged ]

“…One patient had an atypical biliary cirrhosis, and at autopsy the intrahepatic bile ducts were full of small stones. Eight patients with ulcerative colitis had an 'active' cirrhosis histologically, according to the criteria of Read et al (1963), and the clinical features of the cirrhosis were consistent with this. All had hypergammaglobulinemia and one had LE cells in the peripheral blood.…”

Hyperglobulinaemic Renal Tubular Acidosis: A Report of Nine Cases

“…Three patients in our series had a history of recurrent attacks of purpura, and in none was a coagulation defect or a cryoglobulin found. Two of our cases had active chronic hepatitis, and this association has been reported previously (Morris and Fudenberg, 1967;Read et al, 1963;Seedat and Raine, 1965). The concurrence of renal tubular acidosis with primary biliary cirrhosis has been recorded only once (Leeson and Fourman, 1967), and the defect of renal acidification in this patient was thought to be caused by a raised level of serum copper.…”

Ulcerative colitis with chronic liver disease, eosinophilia and auto-immune thyroid disease.

“…Although the hepatic disorder in the patient described in this report has run a benign course consistent with the histological finding of chronic persistent hepatitis, it has had some of the serological accompaniments normally associated with chronic aggressive hepatitis-a persistently positive smooth muscle antibody and a substantially raised concentration of serum IgG. Antibodies to thyroglobulin have an increased incidence in women with chronic active hepatitis (Doniach et al, 1966) and there is an increased incidence of thyrotoxicosis and Hashimoto's thyroiditis in these patients (Read, Sherlock and Harrison, 1963;Doniach et al, 1966). This is of particular interest in view of the present patient's myxoedema and the high titre antibody to thyroglobulin in her serum.…”

“…There was also a steep rise in the eosinophil count at the time of her presumed hypersensitivity reaction to sulphasalazine; this was similar to the eosinophilia noted with pulmonary allergy to this drug (Jones and Malone, 1972). A mild blood eosinophilia is not uncommon in patients with ulcerative colitis, the counts raising in relapse and falling in remission (Riisager, 1959;Riis and Anthonisen, 1964;Wright and Truelove, 1966) and a blood eosinophilia is seen, but to a lesser extent, in Crohn's disease (Haeberle and Griffen, 1972). Tissue eosinophilia occurs in 80% of patients with ulcerative colitis, and eosinophils are frequently found on cytological examination of the rectal mucus of such patients (Riis and Anthonisen, 1964;Anthonisen and Riis, 1971).…”

Ulcerative Colitis and Cirrhosis of the Liver [ Abridged ]

“…One patient had an atypical biliary cirrhosis, and at autopsy the intrahepatic bile ducts were full of small stones. Eight patients with ulcerative colitis had an 'active' cirrhosis histologically, according to the criteria of Read et al (1963), and the clinical features of the cirrhosis were consistent with this. All had hypergammaglobulinemia and one had LE cells in the peripheral blood.…”

Hyperglobulinaemic Renal Tubular Acidosis: A Report of Nine Cases

“…Three patients in our series had a history of recurrent attacks of purpura, and in none was a coagulation defect or a cryoglobulin found. Two of our cases had active chronic hepatitis, and this association has been reported previously (Morris and Fudenberg, 1967;Read et al, 1963;Seedat and Raine, 1965). The concurrence of renal tubular acidosis with primary biliary cirrhosis has been recorded only once (Leeson and Fourman, 1967), and the defect of renal acidification in this patient was thought to be caused by a raised level of serum copper.…”

Ulcerative colitis with chronic liver disease, eosinophilia and auto-immune thyroid disease.

“…Although the hepatic disorder in the patient described in this report has run a benign course consistent with the histological finding of chronic persistent hepatitis, it has had some of the serological accompaniments normally associated with chronic aggressive hepatitis-a persistently positive smooth muscle antibody and a substantially raised concentration of serum IgG. Antibodies to thyroglobulin have an increased incidence in women with chronic active hepatitis (Doniach et al, 1966) and there is an increased incidence of thyrotoxicosis and Hashimoto's thyroiditis in these patients (Read, Sherlock and Harrison, 1963;Doniach et al, 1966). This is of particular interest in view of the present patient's myxoedema and the high titre antibody to thyroglobulin in her serum.…”

“…There was also a steep rise in the eosinophil count at the time of her presumed hypersensitivity reaction to sulphasalazine; this was similar to the eosinophilia noted with pulmonary allergy to this drug (Jones and Malone, 1972). A mild blood eosinophilia is not uncommon in patients with ulcerative colitis, the counts raising in relapse and falling in remission (Riisager, 1959;Riis and Anthonisen, 1964;Wright and Truelove, 1966) and a blood eosinophilia is seen, but to a lesser extent, in Crohn's disease (Haeberle and Griffen, 1972). Tissue eosinophilia occurs in 80% of patients with ulcerative colitis, and eosinophils are frequently found on cytological examination of the rectal mucus of such patients (Riis and Anthonisen, 1964;Anthonisen and Riis, 1971).…”

Ulcerative Colitis and Cirrhosis of the Liver [ Abridged ]

“…One patient had an atypical biliary cirrhosis, and at autopsy the intrahepatic bile ducts were full of small stones. Eight patients with ulcerative colitis had an 'active' cirrhosis histologically, according to the criteria of Read et al (1963), and the clinical features of the cirrhosis were consistent with this. All had hypergammaglobulinemia and one had LE cells in the peripheral blood.…”