Recurrent Severe Hepatitis of Autoimmune Origin

Abstract The autoimmune liver disease constituent conditions include autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis and IgG-4 associated cholangitis. They remain a diagnostic challenge to the practicing physician due to their close resemblance in clinical course, and laboratory and imaging findings to the vast array of other etiologies of liver injury. We report a case of recurrent severe hepatitis of autoimmune origin in a female patient. The disease course was marked by initial onset at age 39, followed by nearly four years of remission, and a second flare with a more exaggerated severity. Systemic lupus erythematosus was initially deemed as the culprit, however formal diagnostic criteria were not fulfilled and the serological findings were not reproduced at a later date. With the aim of ascertaining the underlying process, the patient underwent an extensive array of testing with regards to infectious, genetic, systemic and autoimmune disease. Positive anti-dsDNA (double stranded DNA) and an antinuclear antibody titer of 1:160 provided the strongest support for an autoimmune etiology, specifically autoimmune hepatitis or possibly an overlap syndrome. An excellent outcome was achieved via treatment with corticosteroids, ursodeoxycholic acid and plasmapheresis.

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Overlap Syndrome of Autoimmune Hepatitis, Primary Biliary Cholangitis, and Primary Sclerosing Cholangitis in a Patient with Systemic Lupus Erythematosus: A Case Report of Complex Autoimmune Liver Disease

The American Journal of Gastroenterology ◽

10.14309/00000434-201610001-01886 ◽

2016 ◽

Vol 111 ◽

pp. S902-S903

Author(s):

Mouhanna Abu ghanimeh ◽

Alisa Likhitsup ◽

Audrey Bearden ◽

Osama Kaddourah ◽

Tarek Tamimi

Keyword(s):

Systemic Lupus Erythematosus ◽

Case Report ◽

Liver Disease ◽

Autoimmune Hepatitis ◽

Primary Sclerosing Cholangitis ◽

Lupus Erythematosus ◽

Sclerosing Cholangitis ◽

Overlap Syndrome ◽

Primary Biliary Cholangitis ◽

Systemic Lupus

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Autoimmune Hepatitis and Primary Biliary Cholangitis Overlap in a Patient with Systemic Lupus Erythematosus and Rheumatoid Arthritis Overlap (Rhupus) - An Unusual Association

International Journal of Health Sciences and Research ◽

10.52403/ijhsr.20220113 ◽

2022 ◽

Vol 12 (1) ◽

pp. 92-98

Author(s):

Anu Yarky ◽

Priyesh Sharma ◽

Vipan Kumar

Keyword(s):

Systemic Lupus Erythematosus ◽

Autoimmune Hepatitis ◽

Lupus Erythematosus ◽

Treatment Plan ◽

Liver Function Tests ◽

Overlap Syndrome ◽

Primary Biliary Cholangitis ◽

Systemic Lupus ◽

Adequate Treatment ◽

Clinical Presentations

The diagnosis of overlap syndrome involving systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) isn’t easily established due to its similar clinical presentations and biochemical features to those of lupus hepatitis. The term overlap syndrome is typically utilized in the context of overlap of autoimmune hepatitis with PSC (primary sclerosing cholangitis) or PBC (primary biliary cholangitis). Few rare cases of AIH complicated by SLE are reported within the literature. Overlapping of SLE and AIH should be suspected when patients with SLE have abnormal liver function tests or AIH patients present with a rash. Liver biopsy plays a really important role in establishing the medical diagnosis of SLE with liver impairment or overlap with AIH. The prompt diagnosis and adequate treatment plan can improve the disease outcome. Key words: autoimmune hepatitis, primary biliary cholangitis, systemic lupus erythematosus, overlap syndrome.

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Non-viral aspects

Thai Journal of Hepatology ◽

10.30856/th.jhep2018vol1iss2_09 ◽

2018 ◽

Vol 1 (2) ◽

pp. 37-39

Author(s):

Roongruedee Chaiteerakij

Keyword(s):

Autoimmune Hepatitis ◽

Liver Diseases ◽

Sclerosing Cholangitis ◽

Promising Result ◽

Primary Biliary Cholangitis ◽

Second Line Therapy ◽

Thai Population ◽

The Past ◽

Line Therapy ◽

New Treatment

Autoimmuneliver diseases, particularly autoimmune hepatitis and primary biliary cholangitis,are not uncommon among the Thai population. This article summarizes main findings of studies of autoimmune liver diseases published during the past year, which included natural history and long-termoutcomes of primary biliary cholangitis treatment, a promising result of the new treatment for primary sclerosing cholangitis and outcomes of a second-line therapy of autoimmune hepatitis.

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Dual threat of comorbidity of celiac disease and systemic lupus erythematosus

Journal of International Medical Research ◽

10.1177/03000605211012258 ◽

2021 ◽

Vol 49 (5) ◽

pp. 030006052110122

Author(s):

Yimin Ma ◽

Duanming Zhuang ◽

Zhenguo Qiao

Keyword(s):

Systemic Lupus Erythematosus ◽

Celiac Disease ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Failure To Thrive ◽

Severe Malnutrition ◽

Systemic Lupus ◽

Electrolyte Disorders ◽

Diagnostic Challenge ◽

Immune Mediated

Celiac disease (CD) is a chronic immune-mediated intestinal disease that is characterized by production of autoantibodies directed against the small intestine. The main clinical manifestations of CD are typically defined as those related to indigestion and malabsorption. These manifestations include unexplained diarrhea or constipation, abdominal pain, bloating, weight loss, anemia, failure-to-thrive in children, and decreased bone density. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous clinical manifestations, which may also involve the gastrointestinal tract. Comorbidity of CD and SLE is rare, and the overlapping symptoms and nonspecific clinical presentation may pose a diagnostic challenge to clinicians. We report here a case of SLE with CD, which mainly manifested as recurrent diarrhea, uncorrectable electrolyte disorders, and severe malnutrition. Through review, we hope to further improve our understanding and diagnostic level of this combination of diseases.

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Association of coexisting anti-ribosomal P and anti-dsDNA antibodies with histology and renal prognosis in lupus nephritis patients

Lupus ◽

10.1177/0961203320983906 ◽

2021 ◽

pp. 096120332098390

Author(s):

Ayako Wakamatsu ◽

Hiroe Sato ◽

Yoshikatsu Kaneko ◽

Takamasa Cho ◽

Yumi Ito ◽

...

Keyword(s):

Lupus Nephritis ◽

Lupus Erythematosus ◽

Renal Outcome ◽

Class V ◽

Double Stranded Dna ◽

Number Of Patients ◽

Renal Histology ◽

Renal Prognosis ◽

Incidence Of Ckd ◽

Ribosomal P

Objectives Anti-ribosomal P protein autoantibodies (anti-P) specifically develop in patients with systemic lupus erythematosus. Associations of anti-P with lupus nephritis (LN) histological subclass and renal outcome remain inconclusive. We sought to determine the association of anti-P and anti-double-stranded DNA antibody (anti-dsDNA) with renal histology and prognosis in LN patients. Methods Thirty-four patients with LN, having undergone kidney biopsy, were included. The 2018 revised ISN/RPS classification system was used for pathophysiological evaluation. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate < 60 mL/min/1.73 m2 for > 3 months. Results Six patients (17.6%) were positive for anti-P and 26 (76.5%) for anti-dsDNA. Among the six patients with anti-P, one did not have anti-dsDNA, but did have anti-Sm antibody, and showed a histological subtype of class V. This patient maintained good renal function for over 14 years. The remaining five patients, who had both anti-P and anti-dsDNA, exhibited proliferative nephritis and were associated with prolonged hypocomplementemia, and the incidence of CKD did not differ from patients without anti-P. Conclusion Although this study included a small number of patients, the results indicated that histology class and renal prognosis associated with anti-P depend on the coexistence of anti-dsDNA. Further studies with a large number of patients are required to confirm this conclusion.

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Longterm Outcomes and Damage Accrual in Patients with Childhood Systemic Lupus Erythematosus with Psychosis and Severe Cognitive Dysfunction

The Journal of Rheumatology ◽

10.3899/jrheum.121096 ◽

2013 ◽

Vol 40 (4) ◽

pp. 513-519 ◽

Cited By ~ 20

Author(s):

Lily Siok Hoon Lim ◽

Arlette Lefebvre ◽

Susanne Benseler ◽

Earl D. Silverman

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Cognitive Dysfunction ◽

Lupus Erythematosus ◽

Clinical Course ◽

Immunosuppressive Treatment ◽

Damage Index ◽

Response To Treatment ◽

Systemic Lupus ◽

Damage Accrual

Objective.(1) To describe the clinical course and response to treatment; and (2) to evaluate and compare damage accrual of distinct phenotypic subgroups of patients with clinically important psychiatric illness of pediatric systemic lupus erythematosus (pSLE).Methods.A single-center cohort study of patients with pSLE followed at a pediatric lupus clinic from 1985 to July 2009. Clinical course and response to treatment were studied. Remission was defined by absence of psychiatric/cognitive symptoms while receiving minimal doses of prednisone. Disease activity and damage were measured using SLE Disease Activity Index and SLE Damage Index.Results.Fifty-three children were included: 40 with psychosis and cognitive dysfunction (PSYC group) and 13 with isolated cognitive dysfunction (COG group). All received immunosuppressive treatment. Eighteen of 32 treated with azathioprine required a change to cyclophosphamide for poor response but none on cyclophosphamide required a change. The median times to remission were 72 weeks (PSYC) and 70 weeks (COG). Eight patients (7 PSYC, 1 COG) experienced flare following response/remission. New damage was noted in 50% of children at a median of 11 months: 57% of PSYC group, 31% of COG group. Persistent cognitive dysfunction was seen in 16% of PSYC patients and 15% of COG patients.Conclusion.Most patients responded to immunosuppressive treatment, although median time to remission was > 1 year. Roughly half the patients acquired a new damage item, most of which did not interfere with functional abilities. Fewer than 20% of patients developed neuropsychiatric damage. Both phenotypes of psychiatric pSLE responded equally well to current treatment.

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