Epidemiology of Autoimmune Hepatitis (AIH) in the United States Between 2014 and 2019

Tunio, Nahel A.; Mansoor, Emad; Sheriff, Mohammed Z.; Cooper, Gregory S.; Sclair, Seth N.; Cohen, Stanley M.

doi:10.1097/mcg.0000000000001449

Cited by 47 publications

(70 citation statements)

References 31 publications

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“…The performance of our algorithms may have been impacted by the underlying case‐mix of potential cases. The demographics of our AIH cohort was congruent with the previously published epidemiology of AIH in the United States, with the exception of having a slight over‐representation of Black patients at UPHS 23 . It is unknown to what extent this impacts the generalizability of our algorithm with regards to research efforts within the US and internationally.…”

Section: Discussionsupporting

confidence: 60%

Validating a novel algorithm to identify patients with autoimmune hepatitis in an administrative database

Bittermann

Mahmud

Levy

et al. 2021

Pharmacoepidemiology and Drug

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Purpose Population‐level studies on the treatment practices and comparative effectiveness of therapies in autoimmune hepatitis (AIH) are lacking due to the absence of validated methods to identify patients with AIH in large databases, such as administrative claims or electronic health records. This study ascertained the performance of International Classification of Diseases (ICD) codes for AIH, and developed and validated a novel algorithm that reliably identifies patients with AIH in health administrative data and claims. Methods This was a cross‐sectional study of patients with ≥1 inpatient or ≥2 outpatient ICD codes for AIH between 2008 and 2019 at a single health system. In a random sample of 250 patients, definite or probable AIH was determined using the Simplified AIH score, Revised AIH score or expert adjudication. The positive predictive value (PPV) was obtained. Variations of this base algorithm were evaluated using additional criteria to increase its performance. Results Of the 250 patients, 143 (57.2%) patients had sufficient records available for review. The PPV of the base algorithm was 77.6% (95% CI: 69.9–84.2%). Exclusion of patients with ≥1 ICD code for primary biliary cholangitis or primary sclerosing cholangitis yielded a PPV of 89.7% (95% CI: 82.8–94.6%). Further exclusion of patients with recent immune checkpoint inhibitor therapy increased the PPV to 92.9% (95% CI: 86.5–96.9%). Conclusions The use of ICD codes for AIH alone are insufficient to reliably identify patients with AIH in health administrative data and claims. Our proposed algorithm that includes additional diagnostic and medication‐related coding criteria demonstrates excellent performance.

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Section: Discussionsupporting

confidence: 60%

Validating a novel algorithm to identify patients with autoimmune hepatitis in an administrative database

Bittermann

Mahmud

Levy

et al. 2021

Pharmacoepidemiology and Drug

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“…Czaja et al, found disparities in the global prevalence of AIH, ranging from as high as 42.9 cases per 100,000 persons in native Alaskans to as low as 2.4 cases per 100,000 persons in children in Canada [ 92 ]. Tunio et al, performed a retrospective analysis of a population-based database and found the prevalence to be 31.2 per 100,000 in the United States [ 93 ]. Rates of AIH are higher in women than in men, with a ratio of 3.6:1 [ 94 ].…”

Section: Gut Microbiota: Link With Autoimmune Hepatitismentioning

confidence: 99%

Trust Your Gut: The Association of Gut Microbiota and Liver Disease

et al. 2022

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The gut microbiota composition is important for nutrient metabolism, mucosal barrier function, immunomodulation, and defense against pathogens. Alterations in the gut microbiome can disturb the gut ecosystem. These changes may lead to the loss of beneficial bacteria or an increase in potentially pathogenic bacteria. Furthermore, these have been shown to contribute to the pathophysiology of gastrointestinal and extra-intestinal diseases. Pathologies of the liver, such as non-alcoholic liver disease, alcoholic liver disease, cirrhosis, hepatocellular carcinoma, autoimmune hepatitis, viral hepatitis, and primary sclerosing cholangitis have all been linked to changes in the gut microbiome composition. There is substantial evidence that links gut dysbiosis to the progression and complications of these pathologies. This review article aimed to describe the changes seen in the gut microbiome in liver diseases and the association between gut dysbiosis and liver disease, and finally, explore treatment options that may improve gut dysbiosis in patients with liver disease.

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“…A possible explanation for the simultaneous occurrence of elevated liver enzymes and febrile attacks is that excessive inflammation due to pyrin inflammasome activation may exacerbate AIH. The prevalence of AIH is 2 per 2,000 FMF patients (5), which is higher than that in the general population of the United States, Denmark, and Israel (31.2, 23.9, and 11.0 per 100,000 individuals, respectively) (12)(13)(14). Although many studies have described the association between FMF and liver injury, only four cases of FMF-associated AIH have been reported to date (4).…”

Section: Discussionmentioning

confidence: 99%

Case Report: A Pediatric Case of Familial Mediterranean Fever Concurrent With Autoimmune Hepatitis

Aoki

Izawa²,

Tanaka³

et al. 2022

Front. Immunol.

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Familial Mediterranean fever (FMF) is a hereditary, autoinflammatory disease that causes recurrent fever, arthritis, and serositis. The diagnosis of FMF is based on the presentation of typical clinical symptoms and the Mediterranean fever gene (MEFV) test. However, the challenge lies in diagnosing atypical cases. In this report, we have described a pediatric patient with complex FMF whose diagnosis required trio-whole exome sequencing (WES) and functional validation of a rare MEFV variant. A 3-year-old boy presented with recurrent episodes of elevated liver enzymes and arthralgia. He was diagnosed with autoimmune hepatitis (AIH), and his liver enzymes improved rapidly with steroid treatment. However, he exhibited recurrent arthralgia and severe abdominal attacks. Trio-WES identified compound heterozygous mutations in MEFV (V726A and I692del). Ex vivo functional assays of the patient’s monocytes and macrophages, which had been pre-treated with Clostridium difficile toxin A (TcdA) and colchicine, were comparable to those of typical FMF patients, thereby confirming the diagnosis of FMF. Although he was intolerant to colchicine because of liver toxicity, subsequent administration of canakinumab successfully ameliorated his abdominal attacks. However, it was ineffective against liver injury, which recurred after steroid tapering. Therefore, in this case, the pathogenesis of AIH was probably interleukin-1β (IL-1β)-independent. In fact, AIH might have been a concurrent disease with FMF, rather than being one of its complications. Nevertheless, further studies are necessary to determine whether FMF-induced inflammasome activation contributes to AIH development. Moreover, we must consider the possibility of mixed phenotypes in such atypical patients who present distinct pathologies simultaneously.

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Epidemiology of Autoimmune Hepatitis (AIH) in the United States Between 2014 and 2019

Cited by 47 publications

References 31 publications

Validating a novel algorithm to identify patients with autoimmune hepatitis in an administrative database

Validating a novel algorithm to identify patients with autoimmune hepatitis in an administrative database

Trust Your Gut: The Association of Gut Microbiota and Liver Disease

Case Report: A Pediatric Case of Familial Mediterranean Fever Concurrent With Autoimmune Hepatitis

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