Eosinophilic Jejunitis Presenting as Intractable Vomiting, Persistent Leukocytosis, and Ascites in a Young Adult Patient.

Eosinophilic enteritis (EoN) poses a distinctive challenge, affecting individuals with various clinical presentations depending on the layer and extent of the bowel wall. We present a case of a 19-year-old female with abdominal pain, vomiting, and loose stools for 1 month. Labs were significant for persistent leukocytosis with peripheral eosinophilia. A computed tomography of the abdomen/pelvis demonstrated moderate abdominal ascites and moderately diffuse mucosal thickening of jejunal loops. A diagnostic paracentesis unveiled low serum ascites albumin gradient and 92% eosinophils. Push enteroscopy resulted in no significant biopsy findings, though a laparoscopic full-thickness jejunal biopsy exhibited increased eosinophils in the bowel wall. Intravenous steroid, proton pump inhibitor, and dietary changes resolved the symptoms and normalized the labs within a week. Our case report highlights a variable presentation of eosinophilic jejunitis uncommon in this disease population. EoN is an easily missed diagnosis and mandates frequent follow-up to prompt relevant investigations. Atopic clinical features are not prevalent in each case. While rare, EoN requires a strong clinical suspicion, even if endoscopic biopsies are unremarkable, prompting timely laparoscopic full-thickness biopsy. Per protocol, physicians must do the infectious and eosinophilia workup to rule out other etiologies. Our case also highlights that worsening clinical condition in EoN warrants early intravenous steroids with a favorable prognosis and considers a psychosocial aspect of the disease on the patient's health.

Refractory Celiac Disease Type II: A Case Report and Literature Review.

We present an unusual case of 68-year-old male, who presented with acute abdomen, ulcerative jejunitis with perforation, and 2 months later with perforation of the sigmoid colon. We will also discuss difficulties in the delay in diagnosis of refractory celiac disease (RCD), specifically the atypical presentation, multiple surgeries, the consecutive failure of distinct therapeutic options, and multiple complications that occurred within the 3 months since first presentation.

Hemorragia digestiva y enfermedad celíaca complicada en paciente anticoagulado / Gastrointestinal bleeding and complicated celiac disease in anticoagulated patient

Resumen La enfermedad celíaca tiene una considerable frecuencia en nuestro medio. La mayoría de los pacientes presenta mejoría clínica, serológica y endoscópica al poco tiempo de iniciada la dieta libre de gluten. Un muy bajo porcentaje puede presentar o desarrollar una "enfermedad celíaca complicada", entidad que comprende el esprue refractario, la yeyunitis ulcerativa y el linfoma intestinal, que conllevan pronósticos desfavorables, con requerimiento de tratamientos más radicales. Presentamos aquí el caso de un paciente de 77 años evaluado en nuestro centro, que ingresó para estudio de hemorragia digestiva aguda y se realizó finalmente diagnóstico de enfermedad celiaca complicada, requiriendo inicio de tratamiento con corticoides sistémicos y seguimiento estrecho ambulatorio.

Abstract Celiac disease is considerably frequent in our media. Gluten-free diet shows clinical, serological and endoscopic improvement in most patients shortly after its start. A few patients may present or develop a "complicated celiac disease", an entity that includes refractory sprue, ulcerative jejunitis and intestinal lymphoma, which carry unfavorable prognoses, requiring more radical treatments. We present here the case of a 77-year-old male patient evaluated in our center, who was admitted for study of acute gastrointestinal bleeding. Complicated celiac disease was diagnosed, systemic corticosteroids were started and a close follow-up was carried out.

[Gastrointestinal bleeding and complicated celiac disease in anticoagulated patient]. / Hemorragia digestiva y enfermedad celíaca complicada en paciente anticoagulado.

Celiac disease is considerably frequent in our media. Gluten-free diet shows clinical, serological and endoscopic improvement in most patients shortly after its start. A few patients may present or develop a "complicated celiac disease", an entity that includes refractory sprue, ulcerative jejunitis and intestinal lymphoma, which carry unfavorable prognoses, requiring more radical treatments. We present here the case of a 77-year-old male patient evaluated in our center, who was admitted for study of acute gastrointestinal bleeding. Complicated celiac disease was diagnosed, systemic corticosteroids were started and a close follow-up was carried out.

La enfermedad celíaca tiene una considerable frecuencia en nuestro medio. La mayoría de los pacientes presenta mejoría clínica, serológica y endoscópica al poco tiempo de iniciada la dieta libre de gluten. Un muy bajo porcentaje puede presentar o desarrollar una "enfermedad celíaca complicada", entidad que comprende el esprue refractario, la yeyunitis ulcerativa y el linfoma intestinal, que conllevan pronósticos desfavorables, con requerimiento de tratamientos más radicales. Presentamos aquí el caso de un paciente de 77 años evaluado en nuestro centro, que ingresó para estudio de hemorragia digestiva aguda y se realizó finalmente diagnóstico de enfermedad celiaca complicada, requiriendo inicio de tratamiento con corticoides sistémicos y seguimiento estrecho ambulatorio.

Geographic Disparities in Clinical Characteristics of Duodenitis-Proximal Jejunitis in Horses in the United States.

Duodenitis-proximal jejunitis (DPJ) is an idiopathic and potentially fatal disease of horses characterized by abdominal pain, proximal intestinal inflammation, and subsequent gastric and small intestinal fluid accumulation. Although this disease is known to be costly and life threatening in the equine industry, the severity of clinical signs can vary widely, and an exact etiology has yet to be elucidated. This study looked to identify differences in clinical parameters of horses with DPJ between geographic regions in an effort to corroborate anecdotal reports and support theories of differing etiologies. Case records were compared from veterinary academic referral hospitals in three different geographic locations in the United States to determine if significant differences in clinical, clinicopathologic, and prognostic characteristics exist among horses with DPJ. Clinical measurements on presentation that were significantly different between regions included heart rate, peritoneal total protein, albumin, anion gap, aspartate aminotransferase, gamma-glutamyl transferase, sodium, chloride, potassium, and creatinine. Duration of hospitalization and maximum body temperature while hospitalized were also different between regions. There were no significant differences in peritoneal cell count, total white blood cell count, neutrophil count, band neutrophils, calcium, total plasma protein, temperature on presentation, duration of reflux, total reflux volume, or age between hospitals. The mortality rates between hospitals were not significantly different. Increased severity of clinical signs and biochemical abnormalities were identified in the Southeastern United States hospital compared with the Northeastern and Western hospitals. A prospective, multicenter case-control study could identify risk factors contributing toward regional differences in this disease in the future.

Refractory Celiac Disease.

PURPOSE OF REVIEW: To review the epidemiology, pathophysiology, diagnosis, management, and prognosis of refractory celiac disease, with a specific emphasis on recent literature. RECENT FINDINGS: While the pathophysiology of type I refractory celiac disease remains unclear, there have been advances in the understanding of the pathophysiology of type II refractory celiac disease. This has included recognition of the significant role of interleukin-15 and somatic mutations in JAK1 or STAT3 in the proliferation of aberrant T cells. This in turn has led to potential novel therapies targeting these factors, one of which has reached the clinical trial stage. The morbidity and mortality associated with type II refractory celiac disease remain significant; however, recent advances in the understanding of the pathophysiology of this condition have led to potential therapeutic options that should be investigated.

Histopathology of Celiac Disease. Position Statements of the Italian Group of Gastrointestinal Pathologists (GIPAD-SIAPEC).

Celiac Disease (CeD) is an immune-mediated inflammatory disorder of the small intestine, affecting genetically susceptible individuals when exposed to gluten. Small intestinal biopsy interpretation has been the "gold standard" for celiac disease (CeD) for over 50 years. Despite today's availability of sensitive and specific serological tests, the histopathological features from mucosal biopsy play a key role in diagnosing when CeD is suspected. Such a diagnostic approach requires a multidisciplinary team to optimize both tissue sampling and interpretation via the interaction between the pathologist and the gastroenterologist. Pathologists of the Italian Group of Gastrointestinal Pathology (GIPAD-SIAPEC), together with a member (TR) of the Italian Society of Technicians (AITIC) and an expert gastroenterologist (CC), provide position statements as a practical tool for reading and interpreting the report. Moreover, a position statement was formulated about the recently described condition known as Non-Celiac Gluten Sensitivity (NCGS). Within such a diagnostic setting, both the architectural abnormalities of the duodenal mucosa, namely glandular hyperplasia, and villous atrophy and the number of intraepithelial T-lymphocytes should be well highlighted. Ancillary tests such as anti-CD3 stain are useful for an accurate count of the intraepithelial T lymphocytes when CeD or NCGS is suspected. Moreover, anti-CD3 and anti-CD8 stains are recommended in patients not responding to the gluten-free diet (GFD) to confirm a diagnosis of Refractory Celiac Disease (RCeD). Diagnostic clues about the differential diagnosis of both CeD and RCeD have also been rendered.

Late Relapse of Henoch-Schönlein Purpura in an Adolescent Presenting as Severe Gastroduodenitis.

Henoch-Schönlein purpura is a systemic vasculitis, commonly affecting children. Gastrointestinal manifestations are observed in 50-75% of patients; it is well known they may occur before skin lesions in about 20% of cases during the first vasculitic episode. Relapses occur in about one third of patients, typically within 4 months from the initial presentation and with milder symptoms. We report the case of a 17-year old girl with an atypical relapse of Henoch-Schönlein purpura, presenting with acute abdominal symptoms 5 years after the first episode. Esophagogastroduodenoscopy showed duodenal multiple hyperemic and hemorrhagic lesions. To our knowledge this is the first case of hemorrhagic-erosive duodenitis representing a relapse of Henoch-Schönlein purpura occurring several years after the initial episode. Duodenojejunal inflammation should be considered as primary manifestation of Henoch-Schönlein purpura, not only during the first episode, but also in relapses. Endoscopy can be helpful for differential diagnosis, especially in patients with atypical manifestations. Further studies are needed to evaluate risk factors for Henoch-Schönlein purpura recurrence and the possible role of fecal calprotectin as an early marker for gastrointestinal involvement.

Transient ischemic jejunitis due to symptomatic isolated superior mesenteric artery dissection: case report and review of literature.

Acute abdominal pain is one of the most common conditions encountered in the emergency department. The differential diagnosis of acute abdominal pain is extensive and identifying the underlying etiology can be challenging. We report a case of acute transient ischemic jejunitis due to symptomatic isolated superior mesenteric artery dissection in a patient with no cardiovascular risk factors or autoimmune diseases. Symptomatic isolated superior mesenteric artery dissection is a rare cause of acute abdominal pain usually treated in the surgical department. The patient had criteria for conservative treatment and rapidly recovered. We highlight a rare condition which should be taken into account for the differential diagnosis of acute abdominal pain.

Cross-sectional imaging in refractory celiac disease.

PURPOSE: The purpose of this study is to identify unique imaging findings of refractory celiac disease (RCD) including Type I RCD, Type II RCD versus healed celiac disease (CD). METHODS: A retrospective study of patients with known CD and refractory symptoms with cross-sectional imaging was performed. We included patients who underwent T cell receptor rearrangement or T-cell immunophenotyping studies on small bowel (SB) biopsies to classify patients into: healed CD, Type I RCD, or Type II RCD. GI radiologists performed a blinded review of the imaging studies. RESULTS: One-hundred eighteen patients (32 healed; 67 Type I RCD; 19 Type II RCD) were included (mean age 53 ± 6 years; 62% female). The presence of any fold pattern abnormality was more likely to be found in Type II and Type I RCD than healed CD (53% vs. 43% vs.16%; p = 0.009). Type II RCD patients were more likely than Type I RCD and healed CD to have imaging findings of ulcerative jejunitis (26% vs. 6% vs. 3%; p = 0.009), SB wall thickening (37% vs. 16% vs. 0%; p = 0.002) and SB dilation (26% vs. 7% vs. 6%; p = 0.04). Type II RCD demonstrated non-significant trends for decreased number of jejunal folds only, SB mass, mesenteric lymphadenopathy, localized peri-mural edema, and intramural duodenal edema. CONCLUSIONS: Fold pattern abnormalities, ulcerative jejunitis, SB wall thickening, and SB dilation are more likely to be identified in cross-sectional imaging of RCD than healed CD. SB dilatation and ulcerative jejunitis are more likely to be found in Type II than Type I RCD.

Approach to patients with refractory coeliac disease.

Refractory coeliac disease (RCD) is a recognised complication, albeit very rare, of coeliac disease (CD). This condition is described when individuals with CD continue to experience enteropathy and subsequent or ongoing malabsorption despite strict adherence to a diet devoid of gluten for at least 12 months and when all other causes mimicking this condition are excluded. Depending on the T-cell morphology and T-cell receptor (TCR) clonality at the ß/γ loci, RCD can be subdivided into type 1 (normal intra-epithelial lymphocyte morphology, polyclonal TCR population) and type 2 (aberrant IELs with clonal TCR). It is important to differentiate between the two types as type 1 has an 80% survival rate and is managed with strict nutritional and pharmacological management. RCD type 2 on the other hand has a 5-year mortality of 50% and can be complicated by ulcerative jejunitis or enteropathy-associated T-cell lymphoma (EATL). Management of RCD type 2 has challenged many experts, and different treatment approaches have been adopted with variable results. Some of these treatments include immunomodulation with azathioprine and steroids, methotrexate, cyclosporine, alemtuzumab (an anti CD-52 monoclonal antibody), and cladribine or fludarabine sometimes with autologous stem cell transplantation. In this article, we summarise the management approach to patients with RCD type 2.

Paroxysmal Nocturnal Hemoglobinuria Presenting as Recurrent Jejunitis / 대한내과학회지

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disorder characterized by chronic complement-mediated intravascular hemolysis, nocturnal hemoglobinuria, predisposition to thrombosis and secondary bone marrow failure. Small bowel ischemia is a complication of PNH but has not been reported to date in a Korean adult. We report here a case of PNH presenting as recurrent jejunitis. Despite the uncommon etiology, PNH should be considered in the differential diagnosis of patients with unexplained recurrent jejunitis.

Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience.

Refractory coeliac disease (RCD) is a rare complication of coeliac disease (CD) and involves malabsorption and villous atrophy despite adherence to a strict gluten-free diet (GFD) for at least 12 months in the absence of another cause. RCD is classified based on the T-cells in the intra-epithelial lymphocyte (IEL) morphology into type 1 with normal IEL and type 2 with aberrant IEL (clonal) by PCR (polymerase chain reaction) for T cell receptors (TCR) at the ß/γ loci. RCD type 1 is managed with strict nutritional and pharmacological management. RCD type 2 can be complicated by ulcerative jejunitis or enteropathy associated lymphoma (EATL), the latter having a five-year mortality of 50%. Management options for RCD type 2 and response to treatment differs across centres and there have been debates over the best treatment option. Treatment options that have been used include azathioprine and steroids, methotrexate, cyclosporine, campath (an anti CD-52 monoclonal antibody), and cladribine or fluadribine with or without autologous stem cell transplantation. We present a tertiary centre's experience in the treatment of RCD type 2 where treatment with prednisolone and azathioprine was used, and our results show good response with histological recovery in 56.6% of treated individuals.

Surgery in (pre)malignant celiac disease.

AIM: To report the outcome of surgery in patients with (pre)malignant conditions of celiac disease (CD) and the impact on survival. METHODS: A total of 40 patients with (pre)malignant conditions of CD, ulcerative jejunitis (n = 5) and enteropathy associated T-cell lymphoma (EATL) (n = 35), who underwent surgery between 2002 and 2013 were retrospectively evaluated. Data on indications, operative procedure, post-operative morbidity and mortality, adjuvant therapy and overall survival (OS) were collected. Eleven patients with EATL who underwent chemotherapy without resection were included as a control group for survival analysis. Patients were followed-up every three months during the first year and at 6-mo intervals thereafter. RESULTS: Mean age at resection was 62 years. The majority of patients (63%) underwent elective laparotomy. Functional stenosis (n = 13) and perforation (n = 12) were the major indications for surgery. In 70% of patients radical resection was performed. Early postoperative complications, mainly due to leakage or sepsis, occurred in 14/40 (35%) of patients. Eight patients required reoperation. More patients who underwent resection in the acute setting (n = 3, 20%) died compared to patients treated in the elective setting. With a median follow-up of 20 mo, seven patients (18%) required reoperation due to long-term complications. Significantly more patients who underwent acute surgery could not be treated with adjuvant chemotherapy. Patients who first underwent surgical resection showed significantly better OS than patients who received chemotherapy without resection. CONCLUSION: Although the complication rate is high, the preferred first step of treatment in (pre)malignant CD consists of local resection as early as possible to improve survival.

Eosinophilic jejunitis presenting as intractable abdominal pain.

Eosinophilic gastroenteritis is an uncommon disease characterized by eosinophilic infiltration of the gastrointestinal tract. The clinical manifestations are related to the layer(s) and extent of the bowel involved. In this paper, we present a case of intractable abdominal pain caused by jejunal submucosal eosinophilic infiltration without mucosal involvement, diagnosed by deep endoscopic biopsies. The patient was successfully treated with steroids without need for surgery for diagnosis or therapy.

[Mesenteric inflammatory veno-occlusive disease is a rare nosological entity: Literature review and the authors' clinical observation].

Mesenteric inflammatory veno-occlusive disease (MIVOD) is a rare disease. Its basis is inflammation of the small and medium-sized mesenteric veins, which is generally complicated by thrombosis and intestinal ischemia. Arteries are not therewith involved in the process. According to the location, either ischemic jejunitis (ileitis) or segmental colitis develops. The etiology and incidence of MIVOD are unknown. Only a few tens of descriptions of clinical cases have been published. MIVOD is differentially diagnosed with Crohn's disease and vasculitides (Behçet's syndrome, Buerger's syndrome, rheumatoid arthritis, and systemic lupus erythematosus). The paper presents a MIVOD patient with partial thrombosis of portal, superior mesenteric and splenic veins, pylephlebitis, extrahepatic portal hypertension and with ischemic jejunitis complicated by fistulas, subcompensated stenosis, and seropurulent peritonitis. At laparotomy, an infiltrate with multiple interintestinal abscesses and a 40-cm jejunal segment with 4 fistular openings up to 1.5 cm in diameter and necrotic walls were removed and an interintestinal anastomosis was applied. A morphological examination of the operative material detected thrombi, stases, and pronounced plethora of the veins with fibrinoid changes in their walls. The lumen of arterial vessels of different diameters was free. No signs of systemic vasculitis and Crohn's disease were found. The postoperative period was uncomplicated.

Cytomegalovirus Jejunitis Diagnosed with Single-Balloon Enteroscopy / 대한소화기학회지

Cytomegalovirus (CMV) infections are usually diagnosed in immunocompromised patients. A 74-year-old male without any significant medical history visited our center because of abdominal pain and diarrhea which began about a month ago. Abdominal computed tomography revealed segmental enhanced bowel wall thickening on jejunum and single-balloon enteroscopy showed multiple geographic shaped ulcerations covered with exudates on proximal jejunum. Biopsy samples taken during endoscopic examination demonstrated necrotic fibrinopurulent tissue debris and benign ulcer. Nested-PCR analysis of CMV DNA from jejunal tissue was positive. The patient was finally diagnosed with CMV jejunitis and was treated by intravenous ganciclovir for 14 days after which, abdominal pain and diarrhea improved. Our case shows that CMV jejunitis can occur in an immunocompetent adult as multiple jejunal ulcers which can be diagnosed using a single-balloon enteroscope.

A Case of Idiopathic Recurrent Duodenojejunitis / 대한소화기학회지

There are various etiologies of duodenojejunitis such as Henoch-Schonlein purpura (H-S purpura), vasculitis, Crohn's disease, celiac sprue, ischemia, lymphoma, Zollinger-Ellison syndrome, bacteria or parasite infection, radiation, drug induced jejunitis, eosinophilic jejunitis, and toxins. A 31-year-old man presented with left upper quadrant pain. He did not have febrile sense, hematochezia, melena, diarrhea, arthralgia and hematuria. He had neither drug history nor traveling history. Esophagogastroduodenoscopy showed diffuse mucosal erythema and segmental hemorrhagic erosions on the distal area to the descending portion of the duodenum and proximal jejunum, which were commonly observed in the gastrointestinal involvement of H-S purpura. However, he showed no skin lesions, joint and urologic problems until the discharge. Autoimmune markers such as antinuclear antibody and antineutrophil cytoplasmic antibody were negative. Celiac and mesenteric angiogram showed no vascular abnormality. After the administration of oral prednisolone 40 mg daily for therapeutic trial, abdominal pain and endoscopic lesions were improved. He experienced relapses of same episode without skin lesions 16 times during follow-up of 8 years, which were also treated with prednisolone. The abdominal computed tomography during the follow-up also showed no significant finding. We report a case of primary recurrent duodenojejunitis similar to the gastrointestinal involvement of H-S purpura without purpura.

A Case of Segmental Nectotizing Jejunitis / 대한소아소화기영양학회지

Segmental necrotizing jejunitis is characterized by severe abdominal pain of acute onset, bilious vomitings and foul smelling loose stools containing blood. Pathologic features include circumferential intestinal wall inflammation ranging from edema with minimal congestion to severe congestion, hemorrhage with necrosis, ulceration, and gangrene with perforation. Early diagnosis and suitable supportive measures prevent unnecessary laparatomy and complications. There was no report of this disease entity in children in Korea. We experienced a case of segmental necrotizing jejunitis with fever, abdominal pain and bloody stools, which was diagnosed by exploration and was treated successfully by antibiotics and supportive measures.