Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis

Abstract

Background & Aims Most studies on autoimmune hepatitis (AIH) in children are in predominantly Caucasian cohorts. Paediatric AIH in African Americans (AA) is understudied, with a dearth of clinical predictors of outcome, often leading to serious complications and even mortality. The aim of the study was to define disease presentation, progression, response to therapy and outcomes in paediatric AIH in a well‐defined, large, single centre, demographically diverse population. Methods We conducted a review of patients with AIH who were followed at this tertiary liver transplant centre. Clinical and laboratory covariates were assessed with regard to disease presentation, progression and outcomes in AA vs Non‐AA children. Results African Americans patients constituted 42% of this cohort. At 1‐year follow‐up, AA children were receiving significantly higher doses of steroids compared to non‐AA. More AA presented with end‐stage liver disease (ESLD) with high immunoglobulin G and GGT:platelet ratio. After adjusting for other risk factor variables like gender, age at presentation and ESLD, AA children were at 4.5 times higher risk for significant outcome liver transplant/death within the first 12 months of presentation. Post‐transplant, recurrent AIH was seen in 50% of AA vs 8% in non‐AA. Conclusions African American patients with AIH are more likely to present with ESLD and have an increased early risk for transplantation with high likelihood of disease recurrence post‐transplantation. Studies are needed to delineate factors such as inherent biology, genetics and access to care. Early referral and tailored immunosuppressive regimens are required for AA patients with AIH.

Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases

“…Alaskan Natives have a high frequency of icteric AIH at presentation, Hispanics more commonly present with cirrhosis, and African Americans have accelerated progression of disease and a higher rate of recurrence after LT compared to other races. (5,6) Female predominance occurs in adults (71%-95% women) (7)(8)(9)(10)(11)(12) and children (60%-76% girls). (13)(14)(15)(16) Early epidemiological reports suggested that the onset of AIH had age peaks at 10-30 and 40-60 years, but the findings may have been influenced by referral bias.…”

The Relationship between Hepatic Myeloid-Derived Suppressor Cells and Clinicopathological Parameters in Patients with Chronic Liver Disease

“…As we all know, GGT is positively correlated with the severity of liver disease. Palle et al had demonstrated that more AIH in African Americans presented with end-stage liver disease (ESLD) with high GGT [29]. Recently, lipid metabolism has been found to play an important role in the developmental differentiation and typing of various immune cells.…”

Perfil clínico de pacientes pediátricos con diagnóstico de hepatitis autoinmune en un centro de alta complejidad del suroccidente colombiano

“…9 Esto coincide con los hallazgos encontrados por Porta et al en la mayor cohorte de pacientes reportada en Brasil (y también para toda Latinoamérica), con una muestra de 828 pacientes, donde el 89,6% se clasificaron como HAI tipo I y fue predominante en el sexo femenino. 10 Vale la pena destacar que estos hallazgos no son solo equiparables a los datos presentados en cohortes latinoamericanas, sino que también coinciden con lo reportado en la literatura mundial, como se evidencia en la serie más grande de pacientes asiáticos con HAI descrita por Lee Way et al, donde un 59% de los pacientes correspondían al sexo femenino y la mayoría se presentaron como HAI tipo I, en un 56%. 11 Sin embargo, dicho porcentaje es significativamente menor que los descritos anteriormente.…”

“…9 En un estudio reciente de HAI se mostró un mayor riesgo de trasplante temprano y recurrencia postrasplante en pacientes pediátricos afroamericanos. 10 La población colombiana desciende principalmente de tres grupos raciales (amerindios, europeos y africanos), lo que da lugar a una considerable diversidad genética. 11 Se han realizado estudios previos de HAI en México, Brasil y Argentina, que muestran resultados similares a reportes internacionales anteriores, evidenciando una mayor prevalencia de HAI tipo I y predominio en el sexo femenino.…”

“…Results. A total of 40 patients were included, 57.5% were female, with a median age of 10.5 years (IQR [4][5][6][7][8][9][10][11][12][13]. The 62.5% presented with acute hepatitis, 20% had liver failure, a 27.5% with cirrhosis at diagnosis; 75% presented compatible liver biopsy, and 82.5% were classified as type 1 autoimmune hepatitis.…”