Clinical features and prognostic implications of severe corticosteroid-treated cryptogenic chronic active hepatitis

A. J. Czaja, J. E. Hay, Jorge Rakela

Research output: Contribution to journalArticle

48 Citations (Scopus)

Abstract

To assess the nature and prognosis of severe chronic active hepatitis of unknown cause, we compared 26 patients who had been fully screened for etiologic factors with 112 patients who had autoimmune chronic active hepatitis after similar durations of corticosteroid therapy (17 ± 2 versus 23 ± 2 months) and follow-up (92 ± 11 versus 103 ± 7 months). Patients with cryptogenic disease could not be distinguished from those with autoimmune disease on the basis of age, sex distribution, duration of illness, immunoglobulin levels, frequency of concurrent immunologic disorders, or histologic findings. Serum γ-globulin levels were higher (3.4 ± 0.1 versus 2.5 ± 0.2 g/dl, P = 0.007) and albumin levels were lower (2.9 ± 0.1 versus 3.3 ± 0.1 g/dl, P = 0.003) in patients with autoimmune disease than in those with cryptogenic disease, but individual findings did not differentiate the patients. Remission (69 versus 75%), treatment failure (23 versus 13%), relapse after drug withdrawal (67 versus 68%), progression to cirrhosis (57 versus 36%), and death from hepatic failure (12 versus 11%) occurred as commonly in patients with cryptogenic as in those with autoimmune disease. Patients with different constellations of immunoserologic findings were similar clinically. We conclude that patients with severe chronic active hepatitis who have been fully screened for etiologic factors cannot be distinguished from patients with autoimmune disease of comparable severity. These two groups of patients have a similar prognosis after corticosteroid therapy, and such treatment should be considered in these highly selected patients.

Original languageEnglish (US)
Pages (from-to)23-30
Number of pages8
JournalMayo Clinic Proceedings
Volume65
Issue number1
StatePublished - 1990

Fingerprint

Chronic Hepatitis
Adrenal Cortex Hormones
Autoimmune Diseases
Serum Globulins
Autoimmune Hepatitis
Sex Distribution
Age Distribution
Liver Failure
Treatment Failure
Immunoglobulins
Albumins
Fibrosis
Therapeutics
Recurrence

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Clinical features and prognostic implications of severe corticosteroid-treated cryptogenic chronic active hepatitis. / Czaja, A. J.; Hay, J. E.; Rakela, Jorge.

In: Mayo Clinic Proceedings, Vol. 65, No. 1, 1990, p. 23-30.

Research output: Contribution to journalArticle

@article{a3742eca7eeb45b2a93915efee44d23e,
title = "Clinical features and prognostic implications of severe corticosteroid-treated cryptogenic chronic active hepatitis",
abstract = "To assess the nature and prognosis of severe chronic active hepatitis of unknown cause, we compared 26 patients who had been fully screened for etiologic factors with 112 patients who had autoimmune chronic active hepatitis after similar durations of corticosteroid therapy (17 ± 2 versus 23 ± 2 months) and follow-up (92 ± 11 versus 103 ± 7 months). Patients with cryptogenic disease could not be distinguished from those with autoimmune disease on the basis of age, sex distribution, duration of illness, immunoglobulin levels, frequency of concurrent immunologic disorders, or histologic findings. Serum γ-globulin levels were higher (3.4 ± 0.1 versus 2.5 ± 0.2 g/dl, P = 0.007) and albumin levels were lower (2.9 ± 0.1 versus 3.3 ± 0.1 g/dl, P = 0.003) in patients with autoimmune disease than in those with cryptogenic disease, but individual findings did not differentiate the patients. Remission (69 versus 75{\%}), treatment failure (23 versus 13{\%}), relapse after drug withdrawal (67 versus 68{\%}), progression to cirrhosis (57 versus 36{\%}), and death from hepatic failure (12 versus 11{\%}) occurred as commonly in patients with cryptogenic as in those with autoimmune disease. Patients with different constellations of immunoserologic findings were similar clinically. We conclude that patients with severe chronic active hepatitis who have been fully screened for etiologic factors cannot be distinguished from patients with autoimmune disease of comparable severity. These two groups of patients have a similar prognosis after corticosteroid therapy, and such treatment should be considered in these highly selected patients.",
author = "Czaja, {A. J.} and Hay, {J. E.} and Jorge Rakela",
year = "1990",
language = "English (US)",
volume = "65",
pages = "23--30",
journal = "Mayo Clinic Proceedings",
issn = "0025-6196",
publisher = "Elsevier Science",
number = "1",

}

TY - JOUR

T1 - Clinical features and prognostic implications of severe corticosteroid-treated cryptogenic chronic active hepatitis

AU - Czaja, A. J.

AU - Hay, J. E.

AU - Rakela, Jorge

PY - 1990

Y1 - 1990

N2 - To assess the nature and prognosis of severe chronic active hepatitis of unknown cause, we compared 26 patients who had been fully screened for etiologic factors with 112 patients who had autoimmune chronic active hepatitis after similar durations of corticosteroid therapy (17 ± 2 versus 23 ± 2 months) and follow-up (92 ± 11 versus 103 ± 7 months). Patients with cryptogenic disease could not be distinguished from those with autoimmune disease on the basis of age, sex distribution, duration of illness, immunoglobulin levels, frequency of concurrent immunologic disorders, or histologic findings. Serum γ-globulin levels were higher (3.4 ± 0.1 versus 2.5 ± 0.2 g/dl, P = 0.007) and albumin levels were lower (2.9 ± 0.1 versus 3.3 ± 0.1 g/dl, P = 0.003) in patients with autoimmune disease than in those with cryptogenic disease, but individual findings did not differentiate the patients. Remission (69 versus 75%), treatment failure (23 versus 13%), relapse after drug withdrawal (67 versus 68%), progression to cirrhosis (57 versus 36%), and death from hepatic failure (12 versus 11%) occurred as commonly in patients with cryptogenic as in those with autoimmune disease. Patients with different constellations of immunoserologic findings were similar clinically. We conclude that patients with severe chronic active hepatitis who have been fully screened for etiologic factors cannot be distinguished from patients with autoimmune disease of comparable severity. These two groups of patients have a similar prognosis after corticosteroid therapy, and such treatment should be considered in these highly selected patients.

AB - To assess the nature and prognosis of severe chronic active hepatitis of unknown cause, we compared 26 patients who had been fully screened for etiologic factors with 112 patients who had autoimmune chronic active hepatitis after similar durations of corticosteroid therapy (17 ± 2 versus 23 ± 2 months) and follow-up (92 ± 11 versus 103 ± 7 months). Patients with cryptogenic disease could not be distinguished from those with autoimmune disease on the basis of age, sex distribution, duration of illness, immunoglobulin levels, frequency of concurrent immunologic disorders, or histologic findings. Serum γ-globulin levels were higher (3.4 ± 0.1 versus 2.5 ± 0.2 g/dl, P = 0.007) and albumin levels were lower (2.9 ± 0.1 versus 3.3 ± 0.1 g/dl, P = 0.003) in patients with autoimmune disease than in those with cryptogenic disease, but individual findings did not differentiate the patients. Remission (69 versus 75%), treatment failure (23 versus 13%), relapse after drug withdrawal (67 versus 68%), progression to cirrhosis (57 versus 36%), and death from hepatic failure (12 versus 11%) occurred as commonly in patients with cryptogenic as in those with autoimmune disease. Patients with different constellations of immunoserologic findings were similar clinically. We conclude that patients with severe chronic active hepatitis who have been fully screened for etiologic factors cannot be distinguished from patients with autoimmune disease of comparable severity. These two groups of patients have a similar prognosis after corticosteroid therapy, and such treatment should be considered in these highly selected patients.

UR - http://www.scopus.com/inward/record.url?scp=0025061012&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0025061012&partnerID=8YFLogxK

M3 - Article

VL - 65

SP - 23

EP - 30

JO - Mayo Clinic Proceedings

JF - Mayo Clinic Proceedings

SN - 0025-6196

IS - 1

ER -