Diagnosis Of Sclerosing Mesenteritis Research Articles

Sclerosing mesenteritis following immune checkpoint inhibitor therapy.

Sclerosing mesenteritis (SM), a fibroinflammatory process of the mesentery, can rarely occur after immune checkpoint inhibitor (ICI) therapy; however, its clinical significance and optimal management are unclear. We aimed to assess the characteristics and disease course of patients who developed SM following ICI therapy at a single tertiary cancer center. We retrospectively identified 12 eligible adult cancer patients between 05/2011 and 05/2022. Patients' clinical data were evaluated and summarized. The median patient age was 71.5years. The most common cancer types were gastrointestinal, hematologic, and skin. Eight patients (67%) received anti-PD-1/L1 monotherapy, 2 (17%) received anti-CTLA-4 monotherapy, and 2 (17%) received combination therapy. SM occurred after a median duration of 8.6months from the first ICI dose. Most patients (75%) were asymptomatic on diagnosis. Three patients (25%) reported abdominal pain, nausea, and fever and received inpatient care and corticosteroid treatment with symptom resolution. No patients experienced SM recurrence after the completion of corticosteroids. Seven patients (58%) experienced resolution of SM on imaging. Seven patients (58%) resumed ICI therapy after the diagnosis of SM. SM represents an immune-related adverse event that may occur after initiation of ICI therapy. The clinical significance and optimal management of SM following ICI therapy remains uncertain. While most cases were asymptomatic and did not require active management or ICI termination, medical intervention was needed in select symptomatic cases. Further large-scale studies are needed to clarify the association of SM with ICI therapy.

Abstract #1385480: Goiter and Abdominal Mass: A Rare Presentation of Riedel’s Thyroiditis

Riedel’s thyroiditis (RT), also known as fibrous thyroiditis, is a rare inflammatory autoimmune disease that is often associated with various forms of systemic fibrosis such as sclerosing mesenteritis (SM). A 38-year-old female with a past medical history of hypertension presented with a painful growing goiter and a mesenteric mass, which was complicated by biliary obstruction and hydronephrosis. Physical exam showed tenderness on the abdomen and a diffusely enlarged firm thyroid gland, with no palpable nodules. Initial labs revealed C-reactive protein of 115 mg/L, TSH 180 mIU/L; Free T4: 0.79 ng/dL, positive Thyroid Peroxidase (>1051 IU/mL); normal IgG1 and IgG4. CT abdomen/pelvis demonstrated a mesenteric mass that encased multiple mesenteric vessels and the right ureter without bowel obstruction. Thyroid ultrasound showed an enlarged heterogeneous thyroid without a discrete nodule consistent with autoimmune thyroiditis. Thyroid fine needle aspiration (FNA) was non-diagnostic. Thyroid biopsy showed mostly fibrosis with scattered lymphoplasmacytic aggregates, with no evidence of carcinoma, lymphoma, IgG4-related disease, and no Hürthle cell presence. Interestingly, the mesenteric mass biopsy revealed the same pattern. Peripheral flow cytometry and infectious workup were negative. Diagnosis of RT and SM were made. The patient was treated with steroids and improved clinically. The hallmark of RT is the extension of fibrosis beyond the thyroid. This patient exhibited common features of RT such as neck pressure, and pain; female gender, elevated inflammatory markers, nondiagnostic FNA (scant cellularity due to fibrosis), and a positive steroid response. Moreover, intense infiltrative fibrosis mixed with lymphocytes and plasma cells instead of an extensive lymphocytic infiltrate with the germinal center formation on histology supports Riedel’s thyroiditis rather than Hashimoto's. Similarly, SM is a rare, non-neoplastic inflammatory and fibrotic disease that affects the integrity of mesenteric vessels causing common bile duct and ureter obstruction. Negative flow cytometry, the presence of sclerosis, and the absence of bowel/liver lesions involvement make other common causes of a mesenteric mass (lymphoma, desmoid tumor, carcinoid) less likely, supporting the diagnosis of SM. The concurrent timing of her thyroid enlargement and the mesenteric mass as well as the similarity in histology (dense fibrosis with patchy lymphoplasmacytic aggregates) suggests an association between RT and SM. Overall, the case highlights the challenges in diagnosing RT given its extreme rarity and emphasizes the importance of early treatment to prevent systemic involvement.

S2168 The Use of Rituximab for IgG4–Related Sclerosing Mesenteritis

Introduction: Sclerosing mesenteritis (SM) is an uncommon fibro-inflammatory disease affecting the abdominal mesentery. Although some patients are asymptomatic or have minimal symptoms, SM can present with complications such as bowel obstruction, chylous ascites, and mesenteric ischemia. First-line therapy includes glucocorticoids in combination with tamoxifen in those who are symptomatic. Case Description/Methods: An 82-year-old man with a history of prostate cancer and melanoma in remission and lymphocytic colitis presented with 4-months of poor appetite and 40-pound unintentional weight loss. Computed tomography (CT) abdomen showed an irregular 8.6 cm mesenteric mass with surrounding misty mesentery (Figure, Panel 1). CT-guided biopsy demonstrated fibro-adipose tissue with increased IgG4-positive plasma cells, supporting a diagnosis of IgG4-related SM. Follow-up CT abdomen 6 months later demonstrated enlargement of the mass with new encasement of the jejunal and ileal branches of the superior mesenteric artery and vein. Given impending mesenteric ischemia, he was treated with rituximab, a monoclonal anti-CD20 antibody, with 2 infusions 2 weeks apart without side-effects. He had contraindications to first-line therapy with glucocorticoids given prior suicidal ideation while on budesonide for microscopic colitis. Three months following treatment, his erythrocyte sedimentation rate improved from 52 to 25 (reference range, 3-28 mm/h) and IgG4 level from 851 to 267 (2.4-121 mg/dL). CT abdomen demonstrated a 50% decrease in the volume of the mesenteric mass without significant vascular involvement (Figure, Panel 2) and he had regained 30 pounds. Discussion: Although rituximab has been studied for IgG4-related disease in general, the use of rituximab specifically for IgG4-related SM is not well-known. We report a case of a patient with IgG4-related SM treated effectively with rituximab, suggesting this may be a suitable medication for those who have contraindications or do not respond to current first-line therapy, especially if IgG4-related. Whether this drug would also work in patients with SM not related to IgG4 disease is unknown. Patients treated with rituximab should be closely monitored for infections, as well as allergic and infusion-related reactions.Figure 1.: CT abdomen pelvis of sclerosing mesenteritis before (1) and after (2) rituximab treatment.

Sclerosing Mesenteritis, a Rare Cause of Mesenteric Mass in a Young Adult: A Case Report

Sclerosing mesenteritis (SM) is a rare fibroinflammatory disorder that involves mesenteric adipose tissue, more frequently localized in the small intestine, with an insidious clinical presentation having symptoms related to mass effect, usually resulting in bowel obstruction, mesenteric ischemia, as well as rapid weight loss. We report a case of a 23-year-old male presenting with palpable abdominal mass, mesogastric pain, and a history of rapid weight loss, who underwent exploratory laparoscopy. A hemorrhagic and gelatinous nodular tumor mass of the mesentery was identified and the surgical procedure was converted to a laparotomic approach. Histologically, the mass was composed of a proliferation of bland-looking spindle cells with slightly eosinophilic cytoplasm and elongated normochromatic nuclei with mild nuclear atypia, haphazardly set in a collagenized stroma; fat necrosis and inflammatory cells (lymphocytes, plasma-cells, and histiocytes) were also evident. The diagnosis of sclerosing mesenteritis was made. Our case emphasizes that histology remains pre-eminent for a correct diagnosis of SM, as pre-operative radiological-based diagnosis is non-specific.

Sclerosing Mesenteritis Presenting with Small Bowel Obstruction in a Patient with Compensated Cirrhosis: A Case Report

Sclerosing Mesenteritis (SM) is a rare fibroinflammatory disease of unknown etiology in which intestinal obstruction results from encasement of variable lengths of bowel by a dense fibrocollagenous membrane that gives the appearance of a cocoon. To our knowledge no cases of SM have been reported in patients with compensated cirrhosis without ascites. We report a case with SM diagnosed at laparotomy due to rapidly worsening small bowel obstruction, in a patient with compensated alcoholic cirrhosis. A 50-yearsold Caucasian male was evaluated because of cramping abdominal pain and discomfort associated with diarrhea, occasional post-prandial vomiting, fatigue and a significant weight loss. Compensated alcoholic cirrhosis had been diagnosed, two months before current presentation. Physical examination revealed abdominal distention with highpitched metallic sounds and bilateral peripheral pedal edema. Computed tomography of the abdomen demonstrated severe dilated small bowel loops as well as fluid into the peritoneal cavity. Based on the imaging findings, a surgical procedure was carried out. During laparotomy, an extra fibrous layer fully surrounding the small intestine was divided and a side-to-side functional ileo-cecum anastomosis was performed. Histopathological examination revealed fibroconnective tissue proliferation and inflammatory infiltration. Combined with the characteristic surgical findings a diagnosis of SM was made in a patient with compensated alcoholic cirrhosis. The patient recovered uneventfully and at 6-month follow-up he had no symptoms or signs of SM recurrence. The insidious onset of bowel obstruction symptoms in patients with even compensated cirrhosis, should include SM in the differential diagnosis.

S2893 A Common Malignant or a Rare Benign? Diagnostic Dilemma: A Case Report

INTRODUCTION: Sclerosing mesenteritis (SM) is a rare benign disease of unknown etiology. About 15 % of patients are asymptomatic or have minimal symptoms. It is usually benign in its clinical course, however, SM can mimic certain malignant disorders, such as lymphoma. It poses a diagnostic challenge for clinicians as it can be mistaken for malignancy and vice versa. In our case, the patient diagnosis of lymphoma was obscured by the picture of sclerosing mesenteritis, especially with negative multiple lymph node biopsies. CASE DESCRIPTION/METHODS: A 31 years old Caucasian male with a medical history of PTSD and a history of intussusception of colon requiring large and small bowel resection at age 14 who presented with severe generalized abdominal pain. CT abdomen ruled out any acute abdominal process, though enlarged mesenteric and retroperitoneal lymph nodes worrisome for lymphoma were noted. No history of weight loss, night sweats, or B symptoms. Laboratory investigations demonstrate transaminitis, otherwise, they are relatively unremarkable. CT chest and neck were significant for cervical chain lymphadenopathy. EGD showed gastritis and colonoscopy showed sigmoid colitis. Given his prior laparotomy for intussusception, the patient had retroperitoneal LN biopsy by IR and the pathology was reactive lymph nodes. Excisional cervical LN biopsy revealed the same diagnosis. After an extensive diagnostic work-up including biopsy sampling, which was negative for malignancy, the symptoms and findings were thought to be related to sclerosing mesenteritis. A trial of steroids and tamoxifen did not improve the symptoms. The patient eventually had mesenteric LN resection and pathology revealed follicular lymphoma. DISCUSSION: Sclerosing mesenteritis is rare. In most cases, it is discovered incidentally on abdominal imaging for evaluation of nonspecific abdominal or systemic symptoms. Our case highlights an important clinical finding that lymphoma can masquerade as sclerosing mesenteritis. A presumptive diagnosis of SM can be based on abdominal CT findings of a halo sign or fat ring. However, a histologic evaluation is necessary to rule out other etiologies and confirm SM. Percutaneous biopsies might be non-diagnostic because fine-needle specimens often lack sufficient architectural representation to exclude malignancy. Excisional biopsy by laparoscopy or laparotomy is usually required to obtain tissue. Pathologic confirmation should be obtained in all cases of suspected sclerosing mesenteritis.

IgG4-related sclerosing mesenteritis causing bowel obstruction: a case report

Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. Recently, SM was reported to be closely related to IgG4-related disease (IgG4-RD). This report describes a patient with SM associated with IgG4-RD. A 77-year-old woman with a history of surgery for ectopic pregnancy and wound dehiscence presented with intestinal obstruction. Abdominal enhanced computed tomography (CT) revealed an enhanced, radially shaped, oval mass, 3 cm in diameter, with an unclear rim in the mesentery of the distal ileum, which may have involved the distal ileum. To remove the cause of bowel obstruction, the SM was resected completely and the ileum was resected partially. Histologic examination showed that the mass was composed of spindle cells arranged in a fascicular or storiform pattern; moreover, fibrous stroma was observed, with dense lymphoplasmacytic infiltration and lymphoid follicles. Immunohistochemically, numerous IgG4-positive plasma cells were observed, at a density of 253 per high-powered field, and the IgG4/IgG ratio was about 50 %. Elastica van Gieson (EVG) staining also showed obstructive phlebitis. These findings indicated IgG4-related SM. Although the accurate diagnosis of SM remains difficult without histological analysis, IgG4-RD should be included in the differential diagnosis of unknown mesenteric tumors. Identification of IgG4-RD may prevent unnecessary surgery because corticosteroids may be effective in these patients.

Successful Treatment of Mesenteric Scleritis with Prednisone and Azathioprine Combination Therapy in an Elderly Male

Purpose: Sclerosingmesenteritis (SM) also known as, mesenteric panniculitis, retractile mesenteritis or mesenteric fibrosis is a rare fibro inflammatory disorder of unknown etiology that primarily affects the small bowel mesentery. Because of the paucity of published cases the optimal treatment options remained uncertain. Method: A 65-year-old male, with a history of hyperlipidemia, hypertension, diabetes mellitus, gout, and membranous glomerulonephritis, presented with a 6 month history of intermittent abdominal pain, fever, and 20-lbs weight loss. The abdominal pain was generalized, intermittent and dull with no change in his bowel habit. He took daily simvastatin, metoprolol, insulin, and glypizide. He denied history of recent travel overseas or tuberculosis exposure. Physical exam was notable for diffuse generalized abdominal tenderness on palpation but otherwise normal exam. Initial lab work revealed an elevated WBC of 17,000 with 80% Neutrophils, anemia with a Hgb of 9.9. His chem profile was normal with the exception of Cr of 2.5 (his baseline was 2-2.8). He had an elevated ESR and CRP. Infectious work up including urine, blood, and stool cultures, chest X-ray, and PPD test were normal but he was started on empirical antibiotic therapy. A non-contrast CT (due to contrast allergy) was performed which revealed diffuse subtle increased attenuation within the colonic mesenteric fat. Subsequent colonoscopy with random biopsy was performed which was normal. After obtaining surgical consultation, the patient underwent a diagnostic laparoscopy with peritoneal biopsy. Biopsies confirmed the diagnosis of SM with extensive fat necrosis with areas of fibrosis, presence of foamy histiocytes and inflammatory cells with paucity of IgG4-positive plasma cell. Results: Post-operatively he was started on prednisone 50 mg daily for one month and switched to Azathioprine 100 mg with prednisone taper. At 3 months follow-up, he remained afebrile and pain free. His inflammatory markers and WBC had all normalized. Conclusion: Although a relatively benign disease, sclerosing mesenteritis can sometime have a debilitating and fatal course; the need to treat patients with a diagnosis of SM remains debated. Approximately 50% of the patients might not require any treatment however symptomatic patients seem to benefit from treatment. A variety of anti-inflammatory and immunomodulatory agents have been used to treat patients, including steroids, azathioprine, colchicine and tamoxifen, but there is little data on which agents are most successful. In our patient, combination therapy with azathioprine and prednisone taper resulted in significant improvement in the patient's clinical course and lab values.

Sclerosing mesenteritis: A rare case of large bowel and rectum involvement

Sclerosing mesenteritis (SM) is an uncommon non-neoplastic inflammatory process in the mesentery that is seen as a pseudotumour, usually involving the small-bowel mesentery and, less commonly, the mesentery of the large bowel. The disease has two well-established histological types: the acute or sub-acute form known as mesenteric panniculitis and the chronic form known as retractile or SM. Because SM lacks special clinical manifestation and typical signs, the possibility of misdiagnosis is very high. The correct diagnosis of SM depends on pathological examination. Here in, we report a case of a 41-year-old male patient with SM of the large bowel.

A Case of Sclerosing Mesenteritis with Rheumatoid Arthritis

Sclerosing mesenteritis (SM) is an uncommon disorder characterized by chronic nonspecific inflammation involving the adipose tissue of the mesentery. The etiology remains unclear. It has been reported in association with and as an initial presentation in some autoimmune diseases. Its clinical presentation and laboratory findings are typically nonspecific and definitive diagnosis usually requires biopsy or surgical excision. We report a patient with a history of rheumatoid arthritis who was found to have an intra-abdominal mass suspicious for malignancy. A biopsy revealed the diagnosis of SM.

Sclerosing Mesenteritis Manifested as Epigastric Pain

Purpose: Sclerosing mesenteritis (SM) is a rare clinical entity that has been identified recently. We report a case of SM in an elderly gentleman with its clinical course over few years. An 83-year-old gentleman with history of multiple medical co-morbidities (including hypertension, diabetes and coronary artery disease) presented to the primary care clinic complaining of three month history of constant epigastric pain (although waxing and waning), sharp in nature, moderate in severity, radiating occasionally to both lower quadrants. The pain is exacerbated by fasting and spicy food, associated with nausea but no vomiting. Physical exam was benign and laboratory work-up including blood cell count, basic metabolic profile, liver function, and coagulation tests were normal. His description of the pain raised the suspicion of an upper gastrointestinal (GI) pathology like ulcer disease so he was referred to the GI clinic where he was evaluated and the decision was to proceed with an esophagogastroduodenoscopy (EGD) which was performed and was normal. The patient continued to complain of the same pain so a CT abdomen/pelvis was done which showed large central mesenteric mass with calcification raising the suspicion of lymphoma. The next step was CT guided biopsy of that mass which revealed fat necrosis; patchy and mixed B-cell, T-cell, histiocytic infiltrates with no evidence of neoplasia confirming the diagnosis of SM. The patient was then reassured and symptomatic therapy with oxycodone was started which was successful in controlling his pain symptoms very well. Follow-up CT scan was done two years later that showed regression in the size of SM and the patient continued to do well on regular follow-ups. Conclusion: SM is a rare benign disorder of unknown etiology affecting the mesentery. It has complex variable inflammatory changes in the mesenteric adipose tissue with nonspecific clinical presentation making the diagnosis usually late. CT scan is helpful in raising the suspicion of this disease but histological diagnosis remains the gold standard in confirming the diagnosis. It usually has stabile and benign course and there is no definite treatment so far so symptomatic therapy with pain medications remains the mainstay therapy.

Sclerosing mesenteritis associated with giant-cell temporal arteritis

A 72-year-old man presented with a history of progressive abdominal pain associated with simultaneous headache, constitutional syndrome, and weight loss. A CT scan demonstrated sclerosing mesenteritis (SM). A surgical biopsy of mesenteric tissue excluded malignancies and a temporal artery biopsy showed giant-cell arteritis, establishing the diagnosis of SM associated with giant-cell temporal arteritis (GCA). Both entities responded well to steroid therapy and relapsed simultaneously after steroid withdrawal. This association may be fortuitous, but it can also represent a unique clinical setting. We suggest ruling out GCA every time a SM is encountered in the appropriate clinical setting (i.e., in elderly patients with other symptoms and signs suggestive of GCA). A temporal artery biopsy may be systematically considered in these patients.