Esophageal Involvement Research Articles

S501 High Burden of Disease and Associated Costs for Eosinophilic Gastrointestinal Diseases: Results From an Online Patient-Centered Research Network

Introduction: Data regarding health care-related costs and the burden of disease for non-EoE eosinophilic gastrointestinal diseases (EGIDs) are lacking, particularly from the patient perspective. We aimed to determine the patient-reported health care burden of disease and costs related to care for non-EoE EGIDs. Methods: We performed a retrospective cohort study based in EGID Partners (egidpartners.org), an online patient-centered research network designed and launched in 2020 by patient advocacy groups (PAGs) and researchers. Subjects were recruited via informational emails, social media, directed messages through patient healthcare portals, webinars, and by physicians. Adult (≥18 years) EGID patients and caregivers of children < 18 years with EGIDs could join and complete surveys which included a burden of disease questionnaire. This instrument recorded patient-reported healthcare utilization over the prior year related to heath care visits and procedures, as well as out-of-pocket costs/payments. We calculated the median total costs and number of visits. Results: Of 58 non-EoE EGID patients with health care utilization data (mean (SD) age 29 (19) years; 69% female; 95% white; 88% with an atopic condition; mean (SD) 7.4 (9.9) years of symptoms prior to diagnosis), 13 had eosinophilic gastritis (EoG), 17 had eosinophilic enteritis (EoN), 6 had eosinophilic colitis (EoC), and 22 had multiple areas of overlap; additionally, 27 had esophageal involvement. The median number of health care visits over the prior year was 12 (IQR: 5-33; range 1-90), with a median of 5 outpatient doctor visits, 3 urgent care/ED visits or hospitalizations, and 2 endoscopy visits; results were similar for adult patient report (n=42) or caregiver report for pediatric patients (n=16) (Table). The annual median costs were $1000 (IQR: $100-$3570; range: 0-$100,400). Median costs for outpatient visits, testing, and medications were $250, $400, and $325, respectively. Costs were similar for adult patients and caregivers (Table). Conclusion: Patients with non-EoE EGIDs experience a high health care burden, with frequent health care encounters (median of 1 encounter/month) and substantial out-pocket costs (median of $1000/year). Both adult and pediatric providers must maintain awareness of the substantial burden in costs to EGID patients, including challenges for patients to manage their disease given these costs.

S2411 A Case of Esophagitis in a Patient With Poorly Controlled Systemic Lupus Erythematosus

Introduction: Primary Systemic Lupus Erythematosus (SLE) esophagitis is a rare entity that most commonly presents with odynophagia and dysphagia. Though esophageal involvement with SLE is common, it typically manifests as a motility disorder or secondary infectious or pill esophagitis rather than a primary SLE esophagitis. While its underlying pathologic mechanisms may be similar to other manifestations of SLE, SLE esophagitis remains poorly understood. Case Description/Methods: A 33-year-old man with history of juvenile discoid SLE complicated by arthritis, cutaneous involvement, oral ulcers, cerebritis, and 4-limb avascular necrosis secondary to chronic steroid use presented with a week of worsening oral ulcers, new-onset odynophagia, stable diffuse arthralgias, and active discoid lupus with ear and scalp involvement. His oral ulcers were more numerous and painful than was typical for his flares, and he had not previously experienced odynophagia during flares. Esophagogastroduodenoscopy (EGD) revealed diffuse erythematous, exudative, sloughed mucosa throughout the entire esophagus and stomach, while initial pathology revealed nonspecific findings with concern for possible vesiculobullous disorder. The patient was managed with increased immunosuppression for presumed SLE flare and his symptoms improved. Repeat EGD was performed to rule out an autoimmune blistering process, and immunofluorescence demonstrated granular deposits of IgG, IgM, and complement within the epithelial basement membrane, suggestive of SLE as the basis for the esophageal inflammation (Figure). Discussion: SLE esophagitis is an uncommon condition that results from granular deposition of immune complexes in the basement membrane of the esophageal epithelium, representing a form of SLE mucositis in an unusual location. In patients with a history of SLE who present with odynophagia or dysphagia this diagnosis should be considered as a possibility, especially in those with many previous SLE-related complications. Diagnosis requires EGD with biopsy to differentiate between this and more common causes of esophagitis seen in SLE patients. Management of SLE esophagitis should mirror treatment of other SLE flares with appropriate immunosuppressive therapy but warrants follow-up with a gastroenterologist to assess need for future EGD monitoring.Figure 1.: A: Ulcers on palate of mouth; B: Middle third of the esophagus; C: Lower third of the esophagus; D: Antrum of the stomach.

S2310 Esophageal Manifestations of Paraneoplastic Pemphigus Vulgaris Associated With Gastric Neuroendocrine Tumor

Introduction: While pemphigus vulgaris (PV) typically affects the epidermis and oral mucosa, esophageal manifestations are uncommon. The primary symptoms accompanying this manifestation are dysphagia, odynophagia, and hematemesis. In this case report we examine the occurrence of esophageal pemphigus vulgaris associated with a gastric neuroendocrine tumor and note the endoscopic findings of esophagitis dessicans superficialis (EDS) within the esophagus. Case Description/Methods: A 63-year-old female with a history of P-ANCA vasculitis presented for evaluation of dyspnea, hypotension, fatigue, rash, and dysphagia. The patient was thought to have an infectious cause of these signs and symptoms, however given a lack of response to antibiotic and antifungal therapy within 48 hours the differential was broadened to include rheumatologic disease. The patient was treated with intravenous methylprednisolone and continued to experience dysphagia. Esophagogastroduodenoscopy was performed (Figure) and demonstrated a tongue ulceration and extensive desquamation in the distal esophagus appearing similar to a pseudomembrane. Extensive subepithelial hemorrhages were observed in the esophagus, however the gastric and duodenal mucosa were intact. Esophageal and gastric biopsies demonstrated staining for IgG reactive to desmoglein 3 consistent with PV. In addition, a well-differentiated neuroendocrine tumor (NET) in the stomach. The patient was subsequently treated with octreotide for her neuroendocrine tumor, as well as solumedrol and dapsone for her skin lesions. She also received a loading dose of rituximab. She received a second dose of rituximab 21 days after the loading dose and the corticosteroids were tapered as her symptoms of dysphagia gradually improved with immunosuppressive therapy. Discussion: Esophagitis dessicans superficialis has been described as sloughing of the superficial esophageal mucosa and is similar in appearance to the Nikolsky sign of external squamous epithelium seen with immunobullous diseases. Our patient presented with undiagnosed PV and had both esophageal and skin involvement with the concomitant gastric NET. The ultimate diagnosis of PV was confirmed based on the presence of antibodies to desmoglein 3 and it remains unknown if the gastric NET had any role in the development of PV. Clinicians should have a low threshold for endoscopic examination of patients for whom there is suspicion of immunobullous disease to facilitate rapid diagnosis and initiation of appropriate therapy.Figure 1.: Endoscopic images demonstrating tongue ulceration and extensive desquamation in the distal esophagus appearing similar to a pseudomembrane in addition to subepithelial hemorrhages.

S3463 Primary Intestinal Follicular Lymphoma

Introduction: Follicular Lymphoma (FL) mostly presents as a nodal lymphoma, however primary extranodal involvement is rare. The gastrointestinal tract is the most common site of extranodal FL, and with rates of FL increasing it remains important to recognize the characteristic endoscopic features of the disease. It typically carries an indolent course that may not even require treatment. We present a case of primary intestinal FL that involved the entire small intestine. Case Description/Methods: A 53-year-old Hispanic male with a history of gastrointestinal reflux disease (GERD), presented with refractory GERD symptoms despite maximal PPI therapy. An upper endoscopy was performed and revealed an irregular Z line, gastritis and numerous, patchy, small (< 1cm), white nodules in the second and third portion of the duodenum. Multiple biopsies were taken from the nodules. Pathology revealed Grade I-II FL. Capsule endoscopy was performed and revealed numerous similar lesions throughout the entire small intestine. He was referred to Oncology and underwent PET-CT scan which revealed multiple FDG-avid abdominopelvic lymph nodes. After discussion with Oncology, the patient elected for a watch and wait approach. Repeat PET-CT scan 6 months later revealed multiple abdominopelvic lymph nodes that are no longer FDG-avid. He remains asymptomatic and will be monitored closely off therapy. (Figure) Discussion: The most common primary intestinal lymphomas are diffuse large B-cell lymphoma (DLBCL) and mucosa associated lymphoid tissue (MALT) Lymphoma. FL only comprises approximately 1-2% of primary intestinal lymphomas. The small intestine is the most common location, with the second portion of the duodenum as the most likely site of involvement. It is rarely seen in the colon and stomach and there are no reported cases of esophageal involvement. Rates of diagnosis are on the rise, thus highlighting the importance of endoscopic recognition as it may often be seen in the second portion of the duodenum on routine upper endoscopy. Given the indolent course of FL, a watch and wait approach may be reasonable. Other options for therapy include chemotherapy, Rituximab, or radiation.Figure 1.: White nodular mass in duodenum visualized on capsule endoscopy consistent with patient's known diagnosis of Follicular Lymphoma.

S2312 Esophageal Stricture Secondary to Mycophenolate Mofetil-Induced Injury

Introduction: Immunosuppressant medications like mycophenolate mofetil (MMF) have recently garnered more attention in the literature due to gastrointestinal toxicity. There are increasing case reports of transplant patients on MMF who develop inflammation or ulcers throughout the GI tract. It is theorized that the inflammation is due to MMF toxicity from metabolite formation. Although there has been increasing evidence of MMF induced injury to the GI tract, there are few reports of esophageal involvement, which is presented below. Case Description/Methods: This case involves a 40 year-old patient with Type I diabetes mellitus complicated by gastroparesis, renal and pancreatic transplants on immunosuppression with MMF, who developed progressively worsening dysphagia, weight loss, nausea, and vomiting. The patient initially presented to an outside gastroenterologist due to complaints of nausea and hematemesis. An EGD was completed with evidence of ulcerative esophagitis with biopsies negative for bacterial, fungal and viral stains. He was started on a proton pump inhibitor (PPI) for treatment of presumed reflux esophagitis. After no improvement with several months of therapy, he presented to our gastroenterology clinic for evaluation. Upon questioning, he specifically complained of progressive difficulty with swallowing after starting MMF. The patient underwent EGD and showed a severe distal esophageal stricture, which was not able to be traversed with the regular upper endoscope (Figure). Biopsies were taken and revealed acute erosive esophagitis. Immunohistochemical staining was negative for HSV and CMV. Given our high suspicion for MMF-induced esophageal injury, his medication was changed to liquid formulation. The patient underwent repeat serial endoscopies with dilation every 2 weeks for the next 2 months. After transitioning to liquid formulation, each subsequent endoscopy showed improvement in the ulcerated stricture. The patient was last dilated to 45 French in which he reported marked improvement of his dysphagia. Discussion: This case demonstrates a patient who developed a severe esophageal stricture from MMF induced injury with resolution of symptoms after multiple dilations and transition to a liquid formulation of MMF. Though the exact physiologic mechanisms are not well understood, gastroenterologists should be suspicious in patients on mycophenolate who present with dysphagia and stricture that is not explained by infection, malignancy, reflux, or other medications.Figure 1.: A. Initial non-traversable esophageal stricture B. Esophageal stricture status post multiple dilations C. Improving ulcerative stricture after switching to liquid formulation of MMF and multiple dilations.

S3659 Primary Gastric Squamous Cell Carcinoma With Concurrent H. pylori Infection and Colonic Metastasis

Introduction: Primary gastric squamous cell carcinoma (PGSCC) is a rare, aggressive, malignancy that cannot be distinguished from other gastric malignancies clinically and therefore, requires EGD with biopsy and pathology. H. pylori infection is a known risk factor for gastric malignancies but, only one case has been reported with an association between the two. Case Description/Methods: 66-year-old Hispanic male, with no significant medical, family, or social history presented to the hospital with syncope. Further history revealed three weeks of melena, fatigue, exertional dyspnea, and 50 lb weight loss. Upon evaluation, the patient was found to have HGB of 3.2. CT abdomen with contrast showed a mass in the antrum of the stomach, most prominent posteriorly and around the greater curvature. EGD revealed normal esophagus and a 15 cm, oozing, fungating, and partially circumferential gastric mass. It was located in the antrum, involving the entire posterior wall with extension into the greater curvature. The gastric mass was 5 cm below the GE junction, without evidence of esophageal involvement. Biopsy of the mass revealed poorly differentiated squamous cell carcinoma and Helicobacter pylori infection. Colonoscopy then revealed a 4 cm lesion near the splenic flexure which was confirmed to also be poorly differentiated squamous cell carcinoma. The patient was placed on triple therapy for his H. pylori infection. PET scan showed known gastric and colonic mass with multiple enlarged and hypermetabolic perigastric and retroperitoneal lymph nodes consistent with metastasis. He was seen by oncology and started on systemic chemotherapy (Figure 1). Discussion: PGSCC is a rare form of gastric malignancy accounting for roughly 0.2% of primary gastric cancer reported. Compared to the more common gastric adenocarcinoma, SCC tends to be more aggressive with poorer outcomes. Unfortunately, the pathogenesis remains obscure, making early detection difficult. Our case was associated with H. pylori infection which is one of the most significant risk factors for the development of gastric cancer due to the chronic inflammation caused by infection. Additionally, metastasis to the colon is exceptionally rare with most cases metastasizing to liver, peritoneum, lung and bone. Further investigation and studies are needed to understand this rare disease.Figure 1.: Primary gastric tumor (left panel), submucosal colonic mass (right panel).

P27 Challenging management of GI complications in Systemic Sclerosis

Introduction/BackgroundSystemic sclerosis is a chronic, progressive, multisystemic autoimmune disease in which there is excessive collagen deposition in the dermis and internal organs. It is commonly categorized into limited cutaneous or diffuse cutaneous systemic sclerosis. In more than 80% of patients there is GI involvement. Gut complications can impair oral intake, absorption and even faecal continence, and consequently can have severe adverse effect on quality of life. With disease progression, management of GI symptoms may become more of a challenge. We present a challenging case of SSs with esophageal involvement along with severe regurgitation who eventually responded to a specific prokinetic.Description/Method70 y/o female initially presented to gastroenterology clinic in 2013 due to dysphagia to solids and was found to have benign fibrotic oesophageal stricture for which she had therapeutic dilatation, (normal biopsy result). Subsequently, she had barium swallow which showed severe regurgitation. Manometry confirmed severely hypo-contractile oesophagus thus she was referred to rheumatology for suspected connective tissue disorder.On initial review, she did not describe typical inflammatory symptoms, scleroderma, sclerodactyly, Raynaud’s phenomenon, digital ulcers, calcinosis, telangiectasia or any other connective tissue features but was diagnosed with limited cutaneous systemic sclerosis due to ANA & anti-centromere antibody positivity, severe oesophageal dysmotility and diagnosis of mild pulmonary hypertension (on right heart catheter studies).Her main disabling SSs symptom was refractory belching, intermittent dysphagia and reflux disease resulting in poor oral intake and resultant malnutrition. She had 4-week trial of antibiotics to treat for presumptive small intestine bacterial overgrowth (SIBO) due to persistent belching but had no evident improvement. She had multiple endoscopies which only showed grade B oesophagitis due to which she remained on high dose PPI (Omeprazole 40mg BD) but showed no recurrent stricture. She was also given dual therapy with Ranitidine 150mg bd but derived no benefit.In Rheumatology clinic she was started on erythromycin 250 mg BD as a pro-kinetic and in liquid form for better tolerability (due to the dysphagia) with gradual but significant improvement of her upper GI symptoms. Switching liquid form to tablets/capsule form via GP resulted in worsening symptoms so was switched back to liquid form. GI symptoms worsened again in 2017 and impression was tachyphylaxis to erythromycin. She was started on trial of another pro-kinetic (metoclopramide) but symptoms continued worsening. In view of this she was started back on liquid erythromycin and her symptoms gradually improved again and has remained stable since.Discussion/ResultsSystemic sclerosis (SSc) is an incurable connective tissue disorder. It is estimated that approximately 90% of patients with SSc have some form of oesophageal involvement. For first three years, this patient’s main symptoms were her upper GI tract issues with reflux and oesophageal dysmotility causing delayed gastric emptying.Considering the severity of her GI symptoms, it was of great importance for her to maintain a regular follow up with gastroenterology team for a regular review of symptoms and repeat investigations as needed such as multiple repeat UGI endoscopy to ensure no recurrence of oesophageal stricture, monitoring of GORD, and hydrogen breath testing.We recommend for patients with severe oesophageal dysmotility to be started on prokinetics early to control GI symptoms and have minimal impact of these on quality of life. Liquid preparation has shown a significant improvement in symptoms in our patient as compared to tablet or capsule form which actually worsened the symptoms.Key learning points/ConclusionDysphagia can be a presenting symptom of systemic sclerosis in some cases. Upper gastro-intestinal symptoms can be severe with significant effect on quality of life.Management of these symptoms can be challenging and may require a combination of treatments.Pro-kinetics mainly erythromycin can have a major positive impact in controlling GI symptoms,Liquid form had better outcome in this case as compared to tablet or capsule form.Poor control of GI symptoms can result in significant malnutrition which is associated with more aggressive disease progression, so GI symptoms management plays a key role in disease control.

506. MANAGEMENT OF POST INTUBATION TRACHEO-ESOPHAGEAL FISTULA. HOW, WHEN AND WHY TO FACE OFF A POTENTIALLY FATAL INJURY

Abstract Post-orotracheal intubation tracheal injuries are rare but potentially fatal events. The data in the literature show differences in incidence for single-lumen and double-lumen tube intubations. Esophageal involvement may evolve into mediastinitis with poor prognosis. Treatment depends on the location of the lesion, size, extent and the patient's status. Timing and multidisciplinary team evaluation is mandatory for best decision. In a multi-centric observational study between January 2018 and December 2021 we observed 6 consecutive patients with iatrogenic post oro tracheal intubation tracheo-esophageal laceration underwent to multidisciplinary management. 4 patients were treated with end-to-end tracheal resection and anastomosis and closure of the esophageal fistula with cervicotomy access + interposed muscle flap without esophageal exclusion 1 patient was treated with tracheostomy and anterior fistula repair with cervicotomy access + esophageal exclusion 1 patient was treated conservatively for poor performance status. Surgical treatment was within 12 hours from diagnosis. 30-days mortality was observed in one case. Post-orotracheal intubation tracheal injuries are rare but potentially fatal events. Fast and multidisciplinary decision with aggressive treatment are the goal to improve results and reduce mortality.

286. THORACOPHRENOLAPAROMY FOR SURGICAL TREATMENT OF GEJ CANCER

Abstract Nowadays, there is still a lack of consensus about the optimal surgical management of gastroesophageal junction (GEJ) tumor. The CARDIA-trial results, comparing transthoracic esophagectomy versus transhiatal extended gastrectomy for GEJ type II tumor, are still expected. Thoracophrenolaparotomy (TPL) allows to perform radical resections (higher rate of R0 and more extensive mediastinal lymphadenectomy) with both esophago-gastric or esophago-jejunal anastomoses. The aim of this study is to describe TPL for the treatment of GEJ and gastric cancer. A retrospective analysis of all TPL performed for GEJ and gastric cancer with esophageal involvement at the Amsterdam UMC from January 2019 to October 2021 was conducted. The primary endpoint was the evaluation of R1 resections. The secondary endpoints were postoperative and pathological outcomes. Forty-seven patients were included for the analysis. 18 patients underwent an esophago-cardia resection with gastric conduit reconstruction and 29 patients a total gastrectomy with distal esophagectomy. The main indications for TPL were cT3 (74.5%) and cN+ (55.3%) tumors. Postoperative pneumonia occurred in 4 (8.5%) patients and anastomotic leak in 2 (4.3%) patients. The mean hospital stay was 9.8 (±6.1) days. The mean tumor size was 42.3 (±32.8) mm and the mean proximal margin 31.0 (±20.7) mm. Two (4.3%)patients had a R1 resection of whom only one (2.1%) had a positive proximal margin. The mean number of retrieved lymph nodes was 31.6 (±9.9). Thoracophrenolaparotomy for selected patients with GEJ and gastric cancer is associated with high R0 resection rate, low pneumonia and anastomotic leak incidence, together with a short hospital stay. TPL should still be considered a reliable surgical option, even in high-volume centers for minimally invasive upper gastrointestinal surgery.

Application of transverse cervical artery flap in laryngeal function preservation surgery of hypopharyngeal carcinoma

To explore the value and limitation of transverse cervical artery flap in laryngeal function preservation surgery of hypopharyngeal carcinoma. Between January 2013 and December 2019, 18 male patients with hypopharyngeal carcinoma were admitted. The patients' age ranged from 48 to 77 years, with a median age of 65 years. The disease duration ranged from 3 to 8 months (mean, 5 months). All patients were diagnosed as squamous cell carcinoma by biopsy before operation. According to the American Joint Committee on Cancer (AJCC) guidelines (2017, 8th ed), TNM staging was T2N0M0 in 9 cases, T2N1M0 in 2 cases, and T3N0M0 in 7 cases, and cTNM staging was stage Ⅱ in 9 cases and stage Ⅲ in 9 cases. The lesions of 15 cases were located in the piriform fossa of hypopharynx on one side, among which the esophageal entrance was involved in 4 cases. The lesions of 3 cases were located in the posterior wall of the hypopharynx with esophageal entrance involvement. After partial pharyngo- laryngectomy and bilateral neck lymph node dissection, the hypopharyngeal and laryngeal defects were repaired with transverse cervical artery flaps, the size of the flap ranged from 4 cm×3 cm to 6 cm×4 cm. The accompanying vein of transverse cervical artery (7 cases), external jugular vein (6 cases), and combination of both (5 cases) served as venous reflux. Retrograde external jugular venous reflux exercise was performed in 2 flaps with venous reflux obstruction during operation. The incisions at donor sites were directly sutured or via relaxed incision sutured. Radiotherapy and chemotherapy were supplemented within 3 months after operation. Tracheal cannula with air bag was used to prevent patients from aspiration in the early postoperative stage. The operation time was 4-6 hours, with an average of 4.5 hours. All patients were followed up 1-5 years (mean, 2 years and 6 months). Postoperative pathological examination showed that 7 cases had cervical lymph node metastases on the affected side, and there was no lymph node metastasis in cervical region Ⅴ; the remaining 11 cases had no lymph node metastasis. After operation, 16 flaps survived successfully, and 2 flaps with external jugular vein reflux were covered with white pseudomembrane, no flap necrosis was found after the pseudomembrane fell off. Four cases had no obvious accidental aspiration after operation; 14 cases had obvious accidental aspiration, of which 13 cases were significantly reduced at 3 months after operation, and 1 case still had obvious accidental aspiration at 6 months after operation, and the accidental aspiration decreased significantly after pulling out the gastric tube. All patients had no aspiration pneumonia. One case developed upper mediastinal lymph node metastasis at 1 year and 2 months after operation, and died of recurrence and pulmonary infection at 1 year and 3 months after operation. No recurrence or metastasis was found in the remaining 17 cases during follow-up. Tracheal cannula was successfully removed in 7 cases at 2-5 months after operation. Different degrees of accidental aspiration in 11 patients were confirmed by esophagography, so the tracheal cannula was retained. All patients had pronunciation function after operation. All incisions at the donor sites healed by first intention, and the shoulder joint function was normal. Using transverse cervical artery flap to repair the hypopharyngeal and laryngeal defects during hypopharyngeal carcinoma surgery in patients without lymph node metastasis in cervical region Ⅴ, can achieve good results of laryngeal function preservation. In cases with suspected lymph node metastasis in cervical region Ⅴ or venous dysplasia of accompanying vein of transverse cervical artery, there is a risk of tumor recurrence or flap necrosis, and the repair method needs to be cautiously employed.

POS-093 ODYNOPHAGIA AND SEVERE WEIGHT LOSS IN A CHILD TEN YEARS POST KIDNEY TRANSPLANT

Kidney transplantation is an immunosuppressive state with high risk of opprtunistic infections. Case report A 15 year old girl with history of live related kidney transplant at the age of 5 yrs due to end stage renal disease secondary to congenital bilateral dysplastic kidney, presented with low grade fever, odynophagia and significant weight loss of 11 kg over last 3 months (47kg dropped to 36Kg). She was on maintenance tacrolimus, mycopheolate mofetil (MMF) and prednisolone with stable creatinine at 0.7 mg/dl. She had past history of pulmonary aspergillosis 3 years ago for which she was treated with voriconazole for one and half years and recent chest CT was normal. On examination; apart from pallor, there were no other significant findings. Her haemoglobin was 8.8 gm/dl and C - reactive protein was elevated at 1.83 mg/dl (normal <0.6mg/dl). Despite normal looking oropharynx, upper gastrointestinal endoscopy was undertaken. This revealed 2 deep ulcers with raised margin and surrounding erythema in distal esophagus (Fig 1). Histopathology showed dense infiltrate with histiocytes, lymphocytes, polymorphs and fine dot like structures which were confirmed to be PAS positive fungal element (Fig 2a and 2b). Final diagnosis was established as fungal esophagitis due to histoplasmosis. Subsequently urine antigen for histoplasmosis also came as positive. She was treated for disseminated histoplasmosis with amphotericin for 14 days which was thereafter switched to long term itraconazole. Simultaneously her MMF was stopped and she was maintained on Tacrolimus (Target 12 hour’s trough 5ng/ml) and 5 mg of daily steroid. At 6 month follow up her creatininie has remained stable (0.7 mg/dl). She is afebrile and her appetite has normalized and weight is back to pre symptomatic level.View Large Image Figure ViewerDownload Hi-res image Download (PPT)View Large Image Figure ViewerDownload Hi-res image Download (PPT) Histoplasmosis is a common cause of odynophagia especially in immunocompromised patients but esophageal involvement is uncommon. Only less than 1% patients with disseminated histoplasmosis have esophageal involvement. A high index of suspicion and low threshold for undertaking upper gastrointestinal endoscopy helped us in establishing the diagnosis and instituting specific treatment.

Primary Esophageal Tuberculosis

Developing countries such as India, harbor almost a quarter of global tuberculosis (TB) burden. Esophageal involvement in TB, itself is a rare entity with primary esophageal TB reported only as isolated case reports. We report a case of an immunocompetent young adult who presented with dysphagia as the solitary symptom. Upper GI endoscopy and barium swallow showed a midthoracic esophageal stricture. He was subsequently diagnosed with “primary” esophageal TB based on histological examination of endoscopic biopsy showing features consistent with TB and demonstration of normal adjacent pulmonary parenchyma and lymph nodes on a computed tomography scan of the thorax. He showed marked symptomatic improvement with temporary esophageal dilation and stenting which helped in the effective administration of antituberculous therapy as well as attaining nutritional recovery. Our case report emphasizes on this rare scenario of “primary” involvement of esophagus in an otherwise common disease such as TB and its management strategy.

Acute Esophageal Necrosis: A Retrospective Cohort Study Highlighting the Mayo Clinic Experience

ObjectiveTo describe the clinical, endoscopic, and histologic features in patients with acute esophageal necrosis (AEN). Patients and MethodsIn this retrospective cohort study, patients who were diagnosed as having AEN at Mayo Clinic sites in Minnesota, Florida, and Arizona between January 1, 1996, and January 31, 2021, were included. Data were collected on patient clinical characteristics and endoscopic and pathologic findings. ResultsThe study included 79 patients with AEN with a median (range) age of 64 years (12 to 91 years); 53 (67.1%) were men. Predominant presenting symptoms were hematemesis (49 of 79 [62.0%]), abdominal pain (29 [36.7%]), and melena (20 [25.3%]). Shock was the triggering event for AEN in 49 (62.0%). The 30- and 90-day mortality were 24.0% (19 of 79) and 31.6% (25), respectively. The presence of coexisting infection or bacteremia was significantly associated with 90-day mortality (P<.01). Endoscopically, involvement of the distal third only, distal two-thirds only, and entire esophagus was observed in 31.6% (24 of 76), 39.5% (30), and 29.0% (22), respectively. The length of esophageal involvement correlated with duration of hospitalization (P=.05). The endoscopic appearance of the esophageal mucosa ranged from predominantly white (21 of 44 [47.7%]) to mixed white and black (13 [29.6%]) to predominantly black (10 [22.7%]), and sloughing was present in 18 (40.9%). In the 26 patients with histopathologic findings available for review, 25 (96.1%) had necrosis and/or ulceration with abundant pigmentation. Among the 79 patients, 39 (49.4%) had a follow-up esophagogastroduodenoscopy; 26 of these 39 patients (66.7%) had resolution while 5 had persistent AEN, 4 of whom had improvement. Esophageal strictures developed in 7 of the 39 patients (18.0%). ConclusionAcute esophageal necrosis is a serious condition observed in critically ill patients. Its endoscopic appearance can be highly variable. In patients with an unclear diagnosis, esophageal biopsies may be helpful given the characteristic histologic findings.

Changes on chest HRCT in systemic sclerosis-related interstitial lung disease after autologous haematopoietic stem cell transplantation.

To evaluate extent of interstitial lung disease (ILD) and oesophageal involvement using high-resolution computed tomography (HRCT) in early diffuse SSc patients after autologous haematopoietic stem cell transplantation (aHSCT). Overall chest HRCT, lung function and skin score changes were evaluated in 33 consecutive diffuse SSc patients before and after aHSCT during yearly routine follow-up visits between January 2000 and September 2016. Two independent radiologists blindly assessed the ILD extent using semi-quantitative Goh and Wells method, the widest oesophageal diameter (WOD) and the oesophageal volume (OV) on HRCT. Patients were retrospectively classified as radiological responders or non-responders, based on achieved stability or a decrease of 5% or more of HRCT-ILD at 24 months post-aHSCT. Using a linear mixed model, the regressions of the extent of ILD and of ground glass opacities were significant at 12 months (ILD P = 0.001; ground glass opacities P = 0.0001) and at 24 months (ILD P = 0.007; ground glass opacities P = 0.0008) after aHSCT, with 18 patients classified as radiological responders (probability of response 0.78 [95% CI 0.58, 0.90]). Meanwhile the WOD and the OV increased significantly at 12 months (WOD P = 0.03; OV P = 0.34) and at 24 months (WOD P = 0.002; OV P = 0.007). Kaplan-Meier analyses showed a trend towards better 5-year survival rates (100% vs 60%; hazard ratio 0.23 [95% CI 0.03, 1.62], P = 0.11) among radiological responders vs non-responders at 24 month follow-up after aHSCT. Real-world data analysis confirmed significant improvement in extent of HRCT SSc-ILD 24 months after aHSCT, although oesophageal dilatation worsened requiring specific attention.

The therapeutic efficacy and safety of intravenous immunoglobulin in dermatomyositis and polymyositis: A systematic review and meta-analysis.

To evaluate the efficacy and safety of intravenous immunoglobulin (IVIG) in the treatment of dermatomyositis (DM) and polymyositis (PM). We searched PubMed, Embase, and the China National Knowledge Infrastructure for relevant studies from July 1919 to May 2021. Seventeen papers pertinent to our questions were found: In a meta-analysis, we found that IVIG significantly improved the level of CK (SMD (STD. Mean Difference) = -0.69; 95%CI -0.93, -0.46; P < 0.0001), Manual Muscle Test (SMD = 1.12; 95%CI 0.77, 1.47; P < 0.00001), Medical Research Council (SMD = 1.59; 95%CI 0.86, 2.33; P < 0.00001), Activities of Daily Living (SMD = 1.07; 95%CI 0.59, 1.56; P < 0.0001). The CK levels in DM and PM were also significantly improved after IVIG (SMD = -0.73; 95%CI -1.12, -0.34; P = 0.0002 and SMD = -3.29; 95%CI -5.82, -0.76; P < 0.0001, respectively). The meta-analysis of three RCTs showed that there was a statistically significant improvement after IVIG (SMD = 0.63; 95%CI 0.22, 1.03; P = 0.002). In a random effects model, pooled muscle power improvement rate was 77% (95% CI: 66.0-87.0%). Meta-analyses of IVIG as first-line therapy showed a significant improvement of the CK level (SMD = -0.71; 95%CI -1.12, -0.30; P = 0.0007). The polled improvement rate of oesophageal disorders was 88% (95% CI: 80.0-95.0%). There was no statistically significant difference in the rate of improvement between the number of courses <2 and ≥2 (0.80% vs. 0.80%, P = 0.9). The proportion of corticosteroid-sparing success reached 81.8%. Adverse reactions following IVIG administration are usually mild and transient. Seven patients developed serious adverse events. IVIG seems to be an effective drug for DM/PM, improving muscle strength, CK levels, and oesophageal involvement, and it is well tolerated by patients.

A Rare Presentation of Sarcoidosis: Dysphagia, Pancytopenia, and Acute Renal Failure

Sarcoidosis is a multisystem inflammatory chronic disorder that can virtually affect any organ system in the body. Most commonly affected organs are the intrathoracic structures with 90% of the reported cases involving the lungs. Esophageal involvement in sarcoidosis is extremely rare. Involvement of the esophagus and kidney along with hematological involvement is extremely uncommon in the same patient. Here, we present a case of a 58-year-old gentleman with a similar rare presentation. The patient presented with shortness of breath, productive cough, fatigue, and difficulty in swallowing, along with a weight loss of 20-30 pounds over three months. Laboratory workup was significant for leukopenia (2900 K/UL), serum creatinine level of 2.7 mg/dL (baseline: 1.2-1.7), and raised angiotensin-converting enzyme level at 187 nmol/ml/min. Chest X-ray showed bilateral widespread fine reticulonodular opacities, chest CT showed extensive bilateral reticulonodular opacities throughout the lung parenchyma, and fine-needle aspiration cytology of the right lung showed noncaseating granulomas. No fungal or acid-fast organisms were identified, and no evidence of malignancy was seen. Special stains for fungal (Grocott's methenamine silver and periodic acid-Schiff) and acid-fast organisms (acid-fast bacilli (AFB) and fluorescent AFB) were negative. Esophagogastroduodenoscopy (EGD) with gastric biopsy showed acute and chronic inflammation and no intestinal metaplasia, dysplasia, or malignancy was identified. Bronchoalveolar lavage was done, which showed macrophages (74%), neutrophils (6%), eosinophils (3%), and lymphocytes (17%), and was negative for malignant cells. QuantiFERON and AFB sputum/Mycobacterium tuberculosis polymerase chain reaction were negative. The patient was initially started on intravenous fluids and calcitonin, which significantly improved renal function and the calcium status of the body. Then prednisone 40 mg daily was started, which improved swallowing and breathing. After a week, prednisone was changed to 20 mg daily and was continued at the time of discharge.

Safety and effectiveness of esophagojejunostomy through extracorporeal versus intracorporeal methods after laparoscopic total gastrectomy

Objective: To compare the safety and effectiveness of esophagojejunostomy (EJS) through extracorporeal and intracorporeal methods after laparoscopic total gastrectomy (LTG). Methods: A retrospective cohort study was carried out. Clinicopathological data of 261 gastric cancer patients who underwent LTG, D2 lymphadenectomy, and Roux-en-Y EJS with complete postoperative 6-month follow-up data at the General Surgery Department of Nanfang Hospital from October 2018 to June 2021 were collected. Among these 261 patients, 139 underwent EJS with a circular stapler via mini-laparotomy (extracorporeal group), while 122 underwent intracorporeal EJS (intracorporeal group), including 43 with OrVil(TM) anastomosis (OrVil(TM) subgroup) and 79 with Overlap anastomosis (Overlap subgroup). Compared with the extracorporeal group, the intracorporeal group had higher body mass index, smaller tumor size, earlier T stage and M stage (all P<0.05). Compared with the Overlap subgroup, the Orvil(TM) subgroup had higher proportions of upper gastrointestinal obstruction and esophagus involvement, and more advanced T stage (all P<0.05). No other significant differences in the baseline data were found (all P>0.05). The primary outcome was complications at postoperative 6-month. The secondary outcomes were operative status, intraoperative complication and postoperative recovery. Continuous variables with a skewed distribution are expressed as the median (interquartile range), and were compared using Mann-Whitney U test. Categorical variables are expressed as the number and percentage and were compared with the Pearson chi-square, continuity correction or Fisher's exact test. Results: Compared with the extracorporeal group, the intracorporeal group had smaller incision [5.0 (1.0) cm vs. 8.0 (1.0) cm, Z=-10.931, P=0.001], lower rate of combined organ resection [0.8% (1/122) vs. 7.9% (11/139), χ(2)=7.454, P=0.006] and higher rate of R0 resection [94.3% (115/122) vs. 84.9 (118/139), χ(2)=5.957, P=0.015]. The morbidity of intraoperative complication in the extracorporeal group and intracorporeal group was 2.9% (4/139) and 4.1% (5/122), respectively (χ(2)=0.040, P=0.842). In terms of postoperative recovery, the extracorporeal group had shorter time to liquid diet [(5.1±2.4) days vs. (5.9±3.6) days, t=-2.268, P=0.024] and soft diet [(7.3±3.7) days vs. (8.8±6.5) days, t=-2.227, P=0.027], and shorter postoperative hospital stay [(10.5±5.1) days vs. (12.2±7.7) days, t=-2.108, P=0.036]. The morbidity of postoperative complication within 6 months in the extracorporeal group and intracorporeal group was 25.9% (36/139) and 31.1%, (38/122) respectively (P=0.348). Furthermore, there was also no significant difference in the morbidity of postoperative EJS complications [extracorporeal group vs. intracorporeal group: 5.0% (7/139) vs. 82.% (10/122), P=0.302]. The severity of postoperative complications between the two groups was not statistically significant (P=0.289). In the intracorporeal group, the Orvil(TM) subgroup had more estimated blood loss [100.0 (100.0) ml vs.50.0 (50.0) ml, Z=-2.992, P=0.003] and larger incision [6.0 (1.0) cm vs. 5.0 (1.0) cm, Z=-3.428, P=0.001] than the Overlap subgroup, seemed to have higher morbidity of intraoperative complication [7.0% (3/43) vs. 2.5% (2/79),P=0.480] and postoperative complications [37.2% (16/43) vs. 27.8% (22/79), P=0.286], and more severe classification of complication (P=0.289). Conclusions: The intracorporeal EJS after LTG has similar safety to extracorporeal EJS. As for intracorporeal EJS, the Overlap method is safer and has more potential advantages than Orvil(TM) method, and is worthy of further exploration and optimization.

740 Cyclosporine in Stevens Johnson Syndrome &amp; Toxic Epidermal Necrolysis: Experience from a Tertiary Care Center

IntroductionStevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are rare severe cutaneous adverse reactions associated with high morbidity and mortality, however, there lacks an established treatment protocol. Treatment with intravenous immunoglobulin (IVIg) has demonstrated mixed success rates in improving mortality. More recently, cyclosporine (CsA) has been found to have a promising role in management of SJS/TEN owing to its potent anti-apoptotic activity. We present 3 patients with SJS/TEN treated in our burn unit with CsA; all of them demonstrated clinical improvement.MethodsWe conducted a retrospective analysis of patients who were hospitalized with the diagnosis of SJS/TEN in a specialized burn center in the year 2020. Data regarding clinical factors, causative agent(s), disease severity, treatment received, and outcome were collected on chart review. SCORTEN and ABCD-10 prognostic scores were calculated for each patient at the time of admission. All patients were evaluated by a dermatologist and started on CsA at a dosage of 5 mg/kg daily given in 2 divided doses. Predicted mortality rate was compared with observed mortality rate to assess treatment outcomes.ResultsA total of 3 patients were identified with a mean age of 48.33 ± 16.50. All patients had TEN with an average initial BSA involvement of 56.67 ± 20.21 and peak BSA involvement of 63.33 ± 25.17. Lamotrigine was the presumptive causative drug in each case with an average duration of time from starting the medication to symptom onset of 19.3 days. Patients received CsA on average 7 days after symptom onset. Most of our patients had oral (100%), ocular (100%), genital (100%), and esophageal (33.3%) involvement during hospitalization. The observed mortality rate of 0% was lower than that predicted by SCORTEN (12.2% to 32.4%) and ABCD-10 (5.4% to 12.3%). No patients developed complications during admission. All patients had minimal wound care needs on discharge as most of the involved areas were healed by then.ConclusionsOur study sheds light on a possible beneficial role of cyclosporine for the treatment of SJS/TEN and reinforces the necessity of further prospective studies to solidify treatment guidelines.

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies: incidence using different testing criteria, and case series.

To estimate the incidence of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies utilising different testing criteria, and review the clinical details of a series of patients with associated autoimmune myopathy. The incidence of anti-HMGCR antibodies in 2019 from 3 groups, South West London, Berkshire/Surrey and Southampton, were compared in the adult population. Anti-HMGCR antibodies were measured by commercial chemiluminescent and immunodot assays. The case notes of patients with anti-HMGCR antibodies were reviewed for the case series. The estimated incidence of anti-HMGCR antibodies in the first 2 groups was 1.94 per million adults per year, and in the third group 10.3 per million adults per year. In the first 2 groups the test criteria restricted analysis to specific clinician request for anti-HMGCR. In the third group test criteria included cases with less specific clinical features or a cytoplasmic indirect immunofluorescence anti-nuclear antibody pattern. The latter strategy had a positive predictive value of 66.1% for anti-HMGCR associated myopathy. A case series of 27 patients with anti-HMGCR antibodies revealed 19 with myopathy, oesophageal involvement in 26% and median peak CK 8000 IU/L. Response to treatment, including intravenous immunoglobulin, was good with CK normalising after median 5.5 months. In 8 cases there was no evidence of autoimmune muscle disease, 7 not statin exposed. Varying criteria result in a 5-fold difference in estimated incidence of anti-HMGCR antibodies, revealing positive cases without evidence of myopathy. Patients with anti-HMGCR myopathy respond well to immune suppression, supporting wider testing for these antibodies amongst patients with myopathy.

Esophageal Bullous Pemphigoid.

Bullous pemphigoid is a rare autoimmune dermatologic disease that usually occurs in the elderly. Mucous membrane lesions occur in about 10–35% of patients and are almost always limited to the oral mucous membrane. Esophageal involvement is very rare (4% of cases) and usually presents with chest pain, dysphagia, and odynophagia, though patients are frequently asymptomatic. We report the case of newly diagnosed bullous pemphigoid in a 76-year-old man with a past medical history of dementia. He presented with cutaneous manifestations but also severe gastrointestinal bleeding due to extensive esophageal involvement. Although bullous pemphigoid is mainly a skin disease, mucous membrane lesions should not be overlooked as they are associated with an even poorer outcome. A high index of suspicion for esophageal involvement is needed as its presentation can be fatal, as with our patient.LEARNING POINTSBullous pemphigoid is a rare autoimmune disease that should be suspected in elderly patients with itchy cutaneous lesions.Mucous membrane lesions should always be evaluated, as they are associated with a poor prognosis, even if asymptomatic.Early diagnosis should be the main focus, as steroids, the mainstay of treatment, may not be effective in severe cases.