Pediatric Celiac Disease, Cryptogenic Hypertransaminasemia, and Autoimmune Hepatitis

Vajro, Pietro; Paolella, Gaetana; Maggiore, Giuseppe; Giordano, Giuseppe

doi:10.1097/mpg.0b013e31828dc5c5

Cited by 62 publications

(51 citation statements)

References 57 publications

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“…On the other hand, pooled prevalence of CD in children with AIH and vice versa were reported as 6.3% (95% CI 3.87-11.7) and 1.4% (95% CI 0.84-2.15), respectively in a systematic review and meta-analysis of 9 studies with a total of 2,046 paediatric patients [56] . In a separate study, patients with AIH associated with CD were shown to achieve treatment-free sustained remission in a significant proportion in comparison to patients with AIH without CD (33% of patients with a mean period of treatment-free sustained remission of 89 months vs. 8% for those AIH patients without CD), suggesting a possible long-term adjuvant effect of a gluten-free diet [57].…”

Section: And Igadmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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For many patients with autoimmune hepatitis (AIH), the presence of extrahepatic features is well recognised both at the time of presentation and during long-term follow-up. Concomitant ‘autoimmune disorders' have been described in 20-50% of patients with AIH, both in adults and children. Indeed, the presence of these associated phenomena has been incorporated into both the original and revised International AIH group scoring systems as an aid to codifying the diagnosis. In acute index presentations, non-specific joint pains sometimes flitting in nature have been reported in 10-60% of patients, and while joint swelling is uncommon, rheumatoid arthritis and mixed connective tissue disease have been reported in 2-4% of patients with AIH. For a majority of patients, these joint symptoms resolve within days of the introduction of immunosuppressive therapy. Rarer features at index presentation include a maculopapular skin rash and unexplained fever, which are features that tend to resolve quickly with treatment. Interestingly, joint pain and stiffness are also well recognised in the context of steroid withdrawal and cessation in AIH. The occasional co-presentation of AIH with coeliac disease is clinically important (1-6%), since for some patients, there is a risk of immunosuppression malabsorption, thus delaying effective treatment. Similarly, the co-existence of selective IgA deficiency (IgAD) can occur in patients with coeliac disease or in isolation. Selective IgAD as a co-existing extraheaptic feature seems to be more common in paediatric patients with AIH. For these patients, they are at an increased risk of respiratory and sinus infections. Although, typically associated with primary sclerosing cholangitis, the presence of inflammatory bowel disease (IBD; both Crohn's disease and ulcerative colitis) has been described in 2-8% of patients with AIH. Interestingly, for patients with autoimmune sclerosing cholangitis, a distinct pattern of IBD has been recently described. Other conditions have been reported at a lower frequency, including Sjogren's syndrome 1-7%, systemic lupus erythematosus 1-3% and glomerulonephritis 1%. Rarer still and at a frequency of <1% include fibrosing alveolitis, haemolytic anaemia, uveitis, mononeuritis multiplex, polymyositis and multiple sclerosis. In contrast, the reported associations between AIH and thyroiditis 8-23%, diabetes 1-10% and psoriasis 3% are commonly seen and notable in clinical practice.

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Section: And Igadmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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“…Various liver diseases are more common in adult CD patients compared with the general population, including hepatitis, primary sclerosing cholangitis, nonalcoholic fatty liver disease, cirrhosis or fibrosis, and primary biliary cirrhosis [24]. Autoimmune hepatitis is associated with pediatric CD, and any patient with an elevation of transaminases of unknown cause should be tested for CD [25]. Finally, patients with Down syndrome (Trisomy 21), Turner syndrome, and Williams syndrome have been identified as high-risk groups for CD [26,27].…”

Section: Clinical Featuresmentioning

confidence: 99%

Cystic Fibrosis and Celiac Disease

Pohl¹,

Lowichik²,

Cantrell³

2015

Diet and Exercise in Cystic Fibrosis

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“…9 Un metaanálisis reciente revela que aproximadamente uno de cada tres niños celíacos presenta aumento de transaminasas al momento del diagnóstico. 10 Este aumento responde con rapidez a la exclusión del gluten 11 y su origen es probablemente una activación inespecífica del sistema inmune intrahepático frente a un intestino con permeabilidad anormal de antígenos (leaky gut). Con menos frecuencia, el cuadro corresponde a una hepatitis autoinmune definida (alrededor del 1% de los pacientes celíacos); estos últimos pacientes probablemente representen un subgrupo diferente, con predisposición genética particular, y deben recibir tratamiento inmunosupresor para controlar la hepatopatía.…”

Section: Pacienteunclassified

El niño con hipertransaminasemia: ¿cómo continuar?

2014

Arch Argent Pediat

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RESUMENEl aumento de las cifras de transaminasas séricas puede observarse en diferentes escenarios de la consulta médica cada vez con mayor frecuencia. Esto supone un desafío complejo para el pediatra dada la escasa información bibliográfica que existe sobre este tema. Desde el paciente completamente asintomático hasta el niño con signos de cronicidad, el espectro es muy variado y obliga a orientar rápida y eficientemente la investigación a fin de evitar costos y sufrimientos innecesarios para el paciente y su familia. El trabajo coordinado con el hepatólogo es recomendable en la mayoría de los casos. Palabras clave: transaminasas, hipertransaminasemia, niños. ABSTRACTThe raising of serum transaminases is seen with increasing frequency in different scenarios from daily practice. This poses a complex challenge to the pediatrician owing to the scarcity of bibliographic information available on this topic. From the completely asymptomatic patient to the child with signs of chronic liver disease, the full spectrum is highly varied, compelling to guide the investigation efficiently, in order to avoid unnecessary costs and suffering to the patient and their family. Coordinated work with the hepatologist is advisable in most instances.

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Pediatric Celiac Disease, Cryptogenic Hypertransaminasemia, and Autoimmune Hepatitis

Cited by 62 publications

References 57 publications

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Cystic Fibrosis and Celiac Disease

El niño con hipertransaminasemia: ¿cómo continuar?

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