Clinical Features of Japanese Elderly Patients with Type 1 Autoimmune Hepatitis

Miyake, Yasuhiro; Iwasaki, Yoshiaki; Takaki, Akinobu; Kobashi, Haruhiko; Sakaguchi, Kohsaku; Shiratori, Yasushi

doi:10.2169/internalmedicine.46.0420

Cited by 30 publications

(42 citation statements)

References 16 publications

(17 reference statements)

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“…In this study, it was also noted that the range of CAIDs in younger patients with AIH was more diverse when compared to that presented in older patients. In a Japanese study of 160 patients with AIH-1, there was no significant difference in the frequency of CAIDs between the elderly patients ( ≥ 65 years) and younger patients ( ≤ 65 years; 18 vs. 25%, p = 39) [13] . In a study of 83 Chinese adult patients with AIH, there was no disparity in the frequency of CAIDs among the different age groups [17] .…”

Section: Effects Of Aih Types Age Gender and Genetic Backgroundmentioning

confidence: 89%

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Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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For many patients with autoimmune hepatitis (AIH), the presence of extrahepatic features is well recognised both at the time of presentation and during long-term follow-up. Concomitant ‘autoimmune disorders' have been described in 20-50% of patients with AIH, both in adults and children. Indeed, the presence of these associated phenomena has been incorporated into both the original and revised International AIH group scoring systems as an aid to codifying the diagnosis. In acute index presentations, non-specific joint pains sometimes flitting in nature have been reported in 10-60% of patients, and while joint swelling is uncommon, rheumatoid arthritis and mixed connective tissue disease have been reported in 2-4% of patients with AIH. For a majority of patients, these joint symptoms resolve within days of the introduction of immunosuppressive therapy. Rarer features at index presentation include a maculopapular skin rash and unexplained fever, which are features that tend to resolve quickly with treatment. Interestingly, joint pain and stiffness are also well recognised in the context of steroid withdrawal and cessation in AIH. The occasional co-presentation of AIH with coeliac disease is clinically important (1-6%), since for some patients, there is a risk of immunosuppression malabsorption, thus delaying effective treatment. Similarly, the co-existence of selective IgA deficiency (IgAD) can occur in patients with coeliac disease or in isolation. Selective IgAD as a co-existing extraheaptic feature seems to be more common in paediatric patients with AIH. For these patients, they are at an increased risk of respiratory and sinus infections. Although, typically associated with primary sclerosing cholangitis, the presence of inflammatory bowel disease (IBD; both Crohn's disease and ulcerative colitis) has been described in 2-8% of patients with AIH. Interestingly, for patients with autoimmune sclerosing cholangitis, a distinct pattern of IBD has been recently described. Other conditions have been reported at a lower frequency, including Sjogren's syndrome 1-7%, systemic lupus erythematosus 1-3% and glomerulonephritis 1%. Rarer still and at a frequency of <1% include fibrosing alveolitis, haemolytic anaemia, uveitis, mononeuritis multiplex, polymyositis and multiple sclerosis. In contrast, the reported associations between AIH and thyroiditis 8-23%, diabetes 1-10% and psoriasis 3% are commonly seen and notable in clinical practice.

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Section: Effects Of Aih Types Age Gender and Genetic Backgroundmentioning

confidence: 89%

“…CAIDs were shown to occur in a range of 22-46% of patients with AIH-1 from Mexico, the United Kingdom, Argentina, North America and Japan [13][14][15][22][23][24] and in 20-34% of patients with AIH-2 [15,18] .…”

Section: Effects Of Aih Types Age Gender and Genetic Backgroundmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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“…Treatment with UDCA improves serum transaminase levels in patients with type 1 AIH, but change of histological activity after UDCA treatment has been controversial (18). Furthermore, the duration from treatment initiation to normalization of serum ALT levels is longer in UDCA treatment compared with corticosteroid treatment (19). Further studies are necessary to define the optimal therapeutic strategies in patients with overlapping PBC and AIH.…”

Section: F I G U R E 4 Cl I N I C a L C O U R S Ementioning

confidence: 99%

Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome Concomitant with Systemic Sclerosis, Immune Thrombocytopenic Purpura

et al. 2009

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A 58-year-old Japanese woman presented with chronic fluctuating liver dysfunction with purpura. Raynaud's phenomenon had been diagnosed 4 years previously. At the initial examination, skin biopsy showed limited cutaneous systemic sclerosis (SSc). Laboratory investigations revealed liver dysfunction. Anti-nuclear antibodies, anti-mitochondria M2 antibody, anti-thyroglobulin antibody, and platelet-associated IgG were positive. Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) were diagnosed serologically, clinically and histologically. Immune thrombocytopenic purpura (ITP) was diagnosed by bone marrow puncture, clinical and laboratory findings, and Helicobacter pylori IgG was positive. She was treated with prednisolone 30 mg/day, ursodeoxycholic acid 600 mg/day, and a 7-day course of lansoprazole plus amoxicillin and clarithromycin. Thrombocytes increased rapidly and transaminase improved at day 7. We report a rare case of PBC-AIH overlap syndrome with concurrent ITP and SSc which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including PBC, AIH, ITP and SSc.

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“…Older patients with AIH differ from younger patients with regard to clinical and serological parameters: older patients are more likely to have cirrhosis at diagnosis, which was suggested by previous studies [Czaja and Carpenter, 2006;Floreani et al 2006;Miyake et al 2007]. In addition, older patients with AIH are more likely to have other autoimmune diseases, particularly thyroid disease [Czaja and Carpenter, 2006;Granito et al 2005].…”

Section: Autoimmune Hepatitis and Pregnancymentioning

confidence: 87%

“…However, other data show similar responses to standard therapy in older patients [Granito et al 2005;Miyake et al 2007]. Given the often indolent nature of the disease in older patients, studies are required to determine how aggressively immunosuppressive therapy is required in this population.…”

Section: Autoimmune Hepatitis and Pregnancymentioning

confidence: 98%

Management of patients with difficult autoimmune hepatitis

Parker

Adams

2012

Therap Adv Gastroenterol

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Autoimmune hepatitis (AIH) is characterized by a T-cell rich infiltrate associated with lobular and interface hepatitis, hypergammaglobulinemia and production of autoantibodies. Genetic risk is linked to the HLA particularly DRB1*0301 and DRB1*0401 alleles in North American and European Caucasian populations. It has recently been suggested that functional deficiencies in CD4 + CD25 + CD127 low FOXP3 + regulatory T cells contribute to the breakdown of immune tolerance that results in AIH. Most patients respond to immunosuppressive therapy with corticosteroids and can be maintained in remission by low-dose corticosteroid treatment and/or azathioprine. For those who progress to end-stage disease liver transplantation is an effective treatment although it is associated with recurrence. In the future it is likely that biological therapies will allow more targeted therapy designed to switch the balance to immune regulation and thereby restore immune homeostasis. Although treatment for many cases is relatively straightforward and successful problems are encountered in those who fail to respond to standard treatment, are unable to tolerate it or relapse. In such cases alternative therapies should be considered. In addition treatment is complicated in some patients by comorbidity and special care is required during and after pregnancy. We will discuss the current and future therapeutic options for patients with difficult to treat AIH.

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Clinical Features of Japanese Elderly Patients with Type 1 Autoimmune Hepatitis

Cited by 30 publications

References 16 publications

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome Concomitant with Systemic Sclerosis, Immune Thrombocytopenic Purpura

Management of patients with difficult autoimmune hepatitis

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