Autoimmune acute liver failure and seronegative autoimmune liver disease in children: Are they different from classical disease?. Issue 12 (December 2017)

• Record Type: Journal Article
• Authors: Jain, Vikas; Srivastava, Anshu; Yachha, Surender K.; Kumari, Niraj; Kathuria, Rachana; Sarma, Moinak S.; Poddar, Ujjal; Krishnani, Narendra
• Is Part Of: European journal of gastroenterology & hepatology. Volume 29:Issue 12(2017:Dec.)
• Publication Date: 2017-12
• Subjects: acute liver failure -- autoimmune acute liver failure -- autoimmune liver disease -- children -- outcome -- seronegative autoimmune liver disease; Digestive organs -- Diseases -- Periodicals; Liver -- Diseases -- Periodicals; Digestive organs -- Diseases; Liver -- Diseases; Periodicals; 616.33
• DOI: 10.1097/MEG.0000000000000975
• Languages: English
• ISSNs: 0954-691X

Abstract

Abstract : Objectives: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). Materials and methods: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available. Results: Seventy children [AI-ALF=15 and AI-non-ALF=55 (SN-AILD=11, SP-AILD=44)] were evaluated. Age at presentation [7 (1.2–16) vs. 9 (2–17) years] percentage of female patients (67 vs. 62%), and AILD type (type II, 53 vs. 31%) were similar in AI-ALF and AI-non-ALF patients], respectively. 8/15 AI-ALF cases were treated with steroids (improved-4, liver transplant-1, and death-3) and 7/15 died before initiation of therapy. Hepatic encephalopathy (100 vs. 16.3%; P <0.001), massive hepatic necrosis (60 vs. 0%; P <0.001), and higher pediatric end-stage liver disease [ n =53, 29.9 (13.1–56.9) vs. 9.8 (−10–28.7) P <0.001], model for end-stage liver disease [ n =17, 38.5 (24–46) vs. 18 (6–24); P =0.005], and Child–Turcotte–Pugh [ n =70, 13 (8–13) vs. 9 (5–13); P <0.001] scores were features of AI-ALF. Poorer response to immunosuppression (4/8 vs. 48/55; P= 0.02) and higher mortality (11/15 vs. 4/55; P =0.0001) were seen in AI-ALF than in AI-non-ALF patients. Clinicolaboratory profile, therapeutic response, and outcome were similar in SN-AILD and SP-AILD. Conclusion: AI-ALF is characterized by poorer liver function, lower response to immunosuppression, and higher mortality compared with SP or SN AI-non-ALF, which are similar.