Piel y tubo digestivo: dermatitis herpetiforme y enfermedad celíaca / Small bowel and skin: celiac disease and dermatitis herpetiformis

La dermatitis herpetiforme, también denominada Enfermedad de Dühring-Brocq, es una dermatosis autoinmune crónica que evoluciona por brotes, caracterizada por la presencia de ampollas pequeñas que tienden a agruparse, en codos, rodillas y glúteos, con disposición simétrica, intensamente pruriginosas. Es considerada una manifestación cutánea de la enfermedad celíaca. Afecta a adultos jóvenes (20 a 50 años). El estudio histopatológico evidencia ampollas subepidérmicas. La inmunofluorescencia directa es característica: depósitos granulares de IgA en las puntas de las papilas dérmicas. Aún ante falta de sintomatología digestiva debe investigarse enfermedad celíaca en todos los pacientes. La dieta libre de gluten es la clave del tratamiento. En aquellos pacientes con intenso prurito o con una dermatosis muy extensa se puede utilizar dapsona vía oral, que alivia rápidamente las manifestaciones cutáneas, pero no modifica el curso de la enfermedad digestiva. Se presenta un paciente en quien a partir de las lesiones cutáneas se realizó diagnóstico de dermatitis herpetiforme primero y de enfermedad celíaca luego

Dermatitis herpetiformis, also known as Dühring-Brocq disease, is a chronic autoimmune dermatosis that evolves in outbreaks. It is characterized by the presence of small blisters that tend to cluster on the elbows, knees, and buttocks, with a symmetrical distribution and intense itching. It is considered a cutaneous manifestation of celiac disease. It affects young adults (20 to 50 years old). Histopathological examination reveals subepidermal blisters. Direct immunofluorescence is characteristic, showing granular deposits of IgA at the tips of the dermal papillae. Even in the absence of digestive symptoms, celiac disease should be investigated in all patients. A gluten-free diet is the key to treatment. In patients with intense itching or extensive dermatosis, oral dapsone can be used to quickly relieve cutaneous manifestations, but it does not alter the course of the digestive disease. We present a patient in whom the diagnosis of dermatitis herpetiformis was made initially, followed by a diagnosis of celiac disease based on the skin lesions

Expression of M30 and M65 in celiac disease. Analytical cross-sectional study

ABSTRACT BACKGROUND: The role of villous atrophy in apoptosis, a distinctive feature of celiac disease, is a matter of controversy. The aim of this study was to determine the apoptosis rate through immunohistochemical staining for M30 and M65 in celiac disease cases. DESIGN AND SETTING: Analytical cross-sectional study in a tertiary-level center. METHODS: Duodenal biopsies from 28 treatment-naive patients with celiac disease, 16 patients with potential celiac disease, 10 patients with a gluten-free diet and 8 controls were subjected to immunohistochemical staining for the end-apoptotic marker M30 and the total cell death marker M65. H-scores were compared. Several laboratory parameters were recorded concomitantly, and at the one-year follow-up for celiac disease and potential celiac disease patients. RESULTS: There was a significant difference in H-score for M30 expression between the celiac disease, potential celiac disease and gluten-free diet groups (P = 0.009). There was no significant difference in H-score for M65 expression. There was a positive correlation between the H-score for M30 expression and the anti-tissue transglutaminase immunoglobulin A (anti-tTgIgA) and anti-tissue transglutaminase immunoglobulin G (anti-tTgIgG) levels (R = 0.285, P = 0.036; and R = 0.307, P = 0.024, respectively); and between the H-score for M65 expression and the anti-tTgIgA and anti-tTgIgG levels (R = 0.265, P = 0.053; and R=0.314, P = 0.021, respectively). There was no difference between celiac disease and potential celiac disease patients regarding the laboratory parameters selected. CONCLUSION: The rates of apoptosis and nutritional deficiencies in patients with potential celiac disease were similar to those in patients with celiac disease.

Celiac Crisis: an unusual presentation of gluten-sensitive enteropathy

Celiac disease (CD)­also known as gluten-sensitive enteropathy­is a chronic, genetically predisposing and autoimmune entity with a wide range of clinical manifestations triggered by gluten ingestion, which affects 1% of the general population. Currently, up to 60% of the diagnosis of CD is in adults due to the atypical course of the disease. The severe acute onset of CD­also called celiac crisis­is very uncommon and is still not well documented in adults. We report the case of a 58-year-old man who presented a 45-day history of subtle-onset diarrhea followed by malabsorption syndrome with progressive weight loss, anasarca, and electrolyte disturbances. The diagnostic work-up included an upper digestive endoscopy, which showed scalloping of the duodenal mucosa with pathological features confirmed on biopsies. Specific antibodies were positive, and a satisfactory clinical response was obtained once a gluten-free diet was started. Celiac crisis is a rare initial presentation of CD characterized by severe diarrhea, dehydration, weight loss, hypoproteinemia, and metabolic and electrolyte disturbances. Although rare, it should be considered in patients with apparently unexplained chronic diarrhea.

Potencial associação da doença celíaca com anemia ferropriva refratária em crianças e adolescentes / Association of potential celiac disease and refractory iron deficiency anemia in children and adolescents

ABSTRACT BACKGROUND: Celiac disease is an enteropathy caused by dietary gluten. The combination of serologic, genetic and histologic data has led to description of other categories of this disease. OBJECTIVE: There are a number of patients with iron deficiency anemia (IDA) that do not respond to iron treatment and may be repeated for many times, Therefore, we aimed to investigate celiac disease in this group. METHODS: In this cross sectional transverse prospective study from August 2011 to February 2013, in a Pediatric care clinic affiliated to Shiraz University of Medical Sciences, 184 children including 92 IDA patients who responded to treatment using iron supplement, 45 non-responding iron deficient patients, and 47 healthy individuals, with the maximum age of 18 years, with written consent from their parents, participated in serologic screening (with Anti-TTG antibody and anti-Endomysial antibody) for celiac disease. Patients with at least one positive serology test underwent multiple mucosal biopsy from bulb and duodenum. RESULTS: Among 184 participants, 19 (10.3%) subjects had positive serologic test for celiac disease, including 13 (28.9%) patients in the group with refractory IDA, 5 (5.4%) patients in the group with treated IDA, and 1 patient in the healthy group. The frequency of positive serologic test in the group with IDA resistant to treatment was prominently higher than the other two groups (P<0.001). Among the patients with positive serologic celiac test who underwent endoscopy and biopsy, no histologic evidence of celiac disease was seen. They were diagnosed as potential celiac disease. CONCLUSION: Frequency of potential celiac disease in patients with refractory IDA was higher than control the subjects. Therefore, we recommend serologic screening for early detection and minimizing the complications of celiac disease and repeated iron therapy for this group.

RESUMO CONTEXTO: A doença celíaca é uma enteropatia causada pelo glúten na dieta. A combinação de dados sorológicos, genéticos e histológicos proporcionou a descrição de outras categorias desta doença. OBJETIVO: Há pacientes com anemia por deficiência de ferro que não respondem ao tratamento com ferro mesmo que repetido por muitas vezes. O objetivo deste trabalho foi investigar a presença de doença celíaca nestes indivíduos. MÉTODOS: Realizado estudo prospectivo com cruzamento secional transversal, de agosto de 2011 a fevereiro de 2013, em uma clínica de cuidados pediátricos afiliados a Shiraz University Medical Sciences, com 184 crianças incluindo 92 pacientes com anemia por deficiência de ferro que responderam ao tratamento com ferro suplementar, 45 não respondedores e 47 indivíduos sadios, com idade máxima de 18 anos, todos com consentimento informado dos pais. Todos participaram da triagem sorológica (com anticorpos anti-TTG e anticorpo antiendomísio) para doença celíaca. Pacientes com pelo menos um teste de sorologia positiva foram submetidos a biópsia da mucosa múltipla do bulbo e duodeno. RESULTADOS: Entre os 184 participantes, 19 (10,3%) tinham teste sorológico positivo para doença celíaca, incluindo 13 (28,9%) pacientes no grupo com a anemia por deficiência de ferro refratária, 5 (5,4%) pacientes no grupo com anemia por deficiência de ferro tratados e respondedores e 1 paciente do grupo saudável. A frequência de teste sorológico positivo no grupo com anemia por deficiência de ferro resistente ao tratamento foi destacadamente maior do que os outros dois grupos (P<0,001). Entre os pacientes com teste sorológico positivo para doença celíaca submetidos a endoscopia e biópsia, não foi vista nenhuma evidência histológica de doença celíaca. Foram diagnosticados como potencial doença celíaca. CONCLUSÃO: Potencial frequência de doença celíaca em pacientes com anemia por deficiência de ferro refratária foi maior do que nos controles. Portanto, recomendamos testes sorológicos de triagem para a detecção precoce, minimizando as complicações da terapia de ferro repetidas para este grupo.

Magnetic resonance enterography in pediatric celiac disease / A enterografia por ressonância magnética na doença celíaca pediátrica

Abstract Objective: To assess if magnetic resonance enterography is capable of showing evidence/extent of disease in pediatric patients with biopsy-proven celiac disease by comparing with a control group, and to correlate the magnetic resonance enterography findings with anti-endomysial antibody level, which is an indicator of gluten-free dietary compliance. Methods: Thirty-one pediatric patients (mean age 11.7 ± 3.1 years) with biopsy-proven celiac disease and 40 pediatric patients as a control group were recruited in the study. The magnetic resonance enterography images of both patients with celiac disease and those of the control group were evaluated by two pediatric radiologists in a blinded manner for the mucosal pattern, presence of wall thickening, luminal distention of the small bowel, and extra-intestinal findings. Patient charts were reviewed to note clinical features and laboratory findings. The histopathologic review of the duodenal biopsies was re-conducted. Results: The mean duration of the disease was 5.6 ± 1.8 years (range: 3-7.2 years). In 24 (77%) of the patients, anti-endomysial antibody levels were elevated (mean 119.2 ± 66.6 RU/mL). Magnetic resonance enterography revealed normal fold pattern in all the patients. Ten (32%) patients had enlarged mesenteric lymph nodes. Conclusion: Although a majority of the patients had elevated anti-endomysial antibody levels indicating poor dietary compliance, magnetic resonance enterography did not show any mucosal abnormality associated with the inability of magnetic resonance enterography to detect mild/early changes of celiac disease in children. Therefore, it may not be useful for the follow-up of pediatric celiac disease.

Resumo Objetivo: Avaliar se a enterografia por ressonância magnética (ERM) consegue comprovar/mostrar a extensão da doença em pacientes pediátricos com doença celíaca (DC) comprovada por biópsia, comparar com um grupo de controle e correlacionar os achados da ERM com o nível de anticorpo antiendomísio (EMA) indicador de dieta sem glúten. Métodos: Foram recrutados 31 pacientes pediátricos (idade média entre 11,7 ± 3,1 anos) com DC comprovada por biópsia e 40 pacientes pediátricos em um grupo de controle. As imagens da ERM dos pacientes com DC e no grupo de controle foram avaliadas por dois radiologistas pediátricos às cegas para o padrão da mucosa, presença de espessamento da parede, dilatação luminal do intestino delgado e achados extraintestinais. Os prontuários dos pacientes foram revisados para anotação de características clínicas e achados laboratoriais. A avaliação histopatológica das biópsias duodenais foi feita novamente. Resultados: A duração média da doença foi 5,6 ± 1,8 anos (faixa de 3-7,2 anos). Em 24 (77%) dos pacientes, os níveis EMA estavam elevados (média 119,2 ± 66,6 RU/mL). A ERM revelou um padrão de pregas normal em todos os pacientes; 10 (32%) dos pacientes apresentaram gânglios linfáticos mesentéricos aumentados. Conclusão: Apesar de a maioria dos pacientes ter níveis elevados de EMA, o que indica uma dieta pobre, a ERM não mostrou anomalia na mucosa associada à incapacidade de a ERM detectar alterações leves/precoces de DC nas crianças. Portanto, ela pode não ser útil no acompanhamento da DC pediátrica.

Estresse oxidativo e parâmetros inflamatórios em pacientes com doença celíaca / Oxidative stress and inflammatory parameters inpatients presenting celiac disease

Objetivos: Determinar a presença de estresse oxidativo e inflamação no intestino de pacientes com doença celíaca. Método: Foi realizado estudo transversal que incluiu pacientes submetidos à endoscopia gastrointestinal. A população do estudo consistiu em 24 casos e 26 controles. Foram medidos os níveis duodenais de proteínas carboniladas, espécies reativas ao ácido tiobarbitúrico, bem como catalase (CAT), superóxido dismutase (SOD). Também foram determinados os níveis intestinais de interleucina (IL) 6, 10 e 8. A classificação de Marsh foi registrada e utilizada como parâmetro de gravidade da doença. Resultados: Tanto a IL-6 como a IL-10, mas não a IL8, aumentaram nos pacientes com doença celíaca quando comparados com indivíduos saudáveis. Os parâmetros de dano oxidativo foram aumentados,enquanto que as defesas antioxidantes foram reduzidas em nossa amostra. Os níveis de IL6 ea atividade do SOD foram relacionados com a pontuação de Marsh. Conclusões: Diferentes marcadores de inflamação e estresse oxidativo estão alterados no intestino de pacientes com doença celíaca, e alguns deles estão relacionados à gravidade da doença.(AU)

Objectives: Determine the presence of oxidative stress and inflammation in the gut of patients with celiac disease. Methods: Transversal study that included patients undergoing upper gastrointestinal endoscopy was performed. The study population consisted 24 cases and 26 controls. The duodenal levels of protein carbonyls, thiobarbituric acid reactive species, as well as catalase, superoxide dismutase (SOD) activities were measured. Gut levels of interleukin (IL) 6, 10 and 8 were also determined.The Marsh classification was recorded and used as a parameter of disease severity. Results: Both IL-6 and IL-10, but not IL8, were increased in celiac disease patients when compared to healthy individuals. Oxidative damage parameters were increased while antioxidant defenses were decreased in our sample. Both IL6 levels and SOD activity were related to Marsh score. Conclusions: Different markers of inflammation and oxidative stress are altered in the gut of celiac disease patients, and some of them are related to disease severity.(AU)

Enfermedad celíaca, ¿qué hay de nuevo? / Celiac disease, what's new?

La enfermedad celíaca (EC) es una enteropatía autoinmune sensible al gluten, con base genética. La prevalencia de la enfermedad es elevada y conforme aumentan los conocimientos de la patología, aumenta la prevalencia. Es bien conocida la asociación entra la EC y otras enfermedades de origen autoinmunitario. Los síntomas clásicos aparecen secundariamente a la malabsorción intestinal y desaparecen al retirar el gluten de la dieta, si bien el espectro clínico es muy amplio, con síntomas extradigestivos muy variados, lo que da una idea de que es una enfermedad multisistémica. El diagnóstico se realiza mediante biopsia intestinal y los marcadores serológicos. Su único tratamiento consiste en una dieta estricta sin gluten mantenida indefinidamente, ya que sin respetarse la dieta, podría evolucionar una complicación potencial más grave, que es la malignización

Celiac disease is an autoimmune gluten-sensitive enteropathy, with a genetic basis. The prevalence of the disease is high and increases as the knowledge of the disease. It is well known the association enters the EC and other diseases of autoimmune origin. The classic symptoms occur secondary to intestinal malabsorption and disappear when removing gluten from the diet, although the clinical spectrum is very broad, with varied symptoms extradigestive, which gives an idea that is a multisystem disease. The diagnosis is made by intestinal biopsy and serological markers. The only treatment is a strict gluten-free diet, if there is no adherence to the diet could evolve a more serious potential complication, which is malignant

HELICOBACTER PYLORI PREVALENCE IN PATIENTS WITH CELIAC DISEASE: results from a cross-sectional study / Prevalência do Helicobacter pylori em pacientes com doença celíaca: resultados de um estudo transversal

Background Some previously published studies have suggested an inverse relationship between celiac disease and Helicobacter pylori, raising the possibility of the protective role Helicobacter pylori could have against celiac disease development. Nevertheless, this association is inconclusive. Objectives To determine the prevalence of Helicobacter pylori infection in celiac subjects. Methods Between January 2013 and June 2014, patients over 18 years old undergoing upper endoscopy who required both gastric and duodenal biopsies were included for analysis. Enrolled subjects were divided in two groups: those with a diagnosis of celiac disease and those without a celiac disease diagnosis. Helicobacter pylori infection prevalence was compared between groups. Among celiac patients, endoscopic markers of villous atrophy as well as histological damage severity were compared between those with and without Helicobacter pylori infection. Results Overall, 312 patients were enrolled. Seventy two of them had a diagnosis of celiac disease. Helicobacter pylori infection prevalence among celiac disease patients was 12.5%, compared to 30% in non-celiac patients [OR=0.33 (0.15-0.71)]. There was not a significant difference in terms of the severity of villous atrophy in patients with Helicobacter pylori infection compared to those without it. There was a slight increase in the prevalence of endoscopic markers in those Helicobacter pylori-negative celiac subjects. Conclusion Helicobacter pylori infection seems to be less frequent in celiac patients; among those celiac subjects with concomitant Helicobacter pylori infection, histological damage degree and presence of endoscopic markers suggesting villous atrophy seem to be similar to those without Helicobacter pylori infection. .

Contexto Alguns estudos publicados anteriormente sugerem uma relação inversa entre a doença celíaca e Helicobacter pylori, levantando a possibilidade do papel protetor que o Helicobacter pylori poderia ter contra o desenvolvimento de doença celíaca. No entanto, esta associação é inconclusiva. Objetivos Determinar a prevalência da infecção por Helicobacter pylori em indivíduos celíacos. Métodos Entre janeiro de 2013 e de 2014 junho, foram incluídos para análise pacientes com mais de 18 anos de idade submetidos a endoscopia para necessárias biópsias gástricas e duodenais. Os pacientes foram divididos em dois grupos: aqueles com diagnóstico de doença celíaca e aqueles sem um diagnóstico de doença celíaca. A prevalência da infecção por Helicobacter pylori foi comparada entre os grupos. Entre os pacientes celíacos, os marcadores endoscópicos de atrofia das vilosidades, bem como a gravidade do dano histológico foram comparados entre aqueles com e sem infecção pelo Helicobacter pylori. Resultados De um total de 312 pacientes, 72 deles tiveram diagnóstico da doença celíaca. A prevalência de infecção pelo Helicobacter pylori entre pacientes com doença celíaca foi de 12,5%, em comparação com 30% em pacientes não-celíacos [OR=0,33 (0,15-0,71)]. Não houve diferença significativa em termos da gravidade da atrofia das vilosidades em pacientes com infecção pelo Helicobacter pylori em comparação com aqueles sem ele. Houve um ligeiro aumento na prevalência de marcadores endoscópicos nos indivíduos celíacos com Helicobacter pylori-negativo. Conclusão A infecção pelo Helicobacter pylori parece ser menos frequente em pacientes celíacos; entre esses indivíduos celíacos com concomitante infecção por Helicobacter pylori, o grau de dano histológico e a presença de marcadores endoscópicos sugerindo atrofia vilosa, parecem ser semelhantes com os sem infecção. .

Correlation of nndoscopic and histological featres in adults with suspected ceeliac disease in a referral center of minas gerais, Brazil / Correlação entre os aspectos endoscópicos e histológicos de pacientes adultos com suspeita de doença celíaca em um centro de referência de Minas Gerais, Brasil

Context Clinical presentation of celiac disease is extremely variable and the diagnosis relies on serologic tests, mucosal intestinal biopsy and clinic and serologic response to a gluten-free diet. Objectives To correlate the endoscopic and histological aspects of adult patients with suspicion of celiac disease and to evaluate the interobserver histological agreement. Methods Endoscopic aspects of 80 adult patients were evaluated and correlated with the histological features according the Marsh-Oberhuber classification system. The interobserver histological agreement was based on kappa values. Results The symptoms of the patients varied largely, with prominence for chronic diarrhea, present in 48 (60%) patients. The endoscopic aspects related with the duodenal villous atrophy had been observed in 32 (40%) patients. There were confirmed 46 cases of celiac disease, with prevalence of 57.5%. The sensitivity, specificity, positive predictive value and negative predictive value of the endoscopic markers for celiac disease diagnosis were of 60.9%, 88.2%, 87.5% and 62.5%. There was moderate interobserver histological agreement (kappa = 0.46). Conclusions The endoscopic markers of villous atrophy, although not diagnostic, had assisted in the suspicion and indication of the duodenal biopsies for diagnosis proposal. Histology is sometimes contradictory and new biopsies or opinion of another professional can provide greater diagnostic agreement. .

Contexto A apresentação clínica da doença celíaca é extremamente variável e o diagnóstico se baseia em testes sorológicos, histologia intestinal e respostas clínica e sorológica à dieta sem glúten. Objetivos Correlacionar os aspectos endoscópicos e histológicos de pacientes adultos com suspeita de doença celíaca e avaliar a concordância histológica interobservadores. Métodos Os aspectos endoscópicos de 80 pacientes adultos foram avaliados e correlacionados com os achados histológicos de acordo com a classificação de Marsh-Oberhuber. A concordância histológica foi baseada nos valores kappa. Resultados A sintomatologia clínica foi muito variável com destaque para a diarréia crônica, presente em 48 (60%) pacientes. Os aspectos endoscópicos relacionados à atrofia vilositária duodenal foram observados em 32 (40%) pacientes. Foram confirmados 46 casos de doença celíaca, prevalência de 57.5%. A sensibilidade, a especificidade, o valor preditivo positivo e o valor preditivo negativo dos aspectos endoscópicos para o diagnóstico da doença celíaca foram, respectivamente, 60,9%, 88,2%, 87,5% e 62,5%. A concordância histológica interobservadores foi moderada (kappa = 0,46). Conclusões Os aspectos endoscópicos de atrofia vilositária contribuíram para a suspeita e a indicação das biópsias duodenais com objetivo diagnóstico. A histologia pode ser contraditória e novas biópsias ou a opinião de outro profissional podem propiciar maior concordância diagnóstica. .

Prevalence and clinical features of celiac disease in patients with autoimmune thyroiditis: cross-sectional study / Prevalência e características clínicas da doença celíaca em pacientes com tireoidite autoimune: estudo transversal

CONTEXT AND OBJECTIVE: Celiac disease is an autoimmune disorder with an average prevalence of 1% in Europe and the United States. Because of strong European ancestry in southern Brazil, this study aimed to evaluate the seroprevalence of celiac disease among autoimmune thyroiditis patients. DESIGN AND SETTING: Cross-sectional study in a public university hospital. METHODS: This cross-sectional prevalence study included autoimmune thyroiditis patients who were tested for anti-endomysial and anti-transglutaminase antibodies between August 2010 and July 2011. RESULTS: Fifty-three patients with autoimmune thyroiditis were included; 92.5% were women, with mean age of 49.0 ± 13.5 years. Five patients (9.3%) were serologically positive for celiac disease: three of them (5.6%) were reactive for anti-endomysial antibodies and two (3.7%) for anti-transglutaminase. None of them exhibited anemia and one presented diarrhea. Endoscopy was performed on two patients: one with normal histology and the other with lymphocytic infiltrate and villous atrophy. CONCLUSION: The prevalence of celiac disease among patients with autoimmune thyroid disease was 9.3%; one patient complained of diarrhea and none presented anemia. Among at-risk populations, like autoimmune thyroiditis patients, the presence of diarrhea or anemia should not be used as a criterion for indicating celiac disease investigation. This must be done for all autoimmune thyroiditis patients because of its high prevalence. .

CONTEXTO E OBJETIVO: A doença celíaca é uma doença autoimune, com prevalência média de 1% na Europa e nos Estados Unidos. Em função da forte ascendência europeia no sul do Brasil, este estudo objetiva relatar a soroprevalência de doença celíaca em indivíduos com tireoidite autoimune. TIPO DE ESTUDO E LOCAL: Estudo transversal em um hospital público universitário. MÉTODOS: Este estudo transversal de prevalência incluiu pacientes com tireoidite autoimune que foram submetidos a testes de anticorpos antiendomísio e antitransglutaminase entre agosto de 2010 e julho de 2011. RESULTADOS: Foram incluídos 53 pacientes com tireoidite autoimune, 92,5% mulheres, com idade média de 49,0 ± 13,5 anos. Cinco (9,3%) pacientes apresentaram sorologia positiva para doença celíaca, sendo três (5,6%) com anticorpo antiendomísio positivo e dois (3,7%) com antitransglutaminase positivo. Nenhum paciente apresentou anemia e um apresentou diarreia. Apenas dois pacientes realizaram endoscopia: um com histologia normal e outro apresentou infiltrado linfocitário e atrofia vilositária. CONCLUSÕES: A prevalência de doença celíaca entre pacientes com doença autoimune da tireoide foi de 9,3%; um paciente queixou-se de diarreia e ninguém apresentou anemia. Em populações de risco, como é o caso de pacientes com tireoidite autoimune, a presença de diarreia ou anemia não devem ser utilizados como critério para indicar investigação de doença celíaca, que deve ser feita em todos os indivíduos com tireoidite autoimune devido a sua alta prevalência. .

Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment

Researches on DH have shown that it is not just a bullous skin disease, but a cutaneous-intestinal disorder caused by hypersensitivity to gluten. Exposure to gluten is the starting point of an inflammatory cascade capable of forming autoantibodies that are brought to the skin, where they are deposited, culminating in the formation of skin lesions. These lesions are vesico-bullous, pruritic, and localized especially on elbows, knees and buttocks, although atypical presentations can occur. Immunofluorescence of perilesional area is considered the gold standard for diagnosis, but serological tests help in cases where it is negative. Patients who follow glutenfree diets have better control of symptoms on the skin and intestine, as well as lower risks of progression to lymphoma. Dapsone remains the main drug for treatment, but it requires monitoring of possible side effects, some potentially lethal.

Positividad anticuerpo antitransglutaminasa tisular humana en adultos histológicamente compatibles con enfermedad celíaca / Antitransglutaminase antibody positivity in adults histologically compatible with celiac disease

Objetivo: Determinar la frecuencia de positividad de anticuerpo antitransglutaminasa tisular humana en pacientes adultos histológicamente compatibles con enfermedad celiaca. Material y método: El presente trabajo corresponde a un diseño analítico, transversal con régimen de investigación orientado, en el que se realizó una revisión de historias clínicas de pacientes del Servicio de Gastroenterología de la Clínica San Pablo, Lima, Perú, con resultados de biopsias compatible con enfermedad celíaca (EC) desde el año 1994 al 2011 y que además contó con valor de anticuerpo antitransglutaminasa tisular humana (AATG), calculándose la frecuencia de positividad del mismo. Resultados: Según criterios señalados por el presente estudio se trabajó sobre un total de 44 historias clínicas conformadas por 18 (40,9%) correspondientes a hombres y 26 (59,1%) a mujeres, con una edad media al momento del diagnóstico de 51 ± 16,23 años en general de las cuales 12 (27,27%) obtuvieron resultado positivo para AATG, 2 (4,54%) valores indeterminados y 30 (68,18%) resultados negativos con resultados histológico compatible con EC. Conclusión: No es frecuente la positividad del anticuerpo antitransglutaminasa tisular humana en pacientes adultos histológicamente compatibles con enfermedad celiaca.

Objective: To determine the frequency of positive results for antitransglutaminase antibody in adult patients histologically compatible with celiac disease. Material and methods: Cross sectional, descriptive study with research-oriented regime, which included medical records of Gastroenterology Service of San Pablo Clinic, Lima, Peru from 1994 to 2011 with biopsies histologically compatible with CD and antitransglutaminase antibodies to find the frequency of positive serology. Results: According to criteria established by the present study, we worked on a total of 44 medical records which included18 (40.9%) men and 26 (59.1%) women, mean age at diagnosis of 51 ± 16.23 years at the total. From all, 12 (27.27%) were positive for AATG, 2 (4.54%) values were indeterminate and 30 (68.18%) were negative with histological findings compatible with CD. Conclusion: It is not frequent positive results for antitransglutaminase antibody in adult patients histologically compatible with celiac disease.

Doença Celíaca no município de São Paulo: a disponibilidade de um mercado específico / Celiac Disease in São Paulo: the availability of a specific market

Celiac Disease is a genetic enteropathy characterized by permanent intolerance to gluten. Specific and restrictive, gluten-free diet may present a growing demand for products such as breads, pasta, sausages and beverages with special formulations. Whereas no similar studies were found in the literature, the aims of this investigation were to identify the repertoire of gluten-free products available in the market of São Paulo and to estimate the cost difference between a diet consisting of conven¬tional products and other with gluten-free products. Characterized as an exploratory qualitative and quantitative study, it was based on market research. Visits were made to specialty stores in areas of higher population density and/or that concentrate the largest number of establishments in São Paulo. Interviews were carried out with members of associations, universities, hospitals or research and treatment centers of celiac disease. The interview was semi-structured with open questions approaching aspects of the market of gluten-free products and celiac disease. Data analysis was obtained by calculating the percentage difference between the products found and formulated diets. The gluten-free diet represents a significant impact on the cost of food, especially when it contains products such as pasta and flour. In São Paulo, a diet with gluten-free products can be approximately 44% more expensive than a diet with conventional products.

La enfermedad celíaca es una enteropatía de origen genético caracterizada por la intolerancia permanente al gluten. Específica y restrictiva, la dieta del enfermo celíaco puede presentar una demanda creciente de productos tales como panes, pastas, embutidos y bebidas alcohólicas con formulaciones especiales. Considerando que no se encontraron estudios similares en la literatura, los objetivos de esta investigación fueron: identificar el repertorio de productos sin gluten disponibles en el mercado paulistano y estimar la diferencia de coste entre una dieta constituida de productos convencionales y otra sin gluten. Caracterizándose como un estudio exploratorio de tipo cualitativo y cuantitativo, se fundamentó en un estudio de mercado. Se realizaron visitas a las tiendas especializadas en las regiones de mayor densidad poblacional y/o que concentran el mayor número de establecimientos en el municipio de São Paulo, y se entrevistó a los responsables de asociaciones, Universidades, Hospitales o Centros de investigación y tratamiento de enfermos Celíacos. Al responsable se le realizó una entrevista semi-estructurada con preguntas abiertas, donde se abordaban aspectos del mercado de productos sin gluten y de la enfermedad celíaca. El análisis de los datos se obtuvo mediante el cálculo de la diferencia porcentual entre los productos encontrados y las dietas preparadas. La dieta sin gluten representa un impacto significativo en el coste alimenticio, especialmente cuando contiene productos como pasta y harina. En la ciudad de São Paulo, una dieta con productos sin gluten puede ser aproximadamente 44% más cara que una dieta con productos convencionales.

Doença Celíaca é uma enteropatia de origem genética, caracterizada pela intolerância permanente ao glúten. Específica e restritiva, a dieta do Celíaco pode apresentar uma demanda crescente de produtos, como pães, massas, embutidos e bebidas alcoólicas com formulações especiais. Considerando-se que não foram encontrados estudos semelhantes na literatura, os objetivos desta investigação foram identificar o repertório de produtos livres de glúten disponíveis no mercado paulistano e estimar a diferença de custo entre uma dieta constituída de produtos convencionais e outra isenta de glúten. Caracterizando-se como um estudo qualitativo e quantitativo exploratório, fundamentou-se em uma pesquisa de mercado. Foram realizadas visitas a lojas especializadas nas regiões de maior densidade demográfica e/ou que concentram maior número de estabelecimentos no município de São Paulo, e entrevistas com os responsáveis por associações, Universidades, Hospitais ou Centros de Investigação e Tratamento de Celíacos. Com o responsável, foi realizada uma entrevista semiestruturada com perguntas abertas, abordando aspectos sobre o mercado de produtos sem glúten e a Doença Celíaca. A análise dos dados foi obtida a partir do cálculo da diferença percentual entre os produtos encontrados e as dietas elaboradas. A dieta isenta de glúten representa um impacto significativo no custo com alimentação, especial¬mente quando contém produtos como macarrão e farinhas. Na cidade de São Paulo, uma dieta com produtos livres de glúten pode ser aproximadamente 44% mais cara do que uma dieta com produtos convencionais.

Síndrome nefrótico e injuria renal aguda con microangiopatía trombótica en mujer con enfermedad celíaca: Caso clínico / Nephrotic syndrome and acute kidney injury associated with celiac disease: Report of one case

Celiac disease may be associated with other autoimmune diseases and exceptionally with glomerulopathies and nephrotic syndrome. Associations have been reported with IgA nephropathy, membranoproliferative glomerulonephritis, membranous glomerulopathy and minimal change disease. We report a 63-year-old woman who simultaneously presented with massive nephrotic syndrome (proteinuria 46 g/day) and cachexia due to a malabsorption syndrome secondary to celiac disease. The course of her diseases was complicated with cardiomyopathy due to severe malnutrition, septic shock, acute kidney injury that required dialysis for seven weeks and severe hypertension. A renal biopsy showed a membranoproliferative pattern of injury secondary to a thrombotic microangiopathy and diffusepodocyte damage. Fouryears later, the patient was in good general health, the glomerular filtration rate was 30 ml/min/1.73m² and there was non-nephrotic proteinuria.

Valor de la técnica de inmersión en el diagnóstico de las enfermedades de la mucosa duodenal / [Value of immersion technique in the diagnosis of diseases of duodenal mucosa].

INTRODUCTION: We used the immersion technique for the evaluation of duodenal mucosa for several years and we observed discordant results in relation to the literature. OBJECTIVE: To evaluate the immersion technique in the diagnosis of duodenal mucosa diseases. PATIENTS AND METHOD: We performed an upper endoscopy, immersion technique, chromoendoscopy and biopsies of the second portion of the duodenum to the patients who met the inclusion criteria. It was reported the presence of villi and the partial or total absence of them. RESULTS: The standard endoscopy showed absence of villi in 16 patients and normal mucosa in 384. The comparison between these results and those of the biopsies showed that biopsies were normal in 3 patients with presumed absence of villi and had a partial atrophy of villi in 1 patient with normal endoscopy. With the immersion technique and the chromoendoscopy we observed absence of villi in 13 patients and normal mucosa in 386. When we compared these results with those of the biopsies, we observed that 1 normal patient had a partial atrophy of villi. These results indicate that standard endoscopy has a sensitivity of 92

, a specificity of 99

, a positive predictive value of 81

and a negative predictive value of 99

. The immersion technique and chromoendoscopy have a sensitivity of 92

, a specificity of 100

, a positive predictive value of 100

and a negative predictive value of 99

. CONCLUSION: In our experience the immersion technique does not improve the visualization of the villi compared with the standard endoscopy and the chromoendoscopy.

Prevalência de doença celíaca em crianças e adolescentes com miocardite e cardiomiopatia dilatada / Celiac disease prevalence in children and adolescents with myocarditis and dilated cardiomiopathy

OBJETIVO: Determinar a prevalência de doença celíaca em pacientes portadores de cardiomiopatia dilatada e miocardite. MÉTODOS: Foram avaliados 56 pacientes, com idade entre 1 e 18 anos, portadores de cardiomiopatia dilatada ou miocardite, acompanhados no Instituto Materno Infantil Professor Fernando Figueira. Foram excluídos pacientes com diagnóstico prévio de doença celíaca. A classe funcional da insuficiência cardíaca foi determinada segundo os critérios da American Heart Association, como classe funcional I, II, III e IV. O diagnóstico de miocardite foi relatado em prontuário, e o de cardiomiopatia dilatada, pelo ecocardiograma, a partir da presença de disfunção sistólica de um ou ambos os ventrículos, com fração de ejeção menor que 55% e dilatação ventricular, com diâmetro diastólico final ventricular esquerdo maior que 112%. Nos pacientes incluídos no estudo, foi aplicado um formulário com informações sobre sintomatologia gastrointestinal e cardiológica; em seguida, dosadas sorologias para anticorpos antitransglutaminase tecidual humana e antiendomísio. Aqueles com sorologia positiva foram encaminhados à biópsia intestinal para avaliação histológica para doença celíaca, segundo os critérios de Marsh. RESULTADOS: Uma das 56 crianças apresentou sorologia antitransglutaminase positiva (1,8%), porém anticorpo antiendomísio negativo. A histologia intestinal demonstrou atrofia total das vilosidades. Cerca de 30% dos pacientes apresentaram insuficiência cardíaca. Sinais e sintomas gastrointestinais foram frequentes nos pacientes, em especial dor abdominal (70%, 39/56). CONCLUSÃO: A frequência de doença celíaca em pacientes com cardiomiopatia dilatada e miocardite foi de 1,8%. É importante investigar doença celíaca nos pacientes com essas doenças cardíacas para evitar evolução das doenças e deterioração clínica do paciente.

OBJECTIVE: To determine the prevalence of celiac disease in patients with myocarditis and dilated cardiomyopathy. METHODS: Fifty-six patients between 1 and 18 years old with dilated cardiomyopathy or myocarditis were evaluated and followed up at Instituto de Medicina Integral Professor Fernando Figueira. Patients with previous diagnosis of celiac disease were excluded. The functional classification was determined according to the American Heart Association criteria (classes I, II, III and IV). Diagnosis of myocarditis was reported in the patients' medical records. Dilated cardiomyopathy was diagnosed by echocardiogram with systolic dysfunction of one or both ventricles, ejection fraction lower than 55%, ventricular dilatation, and left ventricular diastolic diameter bigger than 112%. Patients answered a questionnaire about gastrointestinal and cardiac symptoms; next, anti-tissue transglutaminase (tTG) and anti-endomysial (EMA) antibodies were dosed. Those with positive antibody results were referred to intestinal biopsy and histological evaluation to detect celiac disease according to Marsh classification. RESULTS: One of the 56 children (1.8%) had positive tTG antibody level, but negative EMA. Intestinal histological evaluation showed total villous atrophy. Approximately, 30% of patients had heart failure. Gastrointestinal symptoms and signs were frequent, especially abdominal pain (70%, 39/56). CONCLUSION: Celiac disease prevalence in pediatric patients with dilated cardiomyopathy or myocarditis was 1.8%. It is important to investigate celiac disease in patients with these conditions to avoid the progression of such diseases and patients' clinical deterioration.

Autism spectrum disorder and celiac disease: no evidence for a link / Transtorno autístico e doença celíaca: sem evidências de associação

OBJECTIVE: To evaluate the possible association between celiac disease (CD) and/or gluten sensitivity (GS) and autism spectrum disorder (ASD). METHODS: Occurrences of CD were determined in a group of children and adolescents affected by ASD and, conversely, occurrences of ASD were assessed in a group of biopsy-proven celiac patients. To detect the possible existence of GS, the levels of antigliadin antibodies in ASD patients were assessed and compared with the levels in a group of non-celiac children. RESULTS: The prevalence of CD or GS in ASD patients was not greater than in groups originating from the same geographical area. Similarly the prevalence of ASD was not greater than in a group of biopsy-proven CD patients. CONCLUSION: No statistically demonstrable association was found between CD or GS and ASD. Consequently, routine screening for CD or GS in all patients with ASD is, at this moment, neither justified nor cost-effective.

OBJETIVO: Avaliar a possível associação entre doença celíaca (DC) e/ou sensibilidade ao glúten (SG) e transtorno do espectro autista (TEA). MÉTODOS: Ocorrências de DC foram determinadas em um grupo de crianças e adolescentes afetados pelo TEA e a ocorrência d TEA foi avaliada em um grupo de pacientes com DC comprovada por biópsia. Para detectar a possível existência de SG, foram determinados níveis de anticorpos antigliadina em pacientes com TEA e comparados ao grupo de crianças sem a doença celíaca. RESULTADOS: A prevalência de DC ou SG não foi maior no grupo de pacientes com TEA quando comparada a grupos de indivíduos originários da mesma região geográfica. De modo similar, a prevalência do TEA não foi maior ao ser comparada ao grupo de pacientes com DC. CONCLUSÃO: Não houve associação estatisticamente demonstrável entre DC ou SG e TEA. Consequentemente, não são justificáveis, no momento, exames de rotina para detecção de DC ou SG em pacientes com TEA.