Materiały źródłowe

• #1 Sclerosing mesenteritis: a comprehensive clinical review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6174187/

  Sclerosing mesenteritis is a rare disease entity initially described in 1924 with a prevalence reported to be less than 1%. […] Although described as a rare disease, it is thought that the incidence of sclerosing mesenteritis could be as high as 3.4%, with a reported range of 0.163.4%. […] A recent systematic review of 192 cases of sclerosing mesenteritis showed an age range of 388 years with a mean age of 55 +/ 19.2 years. […] Most of the studies have been performed on the Caucasian population which limits generalization across the multiple ethnicities. […] Sclerosing mesenteritis is commonly diagnosed in the fifth to seventh decade of life although a case report with a three-year-old patient has also been published. […] However, the main limitation of these imaging-based studies is lack of biopsy for confirmation of diagnosis as well as lack of standard imaging criteria for diagnosis. […] The reported incidence also varies by the method of determination, such as if diagnosed by the histology versus radiologic criteria.

• #1 Sclerosing mesenteritis: a comprehensive clinical review

  https://atm.amegroups.org/article/view/20473/html

  Sclerosing mesenteritis is a rare disease entity initially described in 1924 with a prevalence reported to be less than 1%. […] Although described as a rare disease, it is thought that the incidence of sclerosing mesenteritis could be as high as 3.4%, with a reported range of 0.163.4%. […] The reported incidence also varies by the method of determination, such as if diagnosed by the histology versus radiologic criteria. […] Sclerosing mesenteritis is commonly diagnosed in the fifth to seventh decade of life although a case report with a three-year-old patient has also been published. […] Most of the studies have been performed on the Caucasian population which limits generalization across the multiple ethnicities. […] Most studies show a consistent relative preponderance of male gender, however, this has been debated in some studies.

• #2 IgG4-Related Sclerosing Mesenteritis

  https://www.jpatholtm.org/journal/view.php?number=16583

  Sclerosing mesenteritis (SM) is a rare disease first described by Sulla in 1924 under the name retractile mesenteritis. SM shows fibrosis with fat necrosis and chronic inflammation on microscopic examination. […] In particular, if the histologic and immunologic features are associated with IgG4-related disease (IgG4-RD), the condition is defined as IgG4-related sclerosing mesenteritis (IgG4-RSM). […] SM is a rare benign condition that presents with fibrosis, inflammation, and fat necrosis and occurs idiopathically in the small bowel mesentery. […] Clinically, patients with SM mostly complain of chronic severe pain and chronic non-specific problems such as nausea, vomiting, diarrhea, cramping, weight loss, and fever. […] The age of patients with IgG4-RSM ranges from 48 to 82. IgG4-RSM has a male predominance similar to SM.

• #2 Sclerosing mesenteritis and mesenteric panniculitis – clinical experience and radiological features | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-017-0632-7

  Sclerosing mesenteritis (SM) is a rare but probably underdiagnosed condition of inflammation in the mesentery. […] The reported prevalence in radiological studies ranges from 0.6 to 2.5%. […] The prevalence in clinical practice has not been studied but as an example only 92 cases were identified over a period of 23 years at the Mayo clinic, Rochester, which suggests a discrepancy between clinically relevant cases and the radiological finding in MP. […] The condition has earlier been described as benign but the clinical course may vary from no symptoms to severe and aggressive disease. […] In some studies it has been suggested an overrepresentation of SM in patients with malignant disease, although a matched pair analysis has questioned this. […] The aims of this study were to summarise the clinical experience from two regions in Sweden, to determine whether any correlation could be found between the radiological findings and the clinical disease course and to suggest an appropriate follow up strategy. […] We propose that the term SM should be reserved for this condition.

• #3 Sclerosing mesenteritis: a comprehensive clinical review

  https://atm.amegroups.org/article/view/20473/html

  Sclerosing mesenteritis is a rare disease entity initially described in 1924 with a prevalence reported to be less than 1%. […] Although described as a rare disease, it is thought that the incidence of sclerosing mesenteritis could be as high as 3.4%, with a reported range of 0.163.4%. […] The reported incidence also varies by the method of determination, such as if diagnosed by the histology versus radiologic criteria. […] Sclerosing mesenteritis is commonly diagnosed in the fifth to seventh decade of life although a case report with a three-year-old patient has also been published. […] Most of the studies have been performed on the Caucasian population which limits generalization across the multiple ethnicities. […] Most studies show a consistent relative preponderance of male gender, however, this has been debated in some studies.

• #3 A Clinical Review of Mesenteric Panniculitis – Gastroenterology & Hepatology

  https://www.gastroenterologyandhepatology.net/archives/april-2023/a-clinical-review-of-mesenteric-panniculitis/

  The prevalence of MP has been reported to range from 0.16% to 7.8% based upon radiologic criteria from several large computed tomography (CT) scan databases. […] The wide variation in the prevalence of MP depends upon the methodology of the studies and the CT criteria for diagnosis. […] Epidemiologic data based upon studies that report histopathologic diagnosis confirmation are limited. […] Regarding demographics, most cases occur during the fifth to seventh decades of life, although pediatric cases have been reported. […] There is a 2:1 male to female predominance in most studies; however, several prospective studies have shown a higher female prevalence. […] No clear racial associations have been made, but that could be because most studies have been conducted in White-predominant populations.

• #4 Sclerosing mesenteritis – UpToDate

  https://www.uptodate.com/contents/sclerosing-mesenteritis/print

  Sclerosing mesenteritis appears to be rare, and a significant proportion of patients are asymptomatic. In one autopsy series, the prevalence of sclerosing mesenteritis was one percent. In a prospective evaluation of over 7000 consecutive abdominal computed tomography examinations, the frequency of sclerosing mesenteritis was 0.6 percent. In another study of 613 consecutive unselected patients undergoing multidetector computed tomography, the frequency of mesenteric panniculitis was 7.8 percent. Although sclerosing mesenteritis has been reported in persons as young as three years of age, it is diagnosed most commonly in the fifth to seventh decades of life, with a median age of 65 years. The low prevalence in childhood and adolescence may be attributable to a smaller amount of mesenteric fat. While most studies have found that the prevalence of sclerosing mesenteritis is higher in men, this has not been consistently demonstrated.

• #5 Idiopathic sclerosing mesenteritis in the pediatric patient: A case report | Revista de Gastroenterología de México

  https://www.revistagastroenterologiamexico.org/en-idiopathic-sclerosing-mesenteritis-in-pediatric-articulo-S2255534X14000024

  Sclerosing mesenteritis makes up part of a spectrum of rare idiopathic conditions characterized by chronic inflammation of the mesentery and fibrosis. The epidemiology of sclerosing mesenteritis is unknown. A prevalence of 0.6% has been described from more than 7,000 abdominal computed axial tomography scans. The disease is regarded as rare in the pediatric age and only 17 cases have been reported up to the present date. Even though there are reports on children under 3 years of age, the majority of patients are in the fifth to seventh decades of life. […] It has been suggested in the literature that sclerosing mesenteritis is a rare entity in children and adolescents due to the lower quantity of mesenteric fat, and even though the precise etiology is still unexplained, mechanisms including previous abdominal trauma or surgery, autoimmunity, infection, and ischemia have been proposed.

• #6 Idiopathic Sclerosing Mesenteritis: An Extremely Rare Cause of Mesenteric Mass | Dhruv | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/3825/3177

  Sclerosing mesenteritis is an extremely rare inflammatory process that predominantly affects the mesenteric adipose tissue. […] By 2016, only 300 cases had been reported in the literature, showing the rarity of the clinical entity. […] It is mostly seen in Caucasian males between the fifth and seventh decades of life. […] Several causal factors are suggested like autoimmune disorders, abdominal trauma, malignancy, abdominal infections and even IgG4-related diseases, but the actual cause remains unknown. […] Most cases are asymptomatic. When symptomatic, abdominal pain is the most common symptom. […] CT scan of the abdomen is the best imaging modality to investigate this condition where it is generally described as a heterogeneous solitary mass, mainly localized in the mesentery, usually left-sided, with delocalization of the surrounding structures.

• #7 Idiopathic sclerosing mesenteritis – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_sclerosing_mesenteritis

  The epidemiology of Idiopathic sclerosing mesenteritis disease is extremely rare and has only been diagnosed in about an estimated 300 patients worldwide to date (as of 2014), it is probably under diagnosed.[11] […] It can occur in children.[10]

• #8 Sclerosing Mesenteritis: A Concise Clinical Review for Clinicians – PubMed

  https://pubmed.ncbi.nlm.nih.gov/38702129/

  Sclerosing mesenteritis (SM), an idiopathic nonneoplastic condition affecting 0.18% to 3.14% of the population, is characterized by chronic fat necrosis, inflammation, and fibrosis most commonly of the mesentery of the small intestine. […] Sclerosing mesenteritis typically presents in the fifth or sixth decade of life, where patients with a history of abdominal surgery and/or autoimmune disease may be at higher risk. […] Sclerosing mesenteritis has an overall benign course in most cases, but disease progression and fatal outcomes have been reported.

• #9 Sclerosing mesenteritis: a systematic review of 192 cases

  https://scholar.bridgeporthospital.org/descriptive_study/6/

  AIM: To perform a systematic review of previously reported cases of sclerosing mesenteritis (SM) to determine the epidemiology, risk factors, methods of diagnosis, treatment patterns and outcomes for this disease. […] Patients diagnosed with SM were between ages of 3 and 88 with a mean age of 55 19.2 years. SM was more common in Caucasians (n = 28, 60.8% of those reporting ethnicity) and men (n = 133, 69.3%) with a male to female ratio of 2.3:1. […] 28.6% (n = 55) of patients reported a prior abdominal surgery or abdominal trauma, 8.9% (n = 17) had a history of malignancy, and 5.7% (n = 11) of autoimmune disease.

• #10 Sclerosing Mesenteritis: Diverse clinical presentations and dissimilar treatment options. A case series and review of the literature | International Archives of Medicine | Full Text

  https://intarchmed.biomedcentral.com/articles/10.1186/1755-7682-4-17

  Sclerosing mesenteritis (SM) is a rare pathological condition affecting the mesentery. […] More than 250 cases have been reported in world literature and are often diagnosed incidentally, while SM shows a 0.6% prevalence in patients undergoing abdominal computed tomography (CT) for various reasons. […] The rarity of this condition has restricted the ability to record demographic and clinical features, natural history and response of the disease to therapy. […] Sclerosing mesenteritis is a disease of middle-aged or older adults (age range 20-90 years), primarily diagnosed during the 6th to 7th decade of life; the incidence increases with age, while pediatric cases are very uncommon, probably because children have less mesenteric fat than adults. […] Most studies indicate that the disease appears to be at least twice as common in men as in women.

• #11 Frontiers | Sclerosing Mesenteritis, a Rare Cause of Mesenteric Mass in a Young Adult: A Case Report

  https://www.frontiersin.org/journals/surgery/articles/10.3389/fsurg.2021.722312/full

  Sclerosing mesenteritis (SM) is a rare fibroinflammatory disorder that involves mesenteric adipose tissue, more frequently localized in the small intestine. […] The epidemiology of SM is not well-defined; autopsy studies by Kuhrmeier estimate an incidence of 1% (9/712 autopsies). […] There are also several radiological studies that estimate an incidence of 0.6% but lacking histological confirmation. […] SM typically occurs in the fifth/sixth decade of life, but occasional cases have also been documented in pediatric age. […] Most studies show a male prevalence with a Male/Female ratio of 2:1.

• #12 Sclerosing mesenteritis:an uncommon cause of chronic abdominal pain – MedCrave online

  https://medcraveonline.com/GHOA/sclerosing-mesenteritisan-uncommon-cause-of-chronic-abdominal-pain.html

  Sclerosing mesenteritis (SM), also known as mesenteric panniculitis or mesenteric lipodystrophy, is a rare disease that affects the mesentery of the small intestine. […] Only around 1200 cases have been reported; however, the prevalence is thought to be around 0.6% and many cases remain undiagnosed. […] SM is most common in white men over the age of 50. In systematic review of 192 cases done by Sharma et al. almost 60% of patients were white males with the mean age of 55 years with a male to female ratio of 2.3:1. […] The study by Sharma et al. on 192 cases showed that 28.6% of the cases had history of abdominal trauma or prior surgery, 8.9% had history of cancer and 5.7% had underlying autoimmune disease. […] In a review of 359 cases with SM like CT findings, 27.9% of the cases had either new or old cancer.

• #13 Idiopathic sclerosing mesenteritis in the pediatric patient: A case report | Revista de Gastroenterología de México

  http://www.revistagastroenterologiamexico.org/en-idiopathic-sclerosing-mesenteritis-in-pediatric-articulo-resumen-S2255534X14000024

  Sclerosing mesenteritis makes up part of a spectrum of rare idiopathic conditions characterized by chronic inflammation of the mesentery and fibrosis. The epidemiology of sclerosing mesenteritis is unknown. A prevalence of 0.6% has been described from more than 7,000 abdominal computed axial tomography scans. The disease is regarded as rare in the pediatric age and only 17 cases have been reported up to the present date. Even though there are reports on children under 3 years of age, the majority of patients are in the fifth to seventh decades of life. […] It has been suggested in the literature that sclerosing mesenteritis is a rare entity in children and adolescents due to the lower quantity of mesenteric fat, and even though the precise etiology is still unexplained, mechanisms including previous abdominal trauma or surgery, autoimmunity, infection, and ischemia have been proposed.

• #13 Idiopathic sclerosing mesenteritis in the pediatric patient: A case report | Revista de Gastroenterología de México

  http://www.revistagastroenterologiamexico.org/en-idiopathic-sclerosing-mesenteritis-in-pediatric-articulo-resumen-S2255534X14000024

  Diagnosis is established through histologic evaluation; plain x-ray is non-specific. Ultrasound imaging can aid in better visualizing the mesenteric fat. Computerized tomography scan is the most sensitive image for detection. Pathologic confirmation should be obtained in all cases, as was the case with our patient. It was the basis for establishing the definitive diagnosis.

• #14 Sclerosing Mesenteritis-Update on Diagnostic and Therapeutic Appr

  https://www.itmedicalteam.pl/articles/sclerosing-mesenteritisupdate-on-diagnostic-and-therapeutic-approach-108510.html

  The sclerosing mesenteritis (SM) is a rare, non-specific inflammatory condition, mainly affecting the benign intestinal mesentery. It is known that she is diagnosed mainly during the sixth and seventh decade of life and seems to be two times more common in men than in women. […] Is diagnosed mainly during the sixth and seventh decade of life and seems to be two to three times more common in males than in females. Also, several reports indicate that to SM is more common in Caucasian men. Pediatric cases are exceptional, probably because children have less mesenteric fat, when compared with adults. Prevalence rates reported vary from 0.16%-7.80%. […] The rarity of this condition restricts our study the demographic characteristics and clinical approach, as well as natural history and response to therapy disease.

• #15 Sclerosing Mesenteritis : Causes, Symptoms and Treatment

  https://www.apollohospitals.com/diseases-and-conditions/what-are-the-causes-symptoms-and-treatment-for-sclerosing-mesenteritis/

  Sclerosing Mesenteritis is a rare condition, and its not clear what causes it. […] Sclerosing Mesenteritis is an uncommon disorder that occurs most often in men between their fifth and sixth decades of life and is managed with surgery or medications. […] Although the actual cause of Sclerosing mesenteritis remains unclear, several factors and conditions are believed to be associated with Sclerosing Mesenteritis. […] Factors that may augment the risk of Sclerosing Mesenteritis surgery include: […] Sclerosing Mesenteritis shares symptoms with many other gastrointestinal disorders, it often gets misdiagnosed.

• #16 Idiopathic sclerosing mesenteritis presenting with small bowel volvulus in a patient with antiphospholipid syndrome: A case report

  https://www.wjgnet.com/2307-8960/full/v11/i14/3304.htm

  Sclerosing mesenteritis is a rare disorder involving inflammation of the mesentery. Its etiology remains unclear, but it is believed to be associated with previous abdominal surgery, trauma, autoimmune disorders, infection, or malignancy. […] The incidence of this condition is 0.16%3.30%. The etiology is still unclear; however, it may be associated with various conditions that cause chronic inflammation, such as previous abdominal surgery, trauma, mesenteric ischemia, cancer, infection, and autoimmune conditions. Sclerosing mesenteritis is more common in Caucasians, with a male-to-female ratio of 2-3:1. […] Sclerosing mesenteritis is a rare condition, and patients with no clear etiology should be considered for treatment with immunosuppressive therapy. […] Sclerosing mesenteritis refers to chronic inflammation of the mesentery. This condition is rare and is more common in Caucasian males.

• #17 Rare case of sclerosing mesenteritis and low grade follicular lymphoma

  https://www.wjgnet.com/2307-8960/full/v4/i4/108.htm

  Sclerosing mesenteritis also known as mesenteric lipodystrophy and mesenteric panniculitis is the part of the spectrum of idiopathic primary inflammatory and fibrotic process that affects mesentery. This is a relatively rare condition with clinical data limited only to case reports. It has a male preponderance and generally occurs in the sixth and seventh decades of life. Pathophysiology remains unknown and various mechanisms like trauma (including surgery), autoimmune, malignant and paraneoplastic causes have been postulated. There is an association with lymphoma, breast cancer, lung cancer, melanoma and colon cancer. In one study, prior abdominal surgery was present in about 35% of patients. There have been no reports of sclerosing mesenteritis transforming into a lymphoma, although in one case both of these lesions coexisted. We report an unusual case of long standing sclerosing mesenteritis that subsequently progressed to a follicular Hodgkins lymphoma.

• #18 Sclerosing Mesenteritis: Definition, Types & Treatment

  https://my.clevelandclinic.org/health/diseases/15284-sclerosing-mesenteritis

  Sclerosing mesenteritis is a rare disease that affects your mesentery, the tissue that attaches parts of your intestines to the back wall of your abdomen. […] Sclerosing mesenteritis is rare, affecting only about 0.6% of the population. Only about 200 cases have ever been reported, so we’re still in the early stages of learning about it. It most often affects people after the age of 50. Many people affected have a family history of autoimmune diseases. Research also suggests that as many as 60% of cases may be linked to prior or current cancer, especially lymphoma.

• #19 IgG4-Related Sclerosing Mesenteritis

  https://www.jpatholtm.org/journal/view.php?doi=10.4132/jptm.2015.12.03

  Sclerosing mesenteritis (SM) is a rare disease first described by Sulla in 1924 under the name retractile mesenteritis. […] SM shows fibrosis with fat necrosis and chronic inflammation on microscopic examination. […] In particular, if the histologic and immunologic features are associated with IgG4-related disease (IgG4-RD), the condition is defined as IgG4-related sclerosing mesenteritis (IgG4-RSM). […] Kerdsirichairat et al. conducted a systematic literature review of SM with IgG4 testing, and 11 out of 18 cases in total met the criteria for IgG4-RSM. […] The age of patients with IgG4-RSM ranges from 48 to 82. […] IgG4-RSM has a male predominance similar to SM. […] IgG4-RSM is known as a rare mesenteric disease of unknown etiology, although prior abdominal surgery can result in SM.

• #20 Sclerosing Mesenteritis-Update on Diagnostic and Therapeutic Appr

  https://www.itmedicalteam.pl/articles/sclerosing-mesenteritisupdate-on-diagnostic-and-therapeutic-approach-108510.html

  The sclerosing mesenteritis has usually a benign, self-limited course, slowly progressive evolution and resolves spontaneously in most cases, showing a favorable prognosis. This mainly depends on a correct diagnosis and extent of fibrotic process. The rate of recurrence is rare. However, 20% of patients have significant morbidity and a chronic debilitating course. […] Because of this, the more advanced stages seem to attend with a negative and less therapeutic response. A minority of patients have rapid and fatal evolution, and according to some authors, a reasonable percentage developed malignancies during the studies, including lymphoma, carcinoid syndrome, lung adenocarcinoma and mesothelioma. […] In the series of Daskalogiannaki et al. the sclerosing mesenteritis was related to malignancy in 69% of the patients. The association with malignancy can be coincidental or secondary to autoimmune inflammatory reaction. However, the exact mechanism has yet to be elucidated.

• #21

  https://journals.lww.com/ajg/fulltext/2021/10001/s3014_sclerosing_mesenteritis__a_paraneoplastic.3018.aspx

  Sclerosing mesenteritis is a rare, non-neoplastic, inflammatory and fibrotic disease affecting the mesentery. Most patients are asymptomatic, and the prevalence is reported to be 1%. […] Underlying malignancy has been reported from 1-75% in patients as a paraneoplastic syndrome. Non-Hodgkin lymphoma is most commonly associated. […] A lymph node biopsy should be considered in patients with sclerosing mesenteritis when other etiologies have been ruled out especially in patients with B-symptoms such as ours.

• #22 Sclerosing mesenteritis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/sclerosing-mesenteritis-1?embed_domain=hackmd.io%25252F%252540yIPUAFeCSL2JsU8smR5nJQ%25252Fbnjhjgjghjghjghfavicon.ico&lang=us

  Typically this condition afflicts adults in their 60s with a mild male predilection, although reports vary 1,2,4,19. […] Numerous associated conditions have been suggested including 1,4: malignancy, recent abdominal surgery, systemic inflammatory conditions, autoimmune conditions (may be related to IgG4-related disease), may be related to Weber-Christian disease 2. […] There is debate about the association between systemic inflammatory conditions and mesenteric panniculitis. Determining causation is difficult. The term „secondary mesenteric panniculitis” is reserved by some authors for patients with systemic inflammatory conditions. Most authors would not use the term when there is a local cause for mesenteric inflammation. […] While local lymphoma (in up to 15% of cases) 2,4, and a more general association with malignancy of 37-56% have been suggested 16,24, but a true association has not been proved. In patients diagnosed with mesenteric panniculitis, lymphadenopathy 12 mm, and absence of a 'fat halo’ around lymph nodes and vessels increased the risk of subsequent malignancy diagnosis in one study 24. Limitations of studies suggesting a malignancy association have been highlighted, and contrary evidence including a systematic review and meta-analysis, and case matched cohort studies have dismissed such an association 25-27.

• #23 Ultrasonography-based Management of Sclerosing Mesenteritis | IMCRJ

  https://www.dovepress.com/ultrasonography-based-management-of-sclerosing-mesenteritis-from-diagn-peer-reviewed-fulltext-article-IMCRJ

  Sclerosing mesenteritis (SM) is an uncommon disorder, typically diagnosed in the fifth to seventh decade of life with an estimated prevalence of 1% and male preponderance. […] The rarity of this disease and the lack of clinical trials and practical guidelines have generated many difficulties regarding its definition, the correct establishment of the diagnosis and the possible therapeutic strategies. […] In conclusion, US could be a sensitive, reliable and non-invasive procedure for the diagnosis and the follow-up of SM. Multicenter diagnostic trials, including a wide SM population, are needed to define the actual diagnostic accuracy of US in SM.

• #24 Sclerosing Mesenteritis-Update on Diagnostic and Therapeutic Appr

  https://www.itmedicalteam.pl/articles/sclerosing-mesenteritisupdate-on-diagnostic-and-therapeutic-approach-108510.html

  A more recent study described a prevalence of 0.6% in more than 7000 abdominal computed tomography examinations. […] The SM has unknown etiology and can occur independently or in association with other disorders. It is known that she represents the final stage of progression of chronic inflammatory diseases of the bowel mesentery, with predominance of fibrotic component. […] The purpose of this article is to deepen the understanding of the main features of SM, with a focus on the pathogenesis, clinical presentation, diagnosis, differential diagnosis, treatment and prognosis, currently available on this entity. […] The natural history of sclerosing mesenteritis is not well understood. This happens due to the rarity of this condition, as well as the confused nomenclature which disperses in epidemiological reports and the lack of proper monitoring.

• #25 Sclerosing Mesenteritis: Diverse clinical presentations and dissimilar treatment options. A case series and review of the literature | International Archives of Medicine | Full Text

  https://intarchmed.biomedcentral.com/articles/10.1186/1755-7682-4-17

  The pathophysiology of SM remains unknown. […] The disease is often asymptomatic and indeed most patients in any given series were incidentally identified during a CT examination. […] The diverse clinical presentation of SM was obvious in our series as well. […] Blood tests tend to be within the normal range. […] While a definite diagnosis of SM requires surgical excision biopsy and pathological analysis, in the majority of cases the disease is diagnosed predominantly on the basis of CT features. […] A large series by Kipfer et al, and Akram et al reported that 74% and 50% respectively of patients underwent surgical exploration in order to establish a diagnosis. […] There is no consensus of opinion on medical treatment for symptomatic cases of SM. […] Overall prognosis is usually good and recurrence seems to be rare. However long-term follow-up is needed to document these results.

• #26 Rare case of sclerosing mesenteritis and low grade follicular lymphoma

  https://www.wjgnet.com/2307-8960/full/v4/i4/108.htm

  The clinical presentation of Sclerosing mesenteritis varies from asymptomatic incidental diagnosis to various clinical features including abdominal pain, vomiting, diarrhoea, constipation, anorexia, weight loss, fatigue, fever of unknown origin, ascites, pneumoperitonium, pleural and pericardial effusion. […] The diagnosis of sclerosing mesenteritis requires appropriate clinical and radiological analysis with multidetector CT and MRI being the modality of choice. Biopsy is necessary for definitive diagnosis. The two main CT features are the fat ring sign consisting of area of fat around the mesenteric vessels and presence of a pseudocapsule around the tumour. Other CT findings consist of increased attenuation in mesentery, foci of fat necrosis and fibrosis appearing as low-attenuation areas and a solid mass encasing the mesenteric vessels.

• #27 Idiopathic sclerosing mesenteritis in the pediatric patient: A case report | Revista de Gastroenterología de México

  https://www.revistagastroenterologiamexico.org/en-idiopathic-sclerosing-mesenteritis-in-pediatric-articulo-S2255534X14000024

  Diagnosis is established through histologic evaluation; plain x-ray is non-specific. Ultrasound imaging can aid in better visualizing the mesenteric fat. Computerized tomography scan is the most sensitive image for detection. […] Pathologic confirmation should be obtained in all cases, as was the case with our patient. It was the basis for establishing the definitive diagnosis.

• #28 Sclerosing mesenteritis:an uncommon cause of chronic abdominal pain – MedCrave online

  https://medcraveonline.com/GHOA/sclerosing-mesenteritisan-uncommon-cause-of-chronic-abdominal-pain.html

  The overall all-cause mortality rate was 7.3% and the common causes of death were postoperative complications, bowel ischemia, sepsis and bronchopneumonia, venous thromboembolism, renal failure, cardiac arrest and pulmonary edema. […] There are no specific guidelines for treatment of SM however steroids, colchicine, azathioprine, thalidomide, cyclophosphamide and progesterone have been tried, however asymptomatic cases don’t need therapy. […] A treatment algorithm was prepared by Akram after review on 92 cases. Asymptomatic patients with incidental diagnosis do not need treatment. Patients with features of bowel obstruction need to undergo surgical treatment followed by tamoxifen plus tapering dose of steroids if there is persistence of symptoms.

• #29 IgG4-Related Sclerosing Mesenteritis

  https://www.jpatholtm.org/journal/view.php?doi=10.4132/jptm.2015.12.03

  There is no generalized consensus regarding the treatment of SM, including medical therapy, surgical therapy, and surgery with additional medical therapy. […] Even, when patients with SM are not treated, they show a high rate of spontaneous resolution. […] The rarity of the disease limits its clear characterization, and more cases and studies are needed.

• #30 Rare case of sclerosing mesenteritis and low grade follicular lymphoma

  https://www.wjgnet.com/2307-8960/full/v4/i4/108.htm

  Management of sclerosing mesenteritis is based on expert opinion with no recognised standard therapy. Treatment is generally guided by symptoms. Various treatments have been mentioned in case reports including immunosuppressive therapy with glucocorticoid and tamoxifen as first line therapy while colchicine, azathioprine or cyclophosphamide may be used as adjunct therapy. For refractory cases thalidomide can be considered. Surgery and radiation therapy both have a limited role. Surgery is best reserved for obstructive cases. The largest series was published from the Mayo Clinic in 2007 and based on their advice it is recommended that tamoxifen be continued indefinitely as the rate of recurrence and complications is significant. A combination of steroids (40-60 mg) and Tamoxifen 10 mg BD tapered over three months has been the regimen most widely used. Tamoxifen apparently decreases inflammation and down regulates fibroblast proliferation.

• #31 Idiopathic Sclerosing Mesenteritis: An Extremely Rare Cause of Mesenteric Mass | Dhruv | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/3825/3177

  Definitive diagnosis is made by histopathological analysis of the biopsy specimen. […] Literature review suggests these patients respond to the combination of corticosteroid and tamoxifen when symptomatic in most cases. […] Most cases slowly progress and may resolve over time. […] Overall, most patients will improve over time and if it is secondary to autoimmune conditions, the treatment of the autoimmune condition improves the inflammation in the mesentery. […] Further research is needed to establish the etiology of the disease and proper treatment protocols including medical vs. surgical management and their respective outcomes.

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