Autoimmune Hepatitis and Primary Sclerosing Cholangitis in Children and Adolescents.

Rojas, Claudia; Bodicharla, Rajasekhar; Campuzano-Zuluaga, German; Hernandez, Lina; Rodríguez, Maria M.

doi:10.3109/15513815.2014.898721

Cited by 35 publications

(27 citation statements)

References 10 publications

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“…Although PSC was excluded in the present study, nonexclusion of autoimmune cholangitis could be a reason for the high PBC ratio. Coexistence of AIH and PSC is more common in children, young adults, and those with ulcerative colitis (30). The patients in the present study were older and did not present with ulcerative colitis (except one patient).…”

Section: Discussionmentioning

confidence: 47%

Our Autoimmune Hepatitis Patients: Single Center Experience

Özdoğan¹,

Yaraş²

2020

Istanbul Med J

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Amaç: Otoimmün hepatit (OIH) dolaşan otoantikorlar, otoimmünolojik tabloyla karakterize etiyolojisi bilinmeyen bir kronik hepatittir. Erken tanı ile çoğu kez tedavi edilebilen, tanısında birden çok değerin kullanıldığı, düzenli takip gerektiren bir hastalıktır. Ülkemizde OIH hastalarını tanısından tedaviye yanıtına kadar inceleyen çalışma sayısı yok denecek kadar azdır. Son 10 yılda OIH tanısı alan hastalarımızı retrospektif olarak değerlendirdik.Yöntemler: 2009 ile 2019 yılları arasında kliniğimizde tanı almış veya takip amacıyla başvurmuş OIH hastaları, retrospektif olarak hastane bilgi sisteminden geriye dönük olarak değerlendirildi. Ulaşabilen hastalar kliniğimize davet edilerek, elde olmayan bazı tetkikleri eklendi.Bulgular: 18-73 yaş arası (44,8±14,8 yıl) toplam 48 hasta (10 erkek ve 38 kadın) değerlendirildi. Dört hasta (%8,3) asemptomatik idi. Tedavi öncesi değerlendirmede hastaların %31'i siroz, %21'i akut hepatit, %39'u kronik hepatit idi. %81,2'si tip 1 OIH, %4,2'si tip 2 OIH iken %14,6'sında ise otoantikor negatif OIH saptandı. Hastaların %35,4'ünde (n=17) ise safra yollları hasarının eşlik ettiği overlap sendromu mevcut idi. Tip 1 OIH hastaların %68,8'inde antinükleer antikor, %41,7'sinde anti düz kas antikoru pozitifliği var iken her iki otoantikor pozitif olan hasta sayısı oranı %31,3 idi. %33,3'ünde karaciğer dışı hastalık mevcut idi. Tedaviye yanıt oranları tam yanıt %68,8, kısmi yanıt %18,7, yanıtsız hasta oranı ise %12,5 idi. Ortalama takip süresi 3,54±2,63 yıl olan hastaların halen 37'si tedaviye devam etmekte idi.Sonuç: Kadın erkek oranı, prezetasyonu, tip 1, tip 2 oranları, eşlik eden karaciğer dışı otoimmün hastalık oranı ve tedaviye yanıt oranları bakımından çalışmamızın literatüre benzer şekilde olduğu görüldü. Asemptomatik hasta oranının düşük olması, otoantikorların daha düşük yüzdede saptanması, overlap sendromunun yüksek olması açısından farklılık göstermekte idi.Anahtar Kelimeler: Otoimmün hepatit, otoantikor, primer bilier kolanjit, siroz Introduction: Autoimmune hepatitis (AIH) is a form of chronic hepatitis of unknown etiology characterized by autoimmunological properties and circulating autoantibodies. It can be treated after early diagnosis and often requires a great effort during diagnosis and regular follow-up. In Turkey, the number of studies examining AIH patients from the point of diagnosis to treatment is very low. We retrospectively evaluated patients diagnosed with AIH in the last 10 years. Methods: Between 2009 and 2019, AIH patients who were diagnosed in our hospital or applied for ongoing treatment were evaluated retrospectively from the hospital information system. Patients who responded were invited to the clinic and missing data were added. Results: A total of 48 patients (10 males and 38 females) aged 18-73 years (44.8±14.8 years) were evaluated. Four patients (8.3%) were asymptomatic. In the pre-treatment evaluation, cirrhosis occurred in 31% of patients, acute hepatitis in 21% of patients, and chronic hepatitis in 39% of patients. Type 1 AIH was observed in 81.2...

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Section: Discussionmentioning

confidence: 47%

Our Autoimmune Hepatitis Patients: Single Center Experience

Özdoğan¹,

Yaraş²

2020

Istanbul Med J

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“…1 Pediatric-onset PSC, however, is rare, with a reported incidence of 0.2/100,000 per year for PSC and of 0.1/100,000 per year for PSC/AIH, 3 hampering the studies on clinical course and prognosis. So far, only five long-term follow-up studies focusing on the pediatric population have been published, 3,[5][6][7][8] and only one was conducted in Europe. 5 The aim of this study was to assess the long-term clinical course and prognosis of a cohort of patients with pediatric onset of PSC in Finland who underwent endoscopic retrograde cholangiography (ERC) at diagnosis and during follow-up, in order to contribute to a better definition of the natural history and surveillance of this condition.…”

Section: Introductionmentioning

confidence: 99%

Clinical course and prognosis of pediatric-onset primary sclerosing cholangitis

Tenca

Färkkilä

Arola

et al. 2016

United European Gastroenterology Journal

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Background: The natural history of pediatric-onset primary sclerosing cholangitis (PSC) and overlap with autoimmune hepatitis (PSC/AIH) is poorly known. Objective: The aim of this study was to evaluate the clinical outcome of patients with pediatric-onset disease in a tertiary referral center. Methods: We traced 33 patients (median age at diagnosis 16 years), with PSC or PSC/AIH in cholangiography and liver histology diagnosed between December 1993 and 2011, at Helsinki University Hospital. Diagnostic procedures and long-term follow-up were reassessed until the end of December 2013. Results: PSC was confirmed in all 33 patients; 19 of them had an overlap with AIH. At diagnosis, three of 33 had cirrhosis. Inflammatory bowel disease (IBD) was associated in 76% of the patients, mostly ulcerative colitis (70%); treatment of IBD being a minor determinant of the clinical outcome of liver disease. In the last follow-up (median nine years), all patients were alive, and no malignancy occurred. Most patients (91%) were on ursodeoxycholic acid and 12 PSC/AIH patients on immunosuppression. Endoscopic retrograde cholangiography during follow-up showed a progression of intra-hepatic disease in 12 patients (36%). Four patients (12%) had undergone liver transplantation, and one was listed; no recurrence of the disease in the graft was seen. Conclusion:The clinical course and outcome of pediatric-onset PSC and PSC/AIH seem to be favourable in the majority of patients until early adulthood. In about one-third of patients, however, PSC is progressive, challenging the current treatment guidelines and warranting further studies on disease pathogenesis.

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“…We identified 10 observational studies from MEDLINE and EMBASE that described paediatric cohorts with SC . A recently published multicentre, international cohort study of children with SC (n = 781) reported portal hypertensive and biliary complications in 30% of cases in the first 5 years post‐diagnosis, and 12% of patients required a liver transplantation within 5 years after SC diagnosis .…”

Section: Discussionmentioning

confidence: 99%

“…We identified 10 observational studies from MEDLINE and EMBASE that described paediatric cohorts with SC. 6,7,[16][17][18][19][20][21][22] A recently published multicentre, international cohort study of children with SC (n = 781)…”

Section: Disease Progressionmentioning

confidence: 99%

Disease progression in paediatric‐ and adult‐onset sclerosing cholangitis: Results from two independent Dutch registries

Joosse

Haisma

Sterk

et al. 2019

Liver International

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Background & Aims Sclerosing cholangitis (SC) is a severe liver disease leading to destruction of bile ducts. It is believed to run a milder course in children than in adults. To test this assumption, we evaluated time‐to‐complication curves in two independent paediatric‐onset cohorts from the same geographical area. Methods Short‐term disease outcomes were evaluated with an online clinical registry that was filled with data on children with SC diagnosed between 2000 and 2017 and who were followed bi‐annually thereafter. Long‐term disease outcomes were evaluated in a paediatric‐onset subcohort derived from a previously published population‐based study from the Netherlands. Time‐to‐complication in the first cohort was defined as the time from diagnosis until portal hypertension, biliary obstructions and infections, development of malignancy, or liver transplantation, whichever came first. In the second cohort time‐to‐complication was defined as the time until liver transplantation or PSC‐related death. Results Median age at diagnosis in the first cohort (n = 86) was 12.3 years. In the first 5 years post‐diagnosis 23% of patients developed complications. The patients in the population‐based study (n = 683) were stratified into those diagnosed before the age of 18 years (‘paediatric‐onset’ subcohort, n = 43) and those diagnosed after the age of 18 years (‘adult‐onset’ subcohort, n = 640). Median age at diagnosis was 14.6 and 40.2 years, respectively. Median time‐to‐complication in the paediatric‐onset and adult‐onset subcohorts was not statistically different. Conclusion Paediatric and adult‐onset SC run a similar long‐term disease course. Paediatricians who treat children with SC should monitor them closely to recognize early complications and control long‐term sequelae.

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Autoimmune Hepatitis and Primary Sclerosing Cholangitis in Children and Adolescents.

Cited by 35 publications

References 10 publications

Our Autoimmune Hepatitis Patients: Single Center Experience

Our Autoimmune Hepatitis Patients: Single Center Experience

Clinical course and prognosis of pediatric-onset primary sclerosing cholangitis

Disease progression in paediatric‐ and adult‐onset sclerosing cholangitis: Results from two independent Dutch registries

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