Immunoglobulin G4-Related Disease, A Diagnosis to Take into Account: About a Case

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated and multisystemic condition associated with developing fibroinflammatory lesions in any organ. The diagnosis is made based on the sum of clinical, serological, radiological, and histopathological criteria; however, this is often difficult due to its similarity to neoplasms, infections, or other immune-mediated diseases. Treatment is based on corticosteroids, in a possible combination with immunomodulators. The present case concerns a 59-year-old man with a history of jaundice syndrome and weight loss, admitted for suspected malignant neoplasia of the bile duct. Imaging revealed stricture with dilation of the intrahepatic bile ducts, prominent pancreas, pancreatic duct stricture, and nodular renal lesions. Due to the history of left submandibulectomy two years before the current disease and histology compatible with Küttner's tumor, plus the high IgG4 serum values, the diagnosis of IgG4-RD was established. He started treatment with corticosteroids and was asymptomatic during follow-up.

La enfermedad relacionada con inmunoglobulina G4 (ER-IgG4) es una condición inmunomediada y multisistémica asociada al desarrollo de lesiones fibroinflamatorias en cualquier órgano. El diagnóstico se realiza bajo la suma de criterios clínicos, serológicos, radiológicos e histopatológicos; sin embargo, este suele ser difícil debido a su similitud con neoplasias, infecciones u otras enfermedades inmunomediadas. El tratamiento se basa en corticosteroides, en una posible combinación con inmunomoduladores. El presente caso trata de un varón de 59 años con historia de síndrome ictérico y baja de peso, admitido por sospecha de neoplasia maligna de la vía biliar. Las imágenes revelaron estenosis con dilatación de las vías biliares intrahepáticas, páncreas prominente, estenosis del conducto pancreático y lesiones nodulares renales. Debido al antecedente de submandibulectomía izquierda 2 años antes de la enfermedad actual e histología compatible con tumor de Küttner, más los valores elevados de IgG4 sérico, se estableció el diagnóstico de ER-IgG4. Inició tratamiento con corticosteroides y cursó asintomático durante el seguimiento.

[Inflammatory Bowel Disease in elderly patients: Clinical characteristics and management in a tertiary hospital]. / Enfermedad Inflamatoria Intestinal en el adulto mayor: Características clínicas y manejo en un hospital de referencia.

Inflammatory bowel disease (IBD) in elderly patients is characterized by its clinical variability, different differential diagnoses and therapeutic management. The objective of our investigation is to evaluate the clinical characteristics and management of elderly patients with IBD. We developed an observational, descriptive, retrospective study from January 2011 to December 2019 in patients with IBD at the Gastroenterology Service of Guillermo Almenara Irigoyen National Hospital, Lima-Peru. 55 patients with CD and 107 with UC were evaluated; 45.6% of patients with IBD are older adults. Of these, 28 had CD and 46 UC. Older adults with CD presented predominantly an inflammatory phenotype and colonic location, while extensive and left-sided colitis were the most frequent in UC. Elderly patients had a lower CDAI score (279.8 vs 323.2) and a lower Mayo index (7.1 vs 9.2) in relation to the younger, without significant differences. Regarding treatment, a lower use of azathioprine (2 vs 8, p <0.03) and Anti-TNF (9 vs 18, p <0.01) was observed in the elderly with CD. The need for surgery and the frequency of post-surgical complications were similar between both groups. In conclusion, nearly half of IBD patients are older adults. The colonic location was the most frequent in CD, and in UC extensive and left colitis. We observed a lower use of azathioprine and biological therapy in elderly patients, without significant differences in the use of corticosteroids and aminosalicylates compared to younger people.

Enfermedad Inflamatoria Intestinal en el adulto mayor: Características clínicas y manejo en un hospital de referencia

La enfermedad inflamatoria intestinal (EII) en adultos mayores se caracteriza por su variabilidad clínica, distintos diagnósticos diferenciales y manejo terapéutico. El objetivo de la presente investigación es evaluar las características clínicas y manejo de los pacientes adultos mayores con EII. Se realizó un estudio observacional, descriptivo, retrospectivo de enero del 2011 a diciembre del 2019 en pacientes con EII en el Servicio de Gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen, Lima-Perú. Fueron evaluados 55 pacientes con EC y 107 con CU; 45,6% de pacientes con EII eran adultos mayores. De ellos, 28 tenían EC y 46 CU. Los adultos mayores con EC presentaron fenotipo inflamatorio y localización colónica predominantemente, mientras en CU, la colitis extensa e izquierda fueron las más frecuentes. Asimismo, los ancianos tuvieron menor puntaje CDAI (279,8 vs 323,2) y menor índice de Mayo (7,1 vs 9,2) con relación a los pacientes jóvenes, sin diferencias significativas. Respecto al tratamiento, se observó un menor uso de azatioprina (2 vs 8, p<0,03) y Anti-TNF (9 vs 18, p<0,01) en los adultos mayores con EC. La necesidad de cirugía y la frecuencia de complicaciones post quirúrgicas fueron similares entre ambos grupos. En conclusión, casi la mitad de los pacientes con EII son adultos mayores. La localización colónica fue la más frecuente en EC, y en CU la colitis extensa e izquierda. Observamos un menor uso de azatioprina y terapia biológica en adultos mayores, sin diferencias significativas en el uso de corticoides y aminosalicilatos respecto a los jóvenes.

Inflammatory bowel disease (IBD) in elderly patients is characterized by its clinical variability, different differential diagnoses and therapeutic management. The objective of our investigation is to evaluate the clinical characteristics and management of elderly patients with IBD. We developed an observational, descriptive, retrospective study from January 2011 to December 2019 in patients with IBD at the Gastroenterology Service of Guillermo Almenara Irigoyen National Hospital, Lima-Peru. 55 patients with CD and 107 with UC were evaluated; 45.6% of patients with IBD are older adults. Of these, 28 had CD and 46 UC. Older adults with CD presented predominantly an inflammatory phenotype and colonic location, while extensive and left-sided colitis were the most frequent in UC. Elderly patients had a lower CDAI score (279.8 vs 323.2) and a lower Mayo index (7.1 vs 9.2) in relation to the younger, without significant differences. Regarding treatment, a lower use of azathioprine (2 vs 8, p <0.03) and Anti-TNF (9 vs 18, p <0.01) was observed in the elderly with CD. The need for surgery and the frequency of post-surgical complications were similar between both groups. In conclusion, nearly half of IBD patients are older adults. The colonic location was the most frequent in CD, and in UC extensive and left colitis. We observed a lower use of azathioprine and biological therapy in elderly patients, without significant differences in the use of corticosteroids and aminosalicylates compared to younger people.

[Medical and surgical management of moderate-to-severe inflamatory bowel disease]. / Manejo médico quirúrgico de la enfermedad inflamatoria intestinal moderada-severa.

The management of inflammatory bowel disease (IBD) is mainly medical, however, more than 70% of patients with Crohn's disease (CD) and 25% with ulcerative colitis (UC) will require surgery during their lifetime. OBJECTIVE: To evaluate medical, surgical management and evolution in patients with moderate-to-severe IBD. MATERIALS AND METHODS: Observational, descriptive, retrospective study from January 2011 to December 2019 in the Gastroenterology Service of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. RESULTS: Twenty two patients with IBD, 17 with CD and 5 with UC were included. Male predominance (59%). Emergency surgery was performed in 35.2% and 60% of patients with CD and UC, respectively. Stenosis and toxic megacolon were the most frequent indications. According to the type of surgery, hemicolectomy (41%) and intestinalresection (41%) were the most frequently performed in CD, while in UC it was total colectomy (60%). Among the postoperative complications, dehiscence/fistula and intra-abdominal collections were the most frequently reported in CD; whereas in UC it was surgical site infection and adynamic ileus. After surgery, biologics and 5-ASA associated with immunomodulator were the most used treatment in CD and UC, respectively. Mortality was 17.6% in CD and 60% in UC. CONCLUSIONS: Surgical treatment is an option in the management of moderate-to-severe IBD. Emergency surgery in IBD continues to have a high morbidity and mortality rate.

Manejo de la hemorragia digestiva alta en pacientes con infección por SARS-CoV-2 en un hospital de Lima, Perú / Management of upper gastrointestinal bleeding in patients with SARS-COV-2 infection in a hospital in Lima-Peru

Resumen Introducción: la pandemia por SARS-CoV-2 ha conllevado la reestructuración de las unidades de endoscopía digestiva en el mundo, lo cual ha limitado los procedimientos endoscópicos y priorizado indicaciones de emergencia como la hemorragia digestiva alta (HDA). No obstante, existe escasa evidencia respecto a su impacto en la evolución y resultados. Objetivo: evaluar el manejo de la HDA en el contexto de la pandemia del coronavirus por SARS-CoV-2. Materiales y métodos: estudio observacional, descriptivo, retrospectivo de marzo a agosto de 2020 en pacientes con diagnóstico de HDA e infección por SARS-CoV-2. Resultados: de 4320 pacientes con infección por SARS-CoV-2, 51 presentaron HDA al ingreso. La mediana de edad fue de 70 años. El 58,8 % era de sexo masculino. El 56,9 % tenía una puntuación de Glasgow-Blatchford (SGB) ≥12. El 21,6 % requirió soporte de oxígeno. Solo 34 pacientes (66,7 %) recibieron tratamiento médico; asimismo, 17 (33,3 %) recibieron tratamiento médico más endoscopia digestiva alta (EDA); de estos, a 6 (35,3 %) se les realizó endoscopia terapéutica. La enfermedad ulcerosa péptica fue el hallazgo más frecuente. Al comparar el tipo de tratamiento recibido, no hubo diferencias significativas entre el número de transfusiones de glóbulos rojos, resangrado, reingreso por HDA, estancia hospitalaria ni mortalidad secundaria a la HDA. La mortalidad global fue del 25,4 % (13 pacientes) y se debió, principalmente, al compromiso respiratorio por SARS-CoV-2. Conclusiones: se observa una reducción en el número de EDA de emergencia por HDA en la pandemia actual, así como un tiempo mayor al estándar para su realización. Más del 80 % de los pacientes que recibieron solo tratamiento médico evolucionaron favorablemente, y solo un tercio de los pacientes a quienes se les realizó una EDA requirió terapéutica endoscópica.

Abstract Introduction: The SARS-CoV-2 pandemic has led to the restructuring of digestive endoscopy units around the world, limiting endoscopic procedures and prioritizing emergency indications such as upper gastrointestinal hemorrhage (UGH). However, there is little evidence regarding its impact on evolution and outcomes. Objective: To evaluate the management of UGH in the context of the SARS-CoV-2 coronavirus pandemic. Materials and methods: Observational, descriptive, retrospective study carried out between March and August 2020 in patients with diagnosis of UGH and SARS-CoV-2 infection. Results: Of 4 320 patients with SARS-CoV-2 infection, 51 had UGH on admission. The median age of the population was 70 years and 58.8% were male. Glasgow-Blatchford Bleeding Score (GBS) of ≥12 was obtained in 56.9%. Oxygen support was required by 21.6%. 34 (66.7%) patients received medical treatment only, while 17 (33.3%) received medical treatment plus upper gastrointestinal endoscopy (UGE), of which 6 (35.3%) underwent therapeutic endoscopy. Peptic ulcer disease was the most frequent finding. When comparing the type of treatment received, there were no significant differences between the number of red blood cell transfusions, rebleeding, re-admission due to UGH, hospital stay, or mortality secondary to UGH. Overall mortality was 25.4% (13 patients), mainly due to respiratory failure due to SARS-CoV-2. Conclusions: A reduction in the number of emergency upper gastrointestinal endoscopies for UGH was observed during the current pandemic, as well as a longer than standard time for their performance. More than 80% of patients who received medical treatment alone evolved favorably and only one third of the patients who underwent UGE required endoscopic therapy.

Manejo médico quirúrgico de la enfermedad inflamatoria intestinal moderada-severa

El tratamiento de la enfermedad inflamatoria intestinal (EII) es principalmente médico, sin embargo, más del 70% de pacientes con enfermedad de Crohn (EC) y 25% con colitis ulcerativa (CU) requerirán cirugía. Objetivos: Evaluar el manejo médico- quirúrgico y evolución en pacientes con EII moderada-severa. Materiales y métodos: Estudio observacional, descriptivo, retrospectivo de enero del 2011 a diciembre del 2019 en el Servicio de Gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen, Lima-Perú. Resultados: Se incluyó 22 pacientes con EII, 17 con EC y 5 con CU. El 59% fueron masculinos. Se realizó cirugía de emergencia en el 35,2% y el 60% de los pacientes con EC y CU respectivamente. La estenosis y el megacolon tóxico fueron las indicaciones más frecuentes. Según el tipo de cirugía, la hemicolectomía (41%) y la resección intestinal (41%) fueron las más frecuentemente realizadas en EC, mientras que, en CU fue la colectomía total (60%). Dentro de las complicaciones postquirúrgicas, las dehiscencias/fistulas y colecciones intraabdominales fueron las más frecuentemente reportadas en EC; mientras que, en CU fue la infección del sitio operatorio e íleo adinámico. Luego de cirugía, los biológicos y los 5-ASA asociados a inmunomodulador fueron el tratamiento más utilizados en EC y CU respectivamente. La mortalidad fue del 17,6% en EC y 60% en CU. Conclusiones: El tratamiento quirúrgico es una opción en el manejo de la EII moderada-severa. La cirugía de emergencia en EII continúa presentando una alta morbimortalidad.

The management of inflammatory bowel disease (IBD) is mainly medical, however, more than 70% of patients with Crohn's disease (CD) and 25% with ulcerative colitis (UC) will require surgery during their lifetime. Objective: To evaluate medical, surgical management and evolution in patients with moderate-to-severe IBD. Materials and methods: Observational, descriptive, retrospective study from January 2011 to December 2019 in the Gastroenterology Service of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. Results: Twenty two patients with IBD, 17 with CD and 5 with UC were included. Male predominance (59%). Emergency surgery was performed in 35.2% and 60% of patients with CD and UC, respectively. Stenosis and toxic megacolon were the most frequent indications. According to the type of surgery, hemicolectomy (41%) and intestinal resection (41%) were the most frequently performed in CD, while in UC it was total colectomy (60%). Among the postoperative complications, dehiscence/fistula and intra-abdominal collections were the most frequently reported in CD; whereas in UC it was surgical site infection and adynamic ileus. After surgery, biologics and 5-ASA associated with immunomodulator were the most used treatment in CD and UC, respectively. Mortality was 17.6% in CD and 60% in UC. Conclusions: Surgical treatment is an option in the management of moderate-to-severe IBD. Emergency surgery in IBD continues to have a high morbidity and mortality rate.

[Epidemiology and phenotype of Crohn's disease in a reference hospital in Lima - Peru]. / Epidemiología y fenotipo de la enfermedad de Crohn en un hospital de referencia en Lima - Perú.

Crohn's disease (CD) is a chronic inflammatory pathology of the digestive tract with great impact on the quality of life of patients. Global epidemiology is changing in recent years and its prevalence is increasing in Latin America. OBJECTIVE: To evaluate the epidemiological characteristics, phenotype, clinical course, diagnosis and treatment of CD. MATERIALS AND METHODS: Retrospective, descriptive, observational study of patients diagnosed with CD from January 2004 to December 2019 in the gastroenterology service of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. RESULTS: 55 patients with an average age of 56 ± 8.2 years were included. Male predominance (62%). The average diagnosis time was 18 ± 6.1 months. The most frequent symptoms were: abdominal pain 72.7% and weight loss 60%. Extraintestinal manifestations occurred in 20%. The ileal location (36.4%) was the most frequent, followed by colonic (32.7%). The inflammatory phenotype predominated in half of the patients, followed by stenosing in 25.5%. The most frequent activity clinical and endoscopic was moderate. For induction and maintenance treatment, systemic corticosteroids and biological therapy with anti-TNF were the most widely used, respectively. Approximately a third of patients required surgery during the evolution of the disease. Mortality was 5.4%. CONCLUSIONS: CD is an increasingly frequent disease in our country, with epidemiological and phenotypic characteristics that differ from other continents.

Significancia clínica de la presencia de granulomas en enfermedad de Crohn / Clinical significance of the presence of granulomas in Crohn's disease

RESUMEN Los granulomas epitelioides son hallazgos característicos de un grupo de pacientes con enfermedad de Crohn (EC), pero su significancia con respecto a la severidad y progresión de la enfermedad es aún incierta. Objetivo: Evaluar las características clínicas de los pacientes con EC en relación a la presencia o no de granulomas en los hallazgos histológicos. Materiales y métodos: Estudio observacional, descriptivo, retrospectivo en pacientes con diagnóstico de EC desde enero 2004 a diciembre 2019 en el servicio de gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen, Lima-Perú. Resultados: Se incluyó 55 pacientes con media de edad de 56 ± 8,2 años. La mediana de tiempo de enfermedad fue de 16 meses. Tener dos o más recaídas al año (p<0,001; OR= 9,75), edad menor a 30 años (p<0,001; OR=3,57) y un CDAI moderado a severo (≥220 puntos, OR= 11,4; p<0,008) se asoció significativamente con la presencia de granulomas. La actividad endoscópica severa (p<0,003; OR= 9,91) y el fenotipo estenosante-penetrante (p<0,001; OR= 22,1) también mostraron asociación con la presencia de granulomas. El grupo de granulomas presentó mayor probabilidad de uso de corticoides (p<0,024; OR= 3,92) e inmunomodulador (p<0,001; OR= 7,10) además de necesidad de cirugía de resección intestinal (p< 0,027; OR: 5,07). Conclusiones: La presencia de granulomas en EC podría asociarse a mayor severidad clínica, endoscópica, requerimiento de terapia inmunosupresora y mayor necesidad de resección intestinal.

ABSTRACT Epithelioid granuloma is a characteristic histological feature of Crohn's disease (CD), but their significance with respect to the severity and progression of the disease is still uncertain. Objective: To evaluate the clinical characteristics of CD patients in relation to the presence or not of granulomas in histological findings. Materials and methods: An observational, descriptive, retrospective study in patients diagnosed with CD from January 2004 to December 2019 in the gastroenterology department of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. Results: 55 patients were included. Average age of 56 ± 8.2 years. The median time of disease was 16 months. Have two or more relapses per year (p<0.001; OR=9.75), age less than 30 years (p<0.001; OR=3.57) and a moderate to severe CDAI (≥220 points, OR=11.4, p <0.008) was significantly associated with the presence of granulomas. Severe endoscopic activity (p <0.003; OR=9.91) and the stenosing-penetrating phenotype (p<0.001; OR=22.1) also showed association with the presence of granulomas. The group of granulomas had a higher probability of corticosteroid use (p <0.024; OR=3.92) and immunomodulator (p <0.001; OR=7.10), besides the need for intestinal resection surgery (p<0.027; OR=5.07). Conclusions: The presence of granulomas in CD may be associated with increased clinical severity, endoscopic severity, immunosuppressive therapy requirement and undergo for intestinal resection.

Epidemiología y fenotipo de la enfermedad de Crohn en un hospital de referencia en Lima Perú / Epidemiology and phenotype of Crohn's disease in a reference hospital in Lima Peru

RESUMEN La enfermedad de Crohn (EC) es una patología inflamatoria crónica del tracto digestivo con gran impacto en la calidad de vida de los pacientes. La epidemiología mundial está cambiando en los últimos años y su prevalencia está aumentando en Latinoamérica. Objetivo: Evaluar las características epidemiológicas, fenotipo, curso clínico, diagnóstico y tratamiento de la EC. Materiales y métodos: Estudio observacional, descriptivo, retrospectivo de pacientes con diagnóstico de EC desde enero 2004 a diciembre 2019 en el servicio de gastroenterología del Hospital Nacional Guillermo Almenara Irigoyen, LimaPerú. Resultados: Se incluyó 55 pacientes, con edad promedio de 56 ± 8,2 años. Predominio masculino (62%). El tiempo promedio de diagnóstico fue de 18 ± 6,1 meses. Los síntomas más frecuentes fueron: dolor abdominal 72,7% y baja de peso 60%. Las manifestaciones extraintestinales se presentaron en 20%. La localización ileal (36,4%) fue la más frecuente, seguida de la colónica (32,7%). Predominó el fenotipo inflamatorio en la mitad de los pacientes, seguido de estenosante en 25,5%. La actividad clínica y endoscópica más frecuente fue moderada. Para el tratamiento de inducción y mantenimiento, los corticoides sistémicos y la terapia biológica con anti-TNF fueron los más utilizados respectivamente. Aproximadamente un tercio de pacientes requirieron cirugía durante la evolución de la enfermedad. La mortalidad fue del 5,4%. Conclusiones: La EC es una enfermedad cada vez más frecuente en nuestro país, con características epidemiológicas y fenotípicas que difieren de otros continentes.

ABSTRACT Crohn's disease (CD) is a chronic inflammatory pathology of the digestive tract with great impact on the quality of life of patients. Global epidemiology is changing in recent years and its prevalence is increasing in Latin America. Objective: To evaluate the epidemiological characteristics, phenotype, clinical course, diagnosis and treatment of CD. Materials and methods: Retrospective, descriptive, observational study of patients diagnosed with CD from January 2004 to December 2019 in the gastroenterology service of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. Results: 55 patients with an average age of 56 ± 8.2 years were included. Male predominance (62%). The average diagnosis time was 18 ± 6.1 months. The most frequent symptoms were: abdominal pain 72.7% and weight loss 60%. Extraintestinal manifestations occurred in 20%. The ileal location (36.4%) was the most frequent, followed by colonic (32.7%). The inflammatory phenotype predominated in half of the patients, followed by stenosing in 25.5%. The most frequent activity clinical and endoscopic was moderate. For induction and maintenance treatment, systemic corticosteroids and biological therapy with anti-TNF were the most widely used, respectively. Approximately a third of patients required surgery during the evolution of the disease. Mortality was 5.4%. Conclusions: CD is an increasingly frequent disease in our country, with epidemiological and phenotypic characteristics that differ from other continents.

[Clinical significance of the presence of granulomas in Crohn's disease]. / Significancia clínica de la presencia de granulomas en enfermedad de Crohn.

Epithelioid granuloma is a characteristic histological feature of Crohn's disease (CD), but their significance with respect to the severity and progression of the disease is still uncertain. OBJECTIVE: To evaluate the clinical characteristics of CD patients in relation to the presence or not of granulomas in histological findings. MATERIALS AND METHODS: An observational, descriptive, retrospective study in patients diagnosed with CD from January 2004 to December 2019 in the gastroenterology department of the Guillermo Almenara Irigoyen National Hospital, Lima-Peru. RESULTS: 55 patients were included. Average age of 56 ± 8.2 years. The median time of disease was 16 months. Have two or more relapses per year (p<0.001; OR=9.75), age less than 30 years (p<0.001; OR=3.57) and a moderate to severe CDAI (≥220 points, OR=11.4, p <0.008) was significantly associated with the presence of granulomas. Severe endoscopic activity (p <0.003; OR=9.91) and the stenosing-penetrating phenotype (p<0.001; OR=22.1) also showed association with the presence of granulomas. The group of granulomas had a higher probability of corticosteroid use (p <0.024; OR=3.92) and immunomodulator (p <0.001; OR=7.10), besides the need for intestinal resection surgery (p<0.027; OR=5.07). CONCLUSIONS: The presence of granulomas in CD may be associated with increased clinical severity, endoscopic severity, immunosuppressive therapy requirement and undergo for intestinal resection.