A Rare Cause of Abdominal Pain: IgG4-Related Sclerosing Mesenteritis.

A 67-year-old man with previous cardiovascular disease was referred to our consultation due to a 5-month history of recurrent epigastric pain. Esophagogastroduodenoscopy and full blood workup presented no alterations. CT scan showed an irregularly shaped mass at the root of the mesentery, measuring 40x25x47mm, with spiculated contours and retractile behaviour (a). Simultaneous densification of the adjacent fat and infracentimetric ganglionic formations scattered throughout the mesentery were shown. Surgical biopsy revealed extensive storiform fibrosclerosis, with the presence of interstitial lymphoplasmocytic infiltrate and obliterative phlebitis (b); the plasma cells had mostly IgG expression, with IgG4:IgG ratio >40% (c), accounting for more than 30- 40 IgG4 plasma cells per field. The serum IgG4 level was 137mg/dL. A diagnosis of IgG4-related sclerosing mesenteritis was made, without other organ involvement. Prednisolone (0.6mg/kg/d) improved partially the abdominal pain, so steroid sparing strategy with off-label rituximab was associated. Due to its low prevalence, the understanding of this entity is scarce, and its diagnosis is challenging. Unlike other manifestations of IgG4-related disease, the intra-abdominal disease is identified in later stages, due to unspecific symptoms. This case aims to raise awareness about this condition as a differential diagnosis of abdominal pain.

Visceral extra-abdominal panniculitis after COVID19.

"Retractile mesenteritis" was the first name given to a rare, benign, inflammatory disease that affects the adipose tissue of the intestinal mesentery and less frequently other locations. Now labeled as mesenteric panniculitis, the pathogenic mechanism remains unclear. Several stimuli could be involved, and it is sometimes associated with other conditions such as malignancy or autoimmune diseases. We present a case of mesenteric panniculitis with extensive abdominal and extra-abdominal involvement that developed a few months after SARS-COV2 infection, raising the hypothesis of this virus as a potential trigger for autoinflammatory and autoimmune diseases.

Colchicine as an Alternative First-Line Treatment of Sclerosing Mesenteritis: A Retrospective Study.

BACKGROUND: Sclerosing mesenteritis is a rare condition characterized by chronic inflammation and fibrotic changes of the mesentery. AIMS: To determine the long-term management and outcomes of patients with sclerosing mesenteritis. METHODS: Patients with biopsy-proven sclerosing mesenteritis at the Mayo Clinic between January 2006 and December 2016 were identified. Clinical data were collected retrospectively. RESULTS: One hundred and three patients were identified, median age 68.0 years (range 35.0-85.3). Most patients were symptomatic (87.4%) at presentation. Patients received no treatment (52.4%), medical therapy (42.7%) or surgery (4.9%) on initial diagnosis. The most common initial regimens were prednisone plus tamoxifen (41.9%), prednisone alone (23.3%), and prednisone plus colchicine (11.6%) with 55.6%, 57.2%, and 60% of patients improving, respectively, p = 0.85 for a difference in response rates. At least half of the patients responded to prednisone plus tamoxifen, prednisone plus colchicine, or prednisone alone at 6.0, 7.2, and 8.4 months, respectively. At a median follow-up of 45.6 months (95% CI 24.1-69.7), 65.4% of patients were receiving medical therapy. Of those receiving tamoxifen-based, steroid-based, or steroid-sparing regimens, 100%, 87.5%, and 77.8% had improved by their last follow-up appointment respectively, p = 0.15. CONCLUSION: Prednisone plus colchicine has a similar efficacy to prednisone plus tamoxifen for the initial and long-term treatment of sclerosing mesenteritis. The majority of patients were initiated on medical therapy over the long term with most reporting symptomatic improvement within a year. Death from SM was rare.

Mesenteric Panniculitis Can Be Diagnosed by Examination and Cured by Comprehensive Therapy.

Mesenteric panniculitis is a rare, slowly progressive, benign, and chronic fibrous inflammatory disease that affects the adipose tissue of the mesentery. In the present study, we aim to investigate its clinical presentations, computed tomography/sonography scan features, effectiveness of the treatment, and overall prognosis. We investigated various presentations, etiologies, diagnostic approaches, potential treatment modalities, and overall prognosis of mesenteric panniculitis. We present one case of mesenteric panniculitis with abdominal pain, which underwent steroid treatment regimens subsequently and gained weight moderately. An abdomen and pelvis cavity computed tomography scan showed misty mesentery, an ill-defined increase in the density of the peritoneal fat at the base of the mesentery with few small associated lymph nodes. The appearance is that of a panniculitis. His symptoms gradually decreased in intensity and disappeared totally within 1 month after oral prednisone 40 mg per day and moderate gain weight. Computed tomography scan features of the disease have recently been delineated clearly. Standard treatment strategy does not exist, and the current ways mainly consist of immunosuppressor or anti-inflammation agents. Overall prognosis is usually good and recurrence seems to be rare.

The Use of Glucocorticoids for Better Control of Diabetes Mellitus: The Paradox of Sclerosing Mesenteritis (The Rare Could Become Common).

BACKGROUND Sclerosing mesenteritis is a rare disease characterized by chronic inflammation of mesenteric adipose tissue. To our knowledge, this is the first case report that presents the effects of glucocorticoid therapy on metabolic control in diabetes mellitus, aggravated by sclerosing mesenteritis. We want to show the significance of this rare disease, which could be underestimated as a cause of decompensation of diabetes mellitus. CASE REPORT A 57-year-old man with diabetes type 2 was admitted to the hospital to obtain better metabolic control of this disease. In addition, he reported persistent pain in the left side of his abdomen. Sclerosing mesenteritis was diagnosed based on the CT and MRI images. Prednisone was administered. The treatment resulted in better glycemic control and abdominal pain reduction. On follow-up after 1 year, the patient reported a decrease in the abdominal pain and an MRI showed a significant reduction of abnormalities in the mesentery. CONCLUSIONS It is known that glucocorticoids exacerbate hyperglycemia, particularly in patients with diabetes mellitus. However, we noticed contrary effects in the case of our patient. We suggest that the inflammatory process occurring in sclerosing mesenteritis was one of the main causes of metabolic decompensation in our patient. The effect of reduction of inflammation with glucocorticoids was stronger than the hyperglycemic effect of this treatment. That is why, in the presence of this autoimmune disease, the use of glucocorticoids can paradoxically lead to better glycemic control.

Mesenteric panniculitis as a presentation of Whipple's disease: case report and review of the literature.

BACKGROUND: Whipple's disease is a rare, multi-organ disease caused by Tropheryma Whipplei. A classic presentation is characterized by arthropathy, diarrhea and weight loss but a broad spectrum of manifestations is possible. We present a case of a patient with mesenteric panniculitis as a manifestation of WD. A comprehensive review of the literature is provided. PATIENT: A 50 year old male presented at the outpatient clinic after an episode of fever and abdominal pain abroad. CT scan showed mesenteric infiltration with associated lymphadenopathies consistent with mesenteric panniculitis. After receiving 6 months of antibiotic therapy abdominal and joint pains improved. CONCLUSION: Clinicians should be aware of Whipple's disease. Mesenteric panniculitis is a rare presentation of this possible lethal infection. The golden standard for diagnosing WD is a PAS positive small bowel biopsy. Adequate antibiotic therapy is the cornerstone of treatment and usually leads to an amelioration of symptoms.

Acute Recurrent Exacerbations of Mesenteric Panniculitis With Immunosuppressive Therapy: A Case Report and a Brief Review.

Mesenteric panniculitis (MP) is a rare, benign, and idiopathic disorder characterized by chronic inflammation of the mesenteric adipose tissue of the small intestine. The exact etiology of MP is unknown and its associations with underlying malignancies continues to be poorly understood. In this case report, we describe a rare case of acute exacerbations of MP in a middle-age female with a known past medical history of non-Hodgkin's lymphoma in remission and small bowel resection for a localized carcinoid tumor. The patient was diagnosed with MP 4 years ago and started on tamoxifen therapy with adequate control of her symptoms. Last year, she reported to the emergency department with multiple episodes of sudden-onset, severe, and localized right upper quadrant abdominal pain and nausea without vomiting. She was diagnosed with an acute exacerbation of MP and a decision was made to add 60 mg prednisone daily in addition to her tamoxifen regimen. She remained symptomatically stable for the next 6 months after the start of dual therapy with tamoxifen and prednisone. However, for the past 6 months, the patient reported to the emergency department on an average of 2 times/month with the same recurrent symptoms despite high compliance with tamoxifen and prednisone therapy. She was admitted for her pain management and her dose of prednisone was increased and she was subsequently discharged home with improvement of her symptoms. Her tamoxifen was switched to mycophenolate on her follow-up visit with gastrointestinal clinic, and her disease has remained stable for the past 2 months. Our case report discusses in-depth the literature on MP and its management. We also detail the steps in management of a rare case of recurrent acute exacerbations of MP despite the patient being on immunosuppressive therapy.

Mesenteric panniculitis in a patient with new onset dermatomyositis.

Mesenteric panniculitis (MP), part of the spectrum of sclerosing mesenteritis, is an often asymptomatic disorder that is characterised by chronic inflammation of abdominal mesentery. We present a case of an 83-year-old woman who presented with proximal muscle weakness and erythematous, photosensitive rash of the face and upper torso and was subsequently diagnosed with dermatomyositis based on skin biopsy, electromyography and muscle biopsy. She had radiographic evidence of panniculitis on CT scan of the abdomen and pelvis for malignancy surveillance, which improved on serial CT scan 3 months after beginning treatment for her underlying dermatomyositis with prednisone and mycophenolate mofetil. Our case highlights that MP can be associated with underlying autoimmune disease. Connective tissue disease could be considered in the differential of MP when other etiologies such as surgery, trauma and malignancy are ruled out.

A severe case of sclerosing mesenteritis.

Sclerosing mesenteritis is a rare benign disease presenting with chronic inflammation of the mesenteric adipose tissue and variable degrees of fibrosis. A 47-year-old black man presented to the emergency department with symptoms of small bowel obstruction, requiring surgical treatment. The laparotomy revealed a stenosis of the distal ileum with mesenteric thickening and an enterectomy was performed. The postoperative period was complicated by the maintenance of bowel obstruction. The patient underwent a second laparotomy, in which the fibrosing process of the mesentery was much aggravated, and an ileo-colic resection was needed. After knowledge of the histological diagnosis, the patient was started on immunomodulators with clinical improvement. At 26 months of follow-up, patient is asymptomatic under medical treatment. This is the first report, to our knowledge, of a patient with progressive fibrosis in such short period of time requiring surgical re-intervention for bowel obstruction, owing to sclerosing mesenteritis.

18F-FDG PET/CT Findings in a Patient With IgG4-Related Sclerosing Mesenteritis.

A large mass in the abdomen was revealed by ultrasonography on a 68-year-old woman presenting with abdominal pain and weight loss. To exclude the malignancy, an F-FDG PET/CT was performed, which showed a solitary soft tissue mass with heterogeneous F-FDG avidity at the root of small bowel mesentery. Because of the elevation of inflammatory markers and serum IgG4 level, IgG4-related sclerosing mesenteritis was suspected, which was proved by the subsequent biopsy. The mass gradually shrunk and ultimately disappeared in 6 months after a low-dose steroid therapy.

Sclerosing mesenteritis diagnosed with computed tomography and ultrasound-guided needle biopsy: the utility of the coaxial technique.

Here we report a case of sclerosing mesenteritis that we diagnosed with needle biopsy under the guidance of computed tomography (CT) and ultrasound (US) observation. An 82-year-old woman presented with appetite loss, weight loss and epigastric pain. CT of the abdomen and pelvis revealed increased density of the mesentery adjacent to the small bowel and enlarged lymph nodes. Sclerosing mesenteritis was suspected, but malignancies, such as lymphoma, were also considered. We performed CT and US-guided needle biopsy with the coaxial technique. An introducer needle was inserted, its correct location was documented with CT, and multiple specimens were taken with a finer needle passed through the introducer without incident. Adequate specimens were obtained, and the histological diagnosis of sclerosing mesenteritis was made. We treated the patient with corticosteroids and her symptoms and the radiographic findings improved. The coaxial technique was a useful and minimally invasive tool for making the diagnosis of sclerosing mesenteritis.

Sclerosing mesenteritis: a benign cause of mesenteric mass lesions.

Sclerosing mesenteritis is a rare disease of the mesentery. Associations with surgery, trauma, autoimmunity and paraneoplastic syndrome have been suggested, but most of the cases remain idiopathic. Diagnosis is often incidental, based upon the finding of a single or multiple mesenteric lesions on abdominal CT and histopathological confirmation. Optimal treatment is still controversial, but most of the cases reported have a favourable prognosis. We present a case of a 54-year-old male with long-standing abdominal pain and nausea, whose CT revealed the presence of a large mesenteric mass. A biopsy was performed, revealing benign chronic inflammation, fibrosis and IgG4-positive plasmocytes consistent with sclerosing mesenteritis. Clinical remission was achieved with corticosteroids and follow-up CTs at six and twelve months documented stability of the lesion. Furthermore, we review the current literature on the diagnosis and treatment options for this rare disease.

An Overlooked Potentially Treatable Disorder: Idiopathic Mesenteric Panniculitis.

OBJECTIVE: The aim of this study was to determine the prevalence of mesenteric panniculitis (MP) and to describe its clinical characteristics, therapy, and outcome. SUBJECTS AND METHODS: This retrospective study was carried out among patients with MP based on computed tomography (CT) scans from January 2012 to December 2015. The CT images were reanalyzed by study radiologists to confirm the previous MP diagnosis. Patients were divided into 2 groups, i.e., idiopathic and secondary, based on the presence or absence of associated predisposing factors such as trauma, malignancy, autoimmune disorders, ischemia, or previous abdominal surgery. The clinical characteristics of the 2 groups, as well as treatments, were assessed. RESULTS: Among the 19,869 CT scans, 36 patients (0.18%) with MP were identified (i.e., 19 [53%] females and 17 [47%] males). The median age was 54 years (range 26 - 76). Twenty-four patients (67%) were categorized into the idiopathic group. Malignancy was the predisposing factor in 8 (22%) of those patients. Furthermore, abdominal pain was the cardinal symptom observed in 22 patients (92%) in the idiopathic group. In the idiopathic group, 15 patients (63%) were treated with antibiotics and 16 (67%) were treated with nonsteroidal anti-inflammatory drugs (NSAID). One unresponsive patient was treated with colchicine. Symptomatic relief was achieved in all of the treated patients. CONCLUSION: In this study, a symptomatic idiopathic subgroup of patients with MP did not have any associated disorder. The response to treatment with antibiotics and NSAID was effective in most of the patients. Based on these findings, anti-inflammatory treatments beyond NSAID and surgery should be reserved for patients who are unresponsive to antibiotics and NSAID.

Mesenteric panniculitis.

A 53-year-old black woman presented with a 3-day history of abdominal pain. Ultrasound of the abdomen showed a gall bladder packed with small stones. She gave a history of abdominal surgery for a gynaecological condition. She had a cholecystectomy done, but her symptoms continued after cholecystectomy. She then had anendoscopic retrograde cholangiopancreatogram (ERCP) and sphincterotomy done, again her symptoms remained the same. A CT scan of the abdomen was done, and mesenteric panniculitis was suspected. A laparoscopic biopsy of the mesentery was performed, and it confirmed mesenteric panniculitis. She was started on a 2-week course of steroids to which she responded very well. Three months after the initial presentation, she was still asymptomatic.

[An analysis of clinical characteristics of twelve cases of mesenteric panniculitis].

Objective: Mesenteric panniculitis is an idiopathic, uncommon disease involving the adipose tissue of mesentery. The etiology, diagnosis and treatment are still unnoticed. We thus reported a case series to improve the understanding of this rare disorder. Methods: We retrospectively analyzed the clinical data of 12 patients with mesenteric panniculitis including manifestation, diagnosis, treatment and prognosis. Results: We found a male predominance (M∶F 3∶1) with the median age of 58 years old at diagnosis. The most common symptom was abdominal pain (9/12), followed by abdominal distension (3/12) and weight loss (3/12). Physical examination was unremarkable in the majority of patients (8/12). C reactive protein (9/12) and erythrocyte sedimentation rate (10/12) were normal in majority of patients. CT findings were of much diagnostic value. All patients had small intestinal mesentery involvement and multi-nodular appearance with increased fat density. Pseudo-capsule sign (8/12) and fat halo sign (6/12) were common. Pathological diagnosis was obtained in 4 cases showing fat tissue inflammation with local necrosis and fibrosis. Six cases all received prednisone, 2 with combined cyclophosphamide, 1 with azathioprine, 1 with tripterygium wilfordii. Short-term clinical response was achieved in all cases, but two patients relapsed. Conclusions: Mesenteric panniculitis occurs predominantly in middle-aged and elderly. Abdominal pain is the leading symptom. Inflammatory markers are often normal while computed tomography is the most important diagnostic tool. Surgery combined with cortical steroid and immunosuppressant agents is effective.

Increased levels of prostaglandin E-major urinary metabolite (PGE-MUM) in active mesenteric panniculitis patients: A case report.

RATIONALE: Mesenteric panniculitis (MP) is a rare disease with abdominal and systemic symptoms and is characterized by nonspecific inflammation, fat necrosis, and fibrosis in mesenteric fat. Active inflammatory responses may increase levels of prostaglandin E-major urinary metabolite (PGE-MUM), which was reported to reflect the disease activity of ulcerative colitis and chronic fibrosing interstitial pneumonia. We recently experienced a case with elevated PGE-MUM at the time of diagnosis of MP and we investigated the potential of PGE-MUM as a biomarker. PATIENT CONCERN: In this report we described 2 active mesenteric panniculitis patients with high PGE-MUM levels. DIAGNOSES: Mesenteric panniculitis INTERVENTIONS:: Both MP patients were measured the levels of PGE-MUM. OUTCOMES: Both MP patients exhibited high levels of PGE-MUM before treatment. In one, the levels were sensitively correlated with clinical symptoms and serological markers on steroids. LESSONS: The study observations suggest the potential of PGE-MUM to reflect the disease activity of MP. To verify its use, more findings based on clinical studies should be accumulated.