Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long‐term outcomes

Abstract

Background: Concurrent extrahepatic autoimmune disease (CEHAID) associated with

Extrahepatic Autoimmune Diseases are Prevalent in Autoimmune Hepatitis Patients and Their First-Degree Relatives: Survey Study (Preprint)

“…We found a high percentage of EHAD within FDR cases (55.9%), compared with only two other published reports that examined EHAD among FDR (42% in the Netherlands [6] and 16% in the United Kingdom [14]). In fact, the odds of having an FDR with EHAD in our cases was double that of controls in an adjusted model.…”

“…and 42% [8,13,14]. In our multivariate model, odds of an EHAD in cases were almost two and a half times that of controls.…”

“…Low EHAD in FDR estimates found in the United Kingdom study are likely artificially low due to study methodology that was founded in medical record review [14].…”

“…Further assessment of EHAD among specific FDRs is shown in Mother, n (%) n=295 n=1110 95 (32) 274 (25) 0.01 Father, n (%) n = 295 n = 1110 45 (15) 130 (12) NS Siblings, n (%) n = 600 n= 1799 82 (14) 120 (7) 0.001 Children, n (%) n = 411 n = 874 36 (9) 40 (5) The findings of our case-control study provide a similar estimate of concurrent EHAD in AIH compared to prior descriptive European reports that document the prevalence between 26…”

“…Beyond limited genome wide data and prior candidate gene studies, descriptive European reports support an underlying autoimmune phenotype, as observation of extrahepatic autoimmune diseases (EHAD) occurring concurrently with AIH has been as high as 42% [6,13,14]. To date, only two European studies have examined AIH patients' family history of EHAD by collecting survey data and retrospectively reviewing patient records [6,14]. A casecontrol assessment of EHAD among AIH patients and their first-degree relatives also remains incomplete.…”