A promising medium‐term follow‐up of pediatric sclerosing cholangitis: Mild phenotype or early diagnosis?

Abstract

In our early diagnosed cohort, the 9-year survival with native liver was better than that reported in other studies. Approximately 15% of patients developed liver-related disease complications, less than previously reported. The long-term course of SC was negatively influenced by the presence of autoimmune features, but not by concomitant IBD.

Treatment of Overlap Syndromes in Autoimmune Liver Disease: A Systematic Review and Meta-Analysis

“…All publications meeting inclusion criteria for the systematic review were observational cohort studies. The systematic review comprised 20 studies pertaining to AIH-PBC [6][7][8]10,11,13,25,57,[64][65][66][67][68][69][70][71][72][73][74][75], two pertaining to AIH-PSC [35,41], two pertaining to AIC [23,24], three pertaining to ASC [33,43,44], and one pertaining to both AIH-PBC and AIC [22]. Of the 21 studies that included patients with AIH-PBC, 17 compared UDCA monotherapy to UDCA + [corticosteroids and/or antimetabolites (AZA or MMF)], i.e., "combination therapy" [6][7][8]10,11,25,57,[65][66][67][68][69][70][71][73][74][75].…”

“…Of the three studies pertaining to AIC, two compared UDCA to corticosteroids ± AZA [22,24], while the third compared combination therapy to a complex personalized regimen consisting of UDCA, prednisolone, AZA, MMF, budesonide, rifampicin, and several other agents [23]. All studies of ASC compared UDCA to combination therapy [33,43,44]. Among the 21 AIH-PBC studies in our systematic review, four were ineligible for meta-analysis: One because it was the only study comparing UDCA to placebo [13], one because of an ambiguous overlap between treatment groups [22], and two because no clinical outcomes occurred during follow-up [69,72].…”

“…One of these studies was conducted in the Czech Republic and followed 11 patients (six female, five male) for a median of 144 months [44]. The other, conducted in Italy, followed 14 patients for a median of 79 months and did not report their gender distribution [43]. The median ages of patients in the former and latter studies were 14 and 9.9 years, respectively.…”

“…The median ages of patients in the former and latter studies were 14 and 9.9 years, respectively. Both studies used a form of the IAIHG criteria to define the AIH component of ASC and for the PSC component, one study required biochemical cholestasis with characteristic findings on either histology or cholangiography [43], while the other study required just cholangiographic findings [44]. Both studies reported biochemical improvement but failed to define it in any detail.…”

“…UDCA alone [33,[38][39][40], or corticosteroids with or without AZA [3,33,35,[40][41][42], have also been used, albeit less often. Similarly, a combination of UDCA and corticosteroids (with or without AZA) is the most common treatment regimen for ASC [33,36,43,44], while UDCA monotherapy has been used in some cases [33,43,44]. Limited experience using MMF as second-line therapy for patients unresponsive to, or intolerant of, corticosteroids or AZA suggests a greater efficacy for AIH-PSC [16] than for ASC [45].…”