Enfermedad relacionada con IgG4 con posible afectación miocárdica / IgG4-related disease with possible myocardial involvement

La enfermedad relacionada con el depósito de IgG4 es una entidad caracterizada por lesiones tumefactas, infiltrado denso linfoplasmocítico con positividad inmunohistoquímica para IgG4, fibrosis estoriforme y, frecuentemente, niveles séricos elevados de IgG4. Puede cursar con afectación multisistémica; sin embargo, la afectación miocárdica, objetivada mediante pruebas de imagen, no ha sido descrita en la literatura médica. Se presenta el caso clínico de un varón afecto de enfermedad relacionada con IgG4 con posible afectación miocárdica, detectada a través de cardiorresonancia magnética, que plantea el diagnóstico diferencial con otras enfermedades como la sarcoidosis y la enfermedad de Fabry, cuyo diagnóstico diferencial es de gran importancia por su repercusión terapéutica

IgG4-related disease is characterized by mass lesions, a dense lymphoplasmacytic infiltrate with immunohistochemical positivity for IgG4, storiform fibrosis and, frequently, elevated serum IgG4 levels. It can be multisystemic; however, myocardial involvement, which is objectively determined by imaging tests, has not been described in the medical literature. We report the case of a man with IgG4-related disease with possible myocardial involvement, detected by cardiac magnetic resonance. This raises the question of a differential diagnosis with other diseases such as sarcoidosis and Fabry disease, the differential diagnosis of which is of great importance due to its therapeutic impact

Miastenia gravis y leucemia de linfocitos T grandes granulares / Myasthenia gravis and large granular lymphocytic leukemia

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IgG4-related disease with possible myocardial involvement. / Enfermedad relacionada con IgG4 con posible afectación miocárdica.

IgG4-related disease is characterized by mass lesions, a dense lymphoplasmacytic infiltrate with immunohistochemical positivity for IgG4, storiform fibrosis and, frequently, elevated serum IgG4 levels. It can be multisystemic; however, myocardial involvement, which is objectively determined by imaging tests, has not been described in the medical literature. We report the case of a man with IgG4-related disease with possible myocardial involvement, detected by cardiac magnetic resonance. This raises the question of a differential diagnosis with other diseases such as sarcoidosis and Fabry disease, the differential diagnosis of which is of great importance due to its therapeutic impact.

IgG4-related disease in thymus. A very rare case of chronic fibrosis mimicking sarcoidosis.

IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lymphoplasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally. The epidemiology remains poorly studied, but it has been noted that in the majority of recorded instances, patients are middle-aged men. IgG4-RD could mimic conditions other than tumors, such as infection, inflammation, or other systemic disorders. To ensure accuracy of diagnosis, an exhaustive histopathological analysis is required, together with clinical, radiological, and serological data. Thymic fibrosis in the absence of other primary thymic lesions is a very rare occurrence; in English literature only 1 case has been reported with scattered IgG4 plasma cells infiltrate and focal obliterative phlebitis. We will describe, for the first time, the case of a 49-year-old man displaying an anterior mediastinic, hilar, and intramyocardial mass simulating a sarcoidosis, with a definitive diagnosis of IgG4-related thymic fibrosis extending to the mediastinum and the heart. At the histological examination, we found many features of IgG4-RD in the thymic tissue, such as diffused storiform fibrosis, dense lymphoplasmacytic infiltrate with abundant plasma cells IgG4 positive (ratio IgG/IgG4: 40%), obliterative phlebitis, eosinophilic infiltrate, and Castleman-like lymphoid follicles. We discussed the differential diagnosis and reviewed the literature and the other cases of IgG4-related diseases that had been diagnosed in our department.

Sprue-like enteropathy due to olmesartan.

A case of spue-like enteropathy due to olmesartan is reported to draw attention to this disease, given the high frequency of use of this drug and the difficulty of diagnosis if the entity if it is not known. In his journal one case was published as Clinical Note in 2014 and we wish to emphasize the importance of knowledge about this relatively new entity.

Enteropatía sprue-like causada por olmesartán / Sprue-like enteropathy due to olmesartan

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Benefit on health-related quality of life of adherence to gluten-free diet in adult patients with celiac disease.

INTRODUCTION: Celiac disease (CD) affects health-related quality of life (HRQOL) of patients suffering it. The exclusion of gluten from the diet (GFD) improves HRQOL, but involves difficulties in following the diet that could adversely affect HRQOL. OBJECTIVE: To determine the effect of adherence to the diet on HRQOL of adult CD patients. METHODS: A prospective, cross-sectional, multicenter study of CD patients treated with a GFD for longer than 1 year. Adherence to the GFD was measured using the Morisky scale, and health status using the specific CD-QOL questionnaire and the generic EuroQol-5D questionnaire. RESULTS: 366 patients from 7 hospitals were included: 71.5% of patients reported a perfect treatment adherence, 23.5% unintentional poor adherence and 5% intentional poor adherence. Good adherence to a GFD was related to a higher mean score onthe CD-QOL (75 vs. 68, respectively, p < 0.05) and EuroQol-5D (0.9 vs. 0.8, respectively, p < 0.05). Ease of adherence to a GFD was also related to a better HRQOL (total CD-QOL score of 82 vs. 67 in patients who consider the GFD difficult to follow, p < 0.05). Good symptom control was also related to a better HRQOL (total CD-QOL score of 78 vs. 67 in asymptomatic vs. symptomatic patients, p < 0.01). The worse scored dimension of CD-QOL was related to "inadequate treatment". CONCLUSIONS: In CD, good adherence to a GFD and adequate symptom control result in improved HRQOL. Many patients consider that the lack of therapeutic alternatives to diet worsens their quality of life.

Benefit on health-related quality of life of adherence to gluten-free diet in adult patients with celiac disease

INTRODUCTION: Celiac disease (CD) affects health-related quality of life (HRQOL) of patients suffering it. The exclusion of gluten from the diet (GFD) improves HRQOL, but involves difficulties in following the diet that could adversely affect HRQOL. OBJECTIVE: To determine the effect of adherence to the diet on HRQOL of adult CD patients. METHODS: A prospective, cross-sectional, multicenter study of CD patients treated with a GFD for longer than 1 year. Adherence to the GFD was measured using the Morisky scale, and health status using the specific CD-QOL questionnaire and the generic EuroQol-5D questionnaire. RESULTS: 366 patients from 7 hospitals were included: 71.5% of patients reported a perfect treatment adherence, 23.5% unintentional poor adherence and 5% intentional poor adherence. Good adherence to a GFD was related to a higher mean score on the CD-QOL (75 vs. 68, respectively, p < 0.05) and EuroQol-5D (0.9 vs. 0.8, respectively, p < 0.05). Ease of adherence to a GFD was also related to a better HRQOL (total CD-QOL score of 82 vs. 67 in patients who consider the GFD difficult to follow, p < 0.05). Good symptom control was also related to a better HRQOL (total CD-QOL score of 78 vs. 67 in asymptomatic vs. symptomatic patients, p < 0.01). The worse scored dimension of CD-QOL was related to 'inadequate treatment.' CONCLUSIONS: In CD, good adherence to a GFD and adequate symptom control result in improved HRQOL. Many patients consider that the lack of therapeutic alternatives to diet worsens their quality of life

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Short-term effect of antibiotics on human gut microbiota.

From birth onwards, the human gut microbiota rapidly increases in diversity and reaches an adult-like stage at three years of age. After this age, the composition may fluctuate in response to external factors such as antibiotics. Previous studies have shown that resilience is not complete months after cessation of the antibiotic intake. However, little is known about the short-term effects of antibiotic intake on the gut microbial community. Here we examined the load and composition of the fecal microbiota immediately after treatment in 21 patients, who received broad-spectrum antibiotics such as fluoroquinolones and ß-lactams. A fecal sample was collected from all participants before treatment and one week after for microbial load and community composition analyses by quantitative PCR and pyrosequencing of the 16S rRNA gene, respectively. Fluoroquinolones and ß-lactams significantly decreased microbial diversity by 25% and reduced the core phylogenetic microbiota from 29 to 12 taxa. However, at the phylum level, these antibiotics increased the Bacteroidetes/Firmicutes ratio (p = 0.0007, FDR = 0.002). At the species level, our findings unexpectedly revealed that both antibiotic types increased the proportion of several unknown taxa belonging to the Bacteroides genus, a Gram-negative group of bacteria (p = 0.0003, FDR<0.016). Furthermore, the average microbial load was affected by the treatment. Indeed, the ß-lactams increased it significantly by two-fold (p = 0.04). The maintenance of or possible increase detected in microbial load and the selection of Gram-negative over Gram-positive bacteria breaks the idea generally held about the effect of broad-spectrum antibiotics on gut microbiota.

Adaptación transcultural y validación del “Celiac Disease Quality of Life (CD-QOL) survey”, un cuestionario específico de medida de la calidad de vida en pacientes con enfermedad celiaca / Transcultural adaptation and validation of the Celiac Disease Quality of Life (CD-QOL) survey, a specific questionnaire to measure quality of life in patients with celiac disease

Introducción: la enfermedad celiaca es una enfermedad crónica que requiere de un tratamiento continuado, lo que conlleva un impacto sobre la calidad de vida relacionada con la salud (CVRS) de las personas que la padecen. La mayoría de estudios en nuestro medio han utilizado cuestionarios genéricos para medir la CVRS en los pacientes celiacos, lo que ha motivado el presente estudio para traducir y validar al castellano un cuestionario específico para la enfermedad celiaca, el “Celiac Disease Quality Of Life Survey” (CD-QOL). Objetivos: traducir y validar al castellano el cuestionario específico para la enfermedad celiaca CD-QOL. Métodos: se ha diseñado un estudio multicéntrico, prospectivo y observacional desarrollado en dos fases: la primera de traducción/adaptación al idioma castellano mediante el procedimiento de traducción/retrotraducción y estudio de comprensibilidad. A continuación se desarrolló la segunda fase de análisis de la consistencia interna del cuestionario traducido. Para ello se han comparado los resultados del CD-QOL con los del EuroQol y la escala de impacto diario de la fatiga (EIDF). El estudio de comprensibilidad del cuestionario traducido y adaptado se realizó en 6 pacientes, y el estudio de validación en 298 celiacos (201 tratados con dieta sin gluten y 97 al momento del diagnóstico). Resultados: en ambos grupos de celiacos el coeficiente alfa de Cronbach fue elevado (0,90), la factibilidad fue excelente (99,2 % de pacientes completaron todas las preguntas) y el efecto techo y suelo fue nulo. La correlación de Spearman con el EuroQol y el EIDF fue estadísticamente significativa (p < 0,05). El CD-QOL puntuó diferente según el estado de salud fuera bueno, regular o malo en función de la puntuación del EuroQol. Conclusión: la versión en castellano del CD-QOL es un instrumento válido de medida de la CVRS de los pacientes celiacos (AU)

Introduction: celiac disease is a chronic condition that requires continued treatment, with the resultant impact on health-related quality of life (HRQOL) of people who suffer it. Most studies in this field have used generic questionnaires to measure HRQOL in celiac patients. It was therefore decided to conduct a study to translate into Spanish and validate a specific questionnaire for celiac disease, the Celiac Disease Quality Of Life Survey (CD-QOL). Objectives: to translate and validate in Spanish the specific celiac disease questionnaire CD-QOL. Methods: a multicenter, prospective, observational study was designed consisting of two phases: In the first phase, the questionnaire was translated and adapted into Spanish using the translation/back translation procedure and an understandability study. In the second phase, internal consistency of the translated questionnaire was analyzed. For this, results of the CD-QOL were compared to those of EuroQol and the Daily Fatigue Impact Scale (D-FIS). Understandability of the translated and adapted questionnaire was tested in six patients, and the validation study was done in 298 celiac patients (201 treated with a gluten-free diet and 97 at diagnosis). Results: in both celiac groups, Cronbach’s alpha coefficient was high (0.90), feasibility was excellent (99.2 % of patients completed all questions), and there were no ceiling and floor effects. Spearman correlation to EuroQol and D-FIS was statistically significant (p < 0.05). CD-QOL score was different depending on whether state of health was good, fair, or poor based on the EuroQol score. Conclusion: the Spanish version of the CD-QOL is a valid tool for measuring HRQOL in celiac patients (AU)

Transcultural adaptation and validation of the Celiac Disease Quality of Life (CD-QOL) Survey, a specific questionnaire to measure quality of life in patients with celiac disease.

INTRODUCTION: celiac disease is a chronic condition that requires continued treatment, with the resultant impact on health-related quality of life (HRQOL) of people who suffer it. Most studies in this field have used generic questionnaires to measure HRQOL in celiac patients. It was therefore decided to conduct a study to translate into Spanish and validate a specific questionnaire for celiac disease, the Celiac Disease Quality Of Life Survey (CD-QOL). OBJECTIVES: to translate and validate in Spanish the specific celiac disease questionnaire CD-QOL. METHODS: a multicenter, prospective, observational study was designed consisting of two phases: In the first phase, the questionnaire was translated and adapted into Spanish using the translation/back translation procedure and an understandability study. In the second phase, internal consistency of the translated questionnaire was analyzed. For this, results of the CD-QOL were compared to those of EuroQol and the Daily Fatigue Impact Scale (D-FIS). Understandability of the translated and adapted questionnaire was tested in six patients, and the validation study was done in 298 celiac patients (201 treated with a gluten-free diet and 97 at diagnosis). RESULTS: in both celiac groups, Cronbach´s alpha coefficient was high (0.90), feasibility was excellent (99.2 % of patients completed all questions), and there were no ceiling and floor effects. Spearman correlation to EuroQol and D-FIS was statistically significant (p < 0.05). CD-QOL score was different depending on whether state of health was good, fair, or poor based on the EuroQol score. CONCLUSION: the Spanish version of the CD-QOL is a valid tool for measuring HRQOL in celiac patients.

Fatigue as a determinant of health in patients with celiac disease.

BACKGROUND: Symptoms of uncontrolled celiac disease impair patients' health-related quality of life, which improves with the exclusion of gluten from the diet. A symptom frequently reported, but difficult to make objective, is fatigue. The Daily Fatigue Impact Scale (D-FIS) questionnaire consisting of 8 items, each scored on a 0 to 4 point scale, with lower scores reflecting greater fatigue, was employed to measure fatigue in celiac patients. GOALS: To assess the influence of fatigue on perception of health in celiac disease patients determined as their quality of life. STUDY: Prospective, cross-sectional study in celiac disease patients diagnosed by serology and histology. Instruments used were the D-FIS to measure fatigue and the generic EuroQol5D to measure quality of life. An additional question on the frequency of problems due to fatigue, scored on a 7-point Likert scale, was used to evaluate the importance of fatigue. RESULTS (IN MEDIANS): In all, 51 patients were included (13 untreated and 38 treated with a gluten-free diet). D-FIS score was significantly worse in untreated celiacs (16.0 vs. 3.0, P<0.001). Scores on the frequency scale of fatigue-related problems were also worse in untreated celiacs (2.0 vs. 6.0, P<0.001). Fatigue and quality-of-life scores were inversely correlated (r=-0.6, P<0.001). Fatigue severity was also greater in patients with worse quality of life (13.0 vs. 2.0, P<0.01). Multiple regression analysis showed fatigue to be an independent determinant of quality of life. CONCLUSIONS: Fatigue is a major concern in untreated celiac disease patients, which impacts their quality of life.

Factors affecting health related quality of life of patients with inflammatory bowel disease.

UNLABELLED: Using a disease specific instrument to measure the health related quality of life (HRQOL) of patients with inflammatory bowel disease (IBD), it has been shown that their perceived HRQOL worsens during active disease. The precise factors involved in HRQOL changes reported by these patients are largely unknown. Our aim was to elucidate which socio-demographic and health status variables are related with HRQOL in IBD patients. To this end, 354 patients with IBD were interviewed. To quantify the impairment in the HRQOL, the 36-item version of the inflammatory bowel disease questionnaire (IBDQ) was administered to all patients. To explore the relation of each individual variable on the HRQOL an univariate analysis by using the Spearman correlation, the Mann-Whitney or the Kruskal-Wallis test was performed when necessary. Factors significant at the univariate analysis were assessed using multiple linear regression modeling with global IBDQ score as the dependent factor. RESULTS: Disease type did not predict IBDQ score in the univariate nor in the multivariate analysis. Consequently, statistical analysis was performed in the global group of 354 patients independently of the type of disease. Lower recurrence/year index, longer disease duration, higher level of education, symptom activity, male gender and non-necessity of hospitalization all predict a better HRQOL (p < 0.05). Factors which remained significant (p < 0.05) in the multiple regression modeling were gender, need of hospitalization, symptomatic activity, recurrence/year index and education level. CONCLUSIONS: Symptomatic activity and socio-demographic variables such as gender and education are the most important factors involved in the impairment of HRQOL in patients with IBD.