Intestinal Abnormalities in Patients With SARS-CoV-2 Infection: Histopathologic Changes Reflect Mechanisms of Disease.

Approximately 20% of patients with symptomatic syndrome-associated coronavirus-2 (SARS-CoV-2) infection have gastrointestinal bleeding and/or diarrhea. Most are managed without endoscopic evaluation because the risk of practitioner infection outweighs the value of biopsy analysis unless symptoms are life-threatening. As a result, much of what is known about the gastrointestinal manifestations of coronavirus disease-2019 (COVID-19) has been gleaned from surgical and autopsy cases that suffer from extensive ischemic injury and/or poor preservation. There are no detailed reports describing any other gastrointestinal effects of SARS-CoV-2 even though >3,000,000 people have died from COVID-19 worldwide. The purpose of this study is to report the intestinal findings related to SARS-CoV-2 infection by way of a small case series including one with evidence of direct viral cytopathic effect and 2 with secondary injury attributed to viral infection. Infection can be confirmed by immunohistochemical stains directed against SARS-CoV-2 spike protein, in situ hybridization for spike protein-encoding RNA, and ultrastructural visualization of viruses within the epithelium. It induces cytoplasmic blebs and tufted epithelial cells without inflammation and may not cause symptoms. In contrast, SARS-CoV-2 infection can cause gastrointestinal symptoms after the virus is no longer detected, reflecting systemic activation of cytokine and complement cascades rather than direct viral injury. Reversible mucosal ischemia features microvascular injury with hemorrhage, small vessel thrombosis, and platelet-rich thrombi. Systemic cytokine elaboration and dysbiosis likely explain epithelial cell injury that accompanies diarrheal symptoms. These observations are consistent with clinical and in vitro data and contribute to our understanding of the protean manifestations of COVID-19.

Case Report: Molecular Diagnosis of Cystoisospora belli in a Severely Immunocompromised Patient with HIV and Kaposi Sarcoma.

Diarrhea in an immunocompromised patient has a broad infectious differential. Diagnosis is difficult despite advances in diagnostic modalities. We report a case of a 45-year-old Nigerian woman who immigrated to the United States 2 years ago. She presented to the hospital with gastrointestinal bleeding, newly diagnosed HIV, and disseminated Kaposi sarcoma. During hospitalization, the patient had an onset of watery diarrhea and high eosinophilia. Subsequent stool analysis using multi-parallel real-time quantitative polymerase chain reaction for 13 parasites was positive for Cystoisospora belli. The patient was treated with trimethoprim-sulfamethoxazole, but had relapsed disease when her antibiotics were stopped prematurely. After restarting trimethoprim-sulfamethoxazole, her diarrhea and eosinophilia improved, and she had undetectable Cystoisospora belli DNA on repeat stool quantitative polymerase chain reaction. This case highlights the importance of a thorough workup for diarrhea, including parasites, especially for immunocompromised patients. Antibiotic prophylaxis is recommended in patients with Cystoisospora belli and HIV/AIDS.

Eosinophilic colitis: an infrequent disease with difficult diagnose.

Eosinophilic colitis (EC) is a rare entity. It is part of eosinophilic gastroenteritis, a rare inflammatory disorder characterised by eosinophilic infiltration of tissues that can affect any segment of the digestive tract. The diagnosis is established by the presence of an increased eosinophilic infiltrate in the colon wall in symptomatic patients. There is no characteristic clinical picture of EC. It can be associated with abdominal pain, changes in bowel movements, diarrhoea and rectal bleeding. Biopsies are mandatory if EC is suspected and despite visualising a normal mucosa. Although there are no protocol guidelines in this regard, steroid treatment is the first option in controlling the disease. Increasing the knowledge of clinicians and pathologists of this disorder and the recording its real incidence and population impact, could improve the understanding and treatment of the disease.

Fatal nosocomial hemorrhagic enterocolitis probably caused by adenovirus. Report of one case.

Adenovirus (ADV) is a recognized cause of severe disease among immunocompromised patients. We report a previously healthy 39-year-old female, admitted with influenza pneumonia and evolving with lung hemorrhage and acute renal failure requiring mechanical ventilation and hemodialysis. She received high corticosteroid doses due to an initial suspicion of alveolar hemorrhage. Lymphopenia already present before steroid use (567/µL), was maintained during the whole hospital stay (mean 782/µL). From the second week of admission she presented a high-volume diarrhea (mean 2.5 L/day) associated to intermittent bloody stools. An ulcerative enterocolitis was confirmed by CT images and colonoscopy. ADV was detected in a colonic tissue sample by real time PCR but not by a commercial filmarray test. Cidofovir-probenecid and racecadotril therapy were indicated without changing the clinical course of diarrhea and the patient finally died.

Fatal nosocomial hemorrhagic enterocolitis probably caused by adenovirus: report of one case / Enterocolitis hemorrágica fatal nosocomial probablemente causada por adenovirus: informe de un caso

ABSTRACT Adenovirus (ADV) is a recognized cause of severe disease among immunocompromised patients. We report a previously healthy 39-year-old female, admitted with influenza pneumonia and evolving with lung hemorrhage and acute renal failure requiring mechanical ventilation and hemodialysis. She received high corticosteroid doses due to an initial suspicion of alveolar hemorrhage. Lymphopenia already present before steroid use (567/μL), was maintained during the whole hospital stay (mean 782/μL). From the second week of admission she presented a high-volume diarrhea (mean 2.5 L/day) associated to intermittent bloody stools. An ulcerative enterocolitis was confirmed by CT images and colonoscopy. ADV was detected in a colonic tissue sample by real time PCR but not by a commercial filmarray test. Cidofovir-probenecid and racecadotril therapy were indicated without changing the clinical course of diarrhea and the patient finally died.

Adenovirus (ADV) es una causa reconocida de enfermedades graves en pacientes inmunocomprometidos. Informamos el caso de una mujer de 39 años, previamente sana, que ingresó por neumonía grave por influenza, evolucionando con hemorragia pulmonar y falla renal aguda, requiriendo ventilación mecánica y hemodiálisis. Recibió altas dosis de corticoides por la sospecha inicial de una hemorragia alveolar. Tuvo linfopenia durante toda su estadía (promedio 782/μL), la que ya estaba presente antes del uso de los corticoides (567/μL). Desde la segunda semana de hospitalización, presentó una diarrea de alto volumen (promedio 2,5 L/día) asociada a la presencia de sangre en deposiciones en forma intermitente. Se confirmó una enterocolitis ulcerativa por tomografía computada y colonoscopía. Se detectó ADV en muestras de biopsia colónica por PCR en tiempo real pero no por un test de PCR múltiples automatizado comercial. Fue tratada con cidofovir-probenecid y racecadrotrilo sin impacto clínico y la paciente finalmente falleció.

Histologic Features of Gastrointestinal Tract Biopsies in IgA Vasculitis (Henoch-Schönlein Purpura).

Immunoglobulin A (IgA) vasculitis or Henoch-Schönlein purpura (HSP) typically occurs in the pediatric population, although rare cases also occur in adults. Gastrointestinal (GI) involvement is common. The "classic" histologic finding in IgA vasculitis (HSP) is leukocytoclastic vasculitis (LCV); other histologic features in biopsies of IgA vasculitis (HSP) have only been rarely described. The pathology archival files at our institution were searched for GI biopsies from patients with IgA vasculitis (HSP). Slides were retrieved and histologic and clinical features were reviewed. We identified 16 patients with IgA vasculitis (HSP) with a GI biopsy series, including both adult and pediatric patients. The most common histologic abnormality was lamina propria hemorrhage (all cases) with many cases also showing lamina propria fibrin deposition with red cell sludging and nuclear debris (7 cases). Twelve of the 16 duodenal biopsies had acute duodenitis; 3 of which were severe and erosive. Several also had an eosinophilic infiltrate. Seven of the 9 jejunal and/or ileal biopsies had acute jejunitis or ileitis. An acute colitis or proctitis was observed in 9/12 colorectal biopsies. Four biopsies contained LCV; in each of these cases, the involved vessels were small capillaries within the lamina propria. Only 1 biopsy contained deeper submucosal vessels, but they were uninvolved. Sites involved by LCV included the colorectum (2 cases), colorectum and terminal ileum, terminal ileum only, duodenum, and jejunum (1 case each). All patients presented with abdominal pain; 13/16 developed a rash, 1 following the index biopsy. Other presenting symptoms included diarrhea and/or hematochezia (8 cases), nausea/vomiting (5 cases), and intussusception (1 case). Four patients had concurrent skin biopsies showing LCV; only 1 of these patients had LCV on GI biopsy. Indications for biopsy included nonspecific presenting symptoms, absence of rash at presentation, and/or failure to respond adequately to steroid therapy. Biopsies are commonly performed in patients with or without suspected IgA vasculitis (HSP) to rule out infection, inflammatory bowel disease, and less commonly, vasculitis. In general, vasculitis is not commonly observed in GI biopsies of patients with IgA vasculitis (HSP), and the spectrum of findings includes neutrophilic infiltrate within the small bowel and colon, with the duodenum most commonly affected. While the clinical and histologic findings may mimic early inflammatory bowel disease, the presence of predominant small bowel involvement, especially erosive duodenitis, should raise suspicion for IgA vasculitis (HSP). Biopsies should be obtained before steroid therapy is initiated, if possible.

[Immunological balance of CD8+CD28+/CD8+CD28- T lymphocytes can predict gastrointestinal hemorrhage in patients with inflammatory bowel disease].

OBJECTIVE: To evaluate the sensitivity and specificity of CD8+CD28+/CD8+CD28- T lymphocyte balance in predicting the gastrointestinal hemorrhage (GH) in patients with inflammatory bowel disease (IBD). METHODS: Forty-nine IBD patients, including 30 with ulcerous colitis (UC) and 19 with Crohn's disease (CD), were enrolled to test peripheral blood CD8+CD28+ and CD8+CD28- T cells using flow cytometry. All the patients were followed up for one year. The receiver-operating characteristic (ROC) curves were used to test the efficiency of CD8+CD28+/CD8+CD28- T lymphocyte balance to predict GH. The differences in lasting time of remission (LTR) under different factors were compared using Kaplan-Meier survival analysis, and the correlation between CD8+ T lymphocytes and the factors were analyzed. RESULTS: The utilization rates of immunosuppressant, steroids, and biological agent (BA) were significantly higher in CD patients than in UC patients (P=0.003, 0.043 and 0.002, respectively). The frequencies of CD8+CD28+T cells were obviously higher in UC patients than those in CD patients (t=3.022, P=0.004). CD8+CD28+T cells, CD8+CD28- T cells, and especially CD8+CD28+/CD8+CD28- ratio (area under curve of 0.977, P=0.000; cut-off value of 1.14 [13.95%/12.24%] with a sensitivity of 93.3% and a specificity of 91.2%) showed good efficiencies in predicting GH (P<0.01). The mean and median of LTR of IBD patients who did not receive BA or surgical treatment were significantly longer (Χ2=9.730, P=0.002; Χ2=15.981, P=0.000). CD8+CD28+/CD8+CD28- ratio was significantly related to both BA (P=0.009) and surgery (P=0.038). CONCLUSION: Both decreased CD8+CD28+T cells and elevated CD8+CD28-T cells are closely correlated with GH, and their ratio can predict the occurrence of GH with a high sensitivity and specificity and is correlated with BA and surgery at the cut-off value of 1.14.

Deposiciones sanguinolentas iniciadas en los primeros días de vida / Bloody stools started in the first days of life

La proctocolitis alérgica del lactante es un trastorno caracterizado por la presencia de deposiciones mucosanguinolentas en los dos primeros meses de vida, pudiendo aparecer en los primeros días de vida. Anteriormente, relacionado con niños alimentados con lactancia artificial, en los últimos años se observa un aumento de la incidencia en niños alimentados con lactancia materna exclusiva debido al paso de proteínas de leche de vaca a la leche de la madre. Recién nacido a término, alimentado con lactancia materna exclusiva inicia a los dos días de vida deposiciones con hebras de sangre de forma intermitente. Todos los estudios realizados resultan normales. La clínica mejora progresivamente tras la eliminación de las proteínas de leche de vaca de la dieta de la madre, por lo que se diagnostica de proctocolitis alérgica. El diagnóstico se basa en la clínica, la desaparición de los síntomas al retirar las proteínas de leche de vaca de la dieta, y en la reaparición de éstos al reintroducirla. Los niños mantienen buen estado general en todo momento, siendo la gran mayoría tolerantes a la leche de vaca al año de vida, por lo que se considera una patología de buen pronóstico.

Allergic colitis is a pathology characterized by blood in faeces, appeared in first two months of life, but it can also appear during the first days of life. Previously it was related with children with non breastfeeding, however in the last years incidence is increasing in children with breastfeeding. This is explained with the presence of cow’s milk proteins in human’s milk. Newborn term, fed with exclusive breastfeeding, starts at second day of life blood in faeces, intermittently. The studies done are normal. The symptoms improve progressively after removing the cow’s milk proteins of the mother’s diet, so the child is diagnosed with allergic protocolitis. Diagnosis is based on the symptoms, the improvement with the removal of the cow’s milk protein of the diet, and the worsening when they are reintroduced. The children conserve good general condition every moment, almost all of them tolerate cow’s milk when they are one year old, and so it is considered pathology of well prognosis.

A case of multiple recurrence of Clostridium difficile infection with severe hematochezia in an immunocompromised host.

Clostridium difficile infection (CDI) is increasing in incidence and severity. Clinically, diarrhea frequently occurs, but severe hematochezia is rarely seen with CDI. We describe here a hematopoietic stem cell transplantation (HSCT) recipient who experienced life-threatening gastrointestinal bleeding due to severe CDI. Subsequent stool surveillance and molecular typing observed the patient who had two episodes of recurrence with a new strain of C. difficile distinct from the initial infection. We analyze C. difficile strains obtained from the patient, and also discuss the diagnosis and treatment of this case.

Short article: Etiologic profile and endoscopic findings in immunocompromised children and adolescents with gastrointestinal bleeding.

BACKGROUND: Gastrointestinal bleeding (GIB) is one of the potential causes of increased morbidity and mortality in immunocompromised patients, but data on characteristics of GIB in immunocompromised children are sparse. OBJECTIVES: This study aimed to identify the etiology, endoscopic, and histologic findings of GIB in immunocompromised children. DESIGN: This was a retrospective descriptive study. PATIENTS: We identified 33 patients (aged<20 years) and 45 GIB episodes related to GIB between January 2007 and April 2015 from a tertiary care and teaching hospital. RESULTS: The mean age at endoscopy was 10.7±4.6 years. Most common indications for endoscopy were melena in upper GIB and hematochezia in lower GIB. The median delay of duration between GIB presentation to endoscopy was 3 days. All except one child had at least one endoscopic abnormality. The most common cause of upper GIB was cytomegalovirus (CMV)-related gastrointestinal disease (35%), followed by esophageal varices (26%), and the most common cause of lower GIB was CMV-related gastrointestinal disease (55%). Fourteen percent of patients died during upper GIB episodes and 15% died during lower GIB episodes. CONCLUSION: Among immunocompromised individuals aged younger than 20 years presenting with GIB, CMV-related gastrointestinal disease is the most prevalent in our study population. However, the etiology of immunocompromised state needs to be taken into consideration when evaluating these children presenting with GIB.

Assessment of disease activity by fecal immunochemical test in ulcerative colitis.

AIM: To evaluate the efficacy of quantitative fecal immunochemical test (FIT) as biomarker of disease activity in ulcerative colitis (UC). METHODS: Between February 2013 and November 2014, a total of 82 FIT results, obtained in conjunction with colonoscopies, were retrospectivelyevaluated for 63 patients with UC. The efficacy of FIT for evaluation of disease activity was compared to colonoscopic findings. Quantitative fecal blood with automated equipment examined from collected feces. Endoscopic disease severity were assessed using the Mayo endoscopic subscore (MES) classification. The extent of disease were classified by proctitis (E1), left sided colitis (E2), and extensive colitis (E3). Clinical activity were subgrouped by remission or active. RESULTS: All of 21 patients with MES 0 had negative FIT (< 7 ng/mL), but 22 patients with MES 2 or 3 had a mean FIT of > 134.89 ng/mL. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of negative FIT about mucosal healing were 73.33%, 81.82%, 91.49%, 51.43% and 73.17%, respectively. The sensitivity, specificity, PPV, NPV and accuracy of predictive value of positive FIT (cutoff value > 100 ng/mL) about active disease status were 45.45%, 93.33%, 71.43%, 82.35% and 26.83%, respectively. Among patients with clinical remission, FIT was negative in 31 (81.6%) of 38 cases, with a mean fecal hemoglobin concentration of 6.12 ng/mL (range, negative to 80.9 ng/mL) for this group of patients. Among patients with clinical active disease, FIT was negative in 16 (36.4%) out of 44 cases, with a mean fecal hemoglobin concentration > 167.4 ng/mL for this group of patients. FIT was positively correlated with endoscopic activity (r = 0.626, P < 0.01) and clinical activity (r = 0.496, P < 0.01). But, FIT did not correlate with the extent of disease (r = -0.047, P = 0.676). CONCLUSION: Quantitative FIT can be a non-invasive and effective biomarker for evaluation of clinical and endoscopic activity in UC, but not predict the extent of disease.

Gastrointestinal mucormycosis in immunocompromised hosts.

Invasive mucormycosis is a rare fungal infection in immunocompromised hosts, but it carries a high mortality rate. Primary gastrointestinal disease is the least frequent form of presentation. Early diagnosis and treatment are critical in the management; however, symptoms are typically non-specific in gastrointestinal disease, leading to delayed therapy. To describe the clinical presentation, diagnosis, treatment and outcomes of gastrointestinal mucormycosis in immunocompromised hosts, we reviewed all cases of primary gastrointestinal mucormycosis in immunocompromised hosts reported in English literature as well as in our Institution from January 1st 1991 to December 31st 2013 for a total of 31 patients. About 52% of patients underwent solid organ transplant (SOT), while the rest had an underlying haematologic malignancy. Abdominal pain was the most common presenting symptom, followed by gastrointestinal bleeding and fever. Gastric disease was more common in SOT, whereas those with haematologic malignancy presented with intestinal disease (P = 0.002). Although gastrointestinal mucormycosis remains an uncommon condition in immunocompromised hosts, it carries significant morbidity and mortality, particularly in cases with intestinal involvement. A high index of suspicion is of utmost importance to institute early and appropriate therapy and improve outcomes.

Histologic Features of Intestinal Thrombotic Microangiopathy in Pediatric and Young Adult Patients after Hematopoietic Stem Cell Transplantation.

High-risk transplantation-associated thrombotic microangiopathy (TMA) can present with multisystem involvement and is associated with a poor outcome after hematopoietic stem cell transplantation (HSCT), with < 20% 1-year survival. TMA may involve the intestinal vasculature and can present with bleeding and ischemic colitis. There are no established pathologic criteria for the diagnosis of intestinal TMA (iTMA). The goal of our study was to identify histologic features of iTMA and describe associated clinical features. We evaluated endoscopic samples from 50 consecutive HSCT patients for 8 histopathologic signs of iTMA and compared findings in 3 clinical groups based on the presence or absence of systemic high-risk TMA (hrTMA) and the presence or absence of clinically staged intestinal graft-versus-host disease (iGVHD): TMA/iGVHD, no TMA/iGVHD, and no TMA/no iGVHD. Thirty percent of the study subjects had a clinical diagnosis of systemic hrTMA. On histology, loss of glands, intraluminal schistocytes, intraluminal fibrin, intraluminal microthrombi, endothelial cell separation, and total denudation of mucosa were significantly more common in the hrTMA group (P < .05). Intravascular thrombi were seen exclusively in patients with hrTMA. Mucosal hemorrhages and endothelial cell swelling were more common in hrTMA patients but this difference did not reach statistical significance. Patients with hrTMA were more likely to experience significant abdominal pain and gastrointestinal bleeding requiring multiple blood transfusions (P < .05). Our study shows that HSCT patients with systemic hrTMA can have significant bowel vascular injury that can be identified using defined histologic criteria. Recognition of these histologic signs in post-transplantation patients with significant gastrointestinal symptoms may guide clinical decisions.

Catastrophic gastrointestinal complication of systemic immunosuppression.

We present a case of acute upper gastrointestinal haemorrhage in a patient with systemic vasculitis immunosuppressed on cyclophosphamide and prednisolone. The patient presented with a diffuse haemorrhagic oesophagitis and a non-specific duodenitis. Biopsies taken from the oesophagus and duodenum demonstrated infection with herpes simplex virus (HSV) and cytomegalovirus (CMV) respectively. Viral infection of the upper gastrointestinal tract is a recognised complication of immunosuppression and HSV is one of the most common pathogens. CMV on the other hand most commonly causes a colitis or less commonly oesophagitis. CMV enteritis is rare as is the synchronous infection with two viral agents in an immunocompromised patient having being described in a few case series only. Viral infection of the gastrointestinal tract in immunocompromised patients should be treated with systemic anti-viral medication and consideration to withdrawal of the immunosuppressive therapy if possible and appropriate. The authors highlight the need for a high suspicion of viral infection in immunosuppressed patients presenting with upper gastrointestinal bleeding.

Características clínicas asociadas a colitis eosinofílica en lactantes con rectorragia persistente / Clinical features of Eosinophilic Colitis in infants with persistent rectal bleeding

The most common presentation of cow's milk protein allergy (CMP) in infants is known as eosinophilic colitis (EC). The aim of this study is to evaluate EC characteristics in infants evaluated with colonoscopy due to the presence of rectorrhagia. Patients and Methods: A retrospective case-control study. Left-sided colonoscopy records of infants with persistent rectal bleeding, conducted between January 2006 and March 2011, were reviewed. The cases corresponded to infants with rectal biopsy compatible with EC and controls with negative biopsy. Telephone questionnaires to parents were conducted, evaluating personal and family history. Results: Complete records were obtained in 61 (79%) of the 77 procedures. 33 (54%) of them were males. Examination average age was 6.3 ± 5.9 months. 25 (41%) patients had EC on their histology. Between cases and controls, no significant difference in gestational age, birth weight and gender, only regarding age at the time of rectal bleeding, were observed. There was also no difference in personal history regarding obstructive bronchitis, allergic rhinitis, family history of asthma, allergic rhinitis or other food allergies. Those who received artificial feeding did not presented greater risk of EC. The most common symptoms in the cases did not differ significantly from the controls. Conclusions: The prevalence of EC in the children studied was 40.9%. Our results show that there are groups of patients with persistent rectal bleeding in which there is no personal or family history that helps diagnosing EC. An endoscopic study could be considered in these patients to establish a correct diagnosis of this condition, avoid unnecessary diets and not to delay the detection of other diseases.

En lactantes, la forma de presentación más común de la alergia a la proteína de la leche de vaca (PLV) es la colitis eosinofílica (CE). El objetivo de este trabajo es evaluar características clínicas asociadas a CE en lactantes evaluados con colonoscopía por la presencia de rectorragia. Pacientes y Método: Estudio caso-control, retrospectivo. Se revisaron registros de colonoscopía izquierda de lactantes con rectorragia persistente, realizadas entre Enero 2006 y Marzo 2011. Casos fueron lactantes con rectorragia y biopsia compatible con CE y controles aquellos con biopsia negativa. Se realizó un cuestionario vía telefónica a los padres, evaluándose antecedentes personales y familiares. Resultados: En 61 (79%) de 77 procedimientos se obtuvo registros completos. 33 (54%) eran hombres. Edad promedio del examen fue 6,3 ± 5,9 meses. 25 (41%) pacientes presentaron CE en la histología. Sin diferencia significativa en edad gestacional, peso de nacimiento ni sexo, pero si en edad de presentación de la rectorragia, entre casos y controles. Tampoco hubo diferencia en antecedentes personales de bronquitis obstructivas, rinitis alérgica, ni antecedentes familiares de asma, rinitis alérgica u otras alergias alimentarias. Quienes recibieron lactancia artificial no tuvieron mayor riesgo de CE. Los síntomas más frecuentes en los casos no se diferenciaron significativamente de los controles. Conclusión: La prevalencia de CE en los niños estudiados fue de 40,9%. Nuestros resultados muestran que hay grupos de pacientes con rectorragia persistente en los cuales no existen antecedentes de la historia familiar ni personal que permitan establecer el diagnóstico de CE. Es en estos pacientes en los cuales podría considerarse el estudio endoscópico para establecer un correcto diagnóstico de esta patología, evitar dietas innecesarias y no retrasar la detección de otras enfermedades.

Immunoglobulin G4-related periaortitis complicated by aortic rupture and aortoduodenal fistula after endovascular AAA repair.

PURPOSE: To report a rare and complicated case of immunoglobulin (Ig) G4-related periaortitis involving both the aortic wall and the retroperitoneum without aneurysmal formation. CASE REPORT: A 79-year-old man with IgG4-related periaortitis suffered aortic rupture despite a normal caliber aorta after 6 months of steroid therapy (20 mg/d). Endovascular repair with an aortic cuff sealed the rupture. Steroid therapy was halted 2 weeks later due to infection. Four months later, a biopsy during esophagogastroduodenoscopy to investigate gastrointestinal bleeding suggested a relapse of IgG4-RD in the duodenum. Subsequent aortoduodenal fistula formation proved fatal. Generally, IgG4-related periaortitis does not result in such complications due to the absence of aneurysm formation and a thick aortic wall. CONCLUSIONS: Our report highlights a rare case of IgG4-related periaortitis where complications resulted following steroid therapy and surgical intervention, emphasizing the difficulties in dealing with IgG4-related cardiovascular lesions.