RESUMO
OBJECTIVES: Refractory celiac disease (RCD) is one of the most serious causes of persistent symptoms in patients with celiac disease (CD). Published reports suggest that approximately half of patients in Europe are RCD type II, which carries a poor prognosis with a 5-year survival rate of ~50% compared with ~90% for RCD type I. However, disease patterns may be different in North America. The aim of this study was to explore the clinical spectrum of RCD in a North American population. METHODS: Medical records of patients with biopsy-proven CD presenting to our institution were reviewed for a diagnosis of RCD. Demographic data, clinical characteristics, and mortality were evaluated and compared with our general CD population. RESULTS: In all, 34 out of 844 (4.0%) CD patients had RCD. The cumulative incidence of RCD for patients diagnosed with CD at our center was 1.5%. Unintentional weight loss at diagnosis of RCD was found in 76.5% (n=26) compared with 16.7% (n=141) at diagnosis of CD (P<0.0001) and diarrhea at diagnosis of RCD was found in 79.4% (n=27) compared with 40.5% (342) at diagnosis of CD (P<0.0001). Five patients (14.7%) were diagnosed with RCD type II and of these, two died of enteropathy-associated lymphoma within 24 months of diagnosis of CD (observed mortality rate 5.9%). CONCLUSIONS: Although RCD is a serious condition with significant morbidity; the observed mortality rates are low in our population. This study suggests that RCD may be less severe in North American vs. European populations.
Assuntos
Doença Celíaca/diagnóstico , Biópsia por Agulha , Doença Celíaca/classificação , Doença Celíaca/mortalidade , Doença Celíaca/patologia , Duodeno/patologia , Europa (Continente) , Humanos , Imunofenotipagem , Incidência , Prognóstico , Taxa de Sobrevida , Estados UnidosRESUMO
GOAL: The goal of this study is to evaluate the safety and efficacy of Small intestinal release mesalamine (SIRM) for symptom relief in refractory celiac disease (RCD). BACKGROUND: Therapeutic options for the RCD are inadequate and treatment with corticosteroids and immunosuppressants is limited by side effects. SIRM has been shown to have local antiinflammatory action and excellent tolerability. STUDY: We reviewed records of the RCD patients who received SIRM in an open-label therapeutic trial. Data included demographics, disease characteristics, dose and duration of SIRM therapy, and response. Response was categorized as complete if there was complete resolution of symptoms, partial if there was at least 50% improvement, and nonresponsive if there was less than 50% improvement. RESULTS: Four patients were treated with SIRM alone and 6 received SIRM and oral budesonide. Within 4 weeks, 50% had complete response and an additional 10% had partial response. Two of the 6 patients were able to discontinue budesonide. One patient discontinued SIRM owing to headaches. CONCLUSION: SIRM seems to be a safe and efficacious treatment option in patients with RCD. Larger, controlled trials of this agent are warranted.
Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Doença Celíaca/tratamento farmacológico , Mesalamina/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Inflamatórios não Esteroides/administração & dosagem , Anti-Inflamatórios não Esteroides/efeitos adversos , Budesonida/administração & dosagem , Budesonida/uso terapêutico , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Cefaleia/induzido quimicamente , Humanos , Intestino Delgado/metabolismo , Masculino , Mesalamina/administração & dosagem , Mesalamina/efeitos adversos , Pessoa de Meia-Idade , Resultado do TratamentoRESUMO
BACKGROUND & AIMS: Celiac crisis is a life-threatening syndrome in which patients with celiac disease have profuse diarrhea and severe metabolic disturbances. Celiac crisis is rare among adults and not well documented. To improve awareness of this condition and to facilitate diagnosis, we reviewed cases of celiac crisis to identify presenting features, formulate diagnostic criteria, and develop treatment strategies. METHODS: Cases of biopsy-proven celiac disease were reviewed. Celiac crisis was defined as acute onset or rapid progression of gastrointestinal symptoms that could be attributed to celiac disease and required hospitalization and/or parenteral nutrition, along with signs or symptoms of dehydration or malnutrition. RESULTS: Twelve patients met preset criteria for celiac crisis; 11 developed celiac crisis before they were diagnosed with celiac disease. Eleven patients had increased titres of transglutaminase antibodies and 1 had immunoglobulin A deficiency. Results of biopsy analyses of duodenum samples from all patients were consistent with a Marsh 3 score (33% with total villous atrophy). Patients presented with severe dehydration, renal dysfunction, and electrolyte disturbances. All patients required hospitalization and intravenous fluids, 6 required corticosteroids, and 5 required parenteral nutrition. All patients eventually had a full response to a gluten-free diet. CONCLUSIONS: Celiac crisis has a high morbidity and, although rarely described, occurs in adults and often has a clear precipitating factor. Patients who present with severe unexplained diarrhea and malabsorption should be tested for celiac disease; treatment with systemic steroids or oral budesonide should be considered. Nutritional support often is required in the short term but most patients ultimately respond to gluten avoidance.
Assuntos
Doença Celíaca/complicações , Doença Celíaca/patologia , Estado Terminal/epidemiologia , Adulto , Idoso , Anti-Inflamatórios/uso terapêutico , Budesonida/uso terapêutico , Doença Celíaca/diagnóstico , Doença Celíaca/tratamento farmacológico , Desidratação/etiologia , Dieta , Feminino , Trato Gastrointestinal/patologia , Hospitalização , Humanos , Masculino , Desnutrição/etiologia , Pessoa de Meia-Idade , Nutrição Parenteral , Esteroides/uso terapêuticoRESUMO
BACKGROUND & AIMS: Although the incidence of celiac disease (CD) is increasing, studies have been hampered by the lack of validated outcome measures. We sought to create a disease-specific Celiac Symptom Index (CSI) to reliably assess relevant symptoms. METHODS: A 36-item questionnaire was created after design by an expert committee and review/revision by patient focus groups. The survey, covering domains of CD-related symptoms and general health, was initially administered to 154 individuals with biopsy-proven CD; immunoglobulin (Ig)A tissue transglutaminase titers were determined, and gluten-free diet adherence was evaluated by a dietitian. The questionnaire was then revised to exclude questions with poor test characteristics and administered to a second, independent group of 52 individuals, to ensure validity. RESULTS: The subscales of "specific symptoms" and "general health" had excellent psychometric qualities that consisted of 11 and 5 items, respectively. The additive score based on these items was correlated with current general health, as measured by a visual analog scale and short form 36 general health subscale (P < or = .001 for both), as well as degree of adherence to the gluten-free diet (P = .008), lending external validity to the CSI. The resulting 16 questions make up the first CD-specific symptom index. CONCLUSIONS: The CSI allows for disease-specific monitoring of symptoms as an independent outcome measure or as part of a surrogate for disease activity in individuals with CD. The CSI might be an important tool for future clinical CD research.
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Doença Celíaca/diagnóstico , Doença Celíaca/fisiopatologia , Índice de Gravidade de Doença , Adulto , Doença Celíaca/patologia , Dieta Livre de Glúten , Feminino , Grupos Focais , Humanos , Imunoglobulina A/sangue , Masculino , Pessoa de Meia-Idade , Inquéritos e QuestionáriosRESUMO
BACKGROUND & AIMS: Celiac disease is an increasingly prevalent disorder. To monitor response to treatment in clinical and research settings, it is essential to accurately measure gluten-free diet (GFD) adherence in a standardized manner. The aim of this study was to develop a valid and reliable Celiac Dietary Adherence Test (CDAT). METHODS: Items and domains believed to be essential for successful GFD adherence were used to develop an 85-item survey with input from patient focus groups. The survey was administered to 200 individuals with biopsy-proven celiac disease who underwent standardized dietician evaluation (SDE) and serologic testing. RESULTS: Of the initial 85 items, 41 were correlated highly with the SDE (P < .01). Responses for all 200 participants for the 41 items were entered into a single database. Computer-generated randomization produced a derivation cohort of 120 subjects and a validation cohort of 80. By using the derivation cohort, a 7-item questionnaire was developed using logistic regression. The additive score based on these items was correlated highly with the SDE in both the derivation and validation cohorts (P < .001) and performed significantly better than immunoglobulin A tissue transglutaminase titers in receiver operating characteristic curve analysis with areas under the curve of 0.830 and 0.652, respectively. CONCLUSIONS: The CDAT is a clinically relevant, easily administered, 7-item instrument that allows for standardized evaluation of GFD adherence and is superior to tissue transglutaminase serology. The CDAT may be useful in both research and clinical settings.
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Doença Celíaca/terapia , Coleta de Dados/métodos , Dieta Livre de Glúten , Comportamento Alimentar , Cooperação do Paciente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Distribuição AleatóriaRESUMO
Hepatitis B and hepatitis C are important causes of chronic liver disease globally. Although HBV/HCV coinfection is not uncommon, its epidemiology is poorly defined. Numerous studies provided evidence that coinfection accelerates liver disease progression and increases the risk of hepatocellular carcinoma. By applying new cell culture models to examine the interaction of both viruses, investigators concluded that HBV and HCV replicate in the same hepatocyte without interference. The roles of innate and adaptive immunity in determining the viral replication and disease outcomes still need rigorous investigation. To date, no standard-of-care recommendation exists for HBV/HCV coinfection. Pegylated interferon and ribavirin combination therapy demonstrated similar efficacy in suppressing HCV RNA in coinfection and HCV monoinfection. However, HBV reactivation during therapy can be a challenge. Future clinical trials evaluating the addition of a nucleoside/nucleotide analog for selective patients with HBV/HCV coinfection are essential for successful management of HBV/HCV coinfection.