Mesenteritis stwardniająca
Patofizjologia i mechanizm

Stwardniające zapalenie krezki (sclerosing mesenteritis) to rzadka, przewlekła choroba zapalna i włókniejąca krezki jelita cienkiego, o nie do końca poznanej etiopatogenezie. Wśród potencjalnych mechanizmów patogenetycznych wyróżnia się przebyte zabiegi chirurgiczne lub urazy jamy brzusznej (występujące u około 30-41% pacjentów), procesy autoimmunologiczne (częsta współistniejąca choroba autoimmunologiczna i odpowiedź na leki immunomodulujące), zespoły paraneoplastyczne (choć związek przyczynowy nie jest jednoznaczny), zaburzenia związane z IgG4 (charakteryzujące się naciekiem limfoplazmocytowym, włóknieniem storiform i zarostowym zapaleniem żył, z ≥40% komórek IgG4-dodatnich) oraz niedokrwienie i infekcje. Choroba przebiega przez trzy stadia: lipodystrofię krezkową, zapalenie tkanki tłuszczowej krezki oraz stwardniające zapalenie krezki z włóknieniem i możliwą niedrożnością naczyń limfatycznych. Diagnostyka opiera się na badaniu histopatologicznym, gdzie obserwuje się martwicę tkanki tłuszczowej, przewlekłe zapalenie, włóknienie oraz makrofagi obładowane tłuszczem, a w postaci związanej z IgG4 dodatkowo naciek limfoplazmocytowy i charakterystyczne włóknienie.

Patofizjologia stwardniającego zapalenia krezki

Stwardniające zapalenie krezki (sclerosing mesenteritis) jest rzadką chorobą zapalną i włókniącą, która dotyka krezkę jelita cienkiego. Charakteryzuje się obecnością przewlekłego, niespecyficznego stanu zapalnego, który prowadzi do włóknienia tkanki tłuszczowej krezki. Dokładna etiopatogeneza tego schorzenia pozostaje niejasna, mimo licznych badań i obserwacji klinicznych.123

Proponowane mechanizmy patogeniczne

W literaturze medycznej opisywanych jest kilka potencjalnych mechanizmów patogenetycznych, które mogą być związane z rozwojem stwardniającego zapalenia krezki. Najczęściej wymieniane to:12

  • Przebyte zabiegi chirurgiczne lub urazy jamy brzusznej
  • Procesy autoimmunologiczne
  • Zespoły paraneoplastyczne
  • Niedokrwienie i infekcje
  • Zaburzenia związane z IgG4

Rola urazów i zabiegów chirurgicznych

Jednym z najczęściej opisywanych czynników związanych z rozwojem stwardniającego zapalenia krezki są przebyte zabiegi chirurgiczne w obrębie jamy brzusznej lub urazy. W przeglądzie systematycznym literatury, wcześniejsze zabiegi chirurgiczne lub urazy jamy brzusznej odnotowano u prawie 30% pacjentów z tym schorzeniem.23

Proponowana hipoteza zakłada, że stwardniające zapalenie krezki rozwija się u osób predysponowanych genetycznie, które mają nieprawidłową odpowiedź na procesy gojenia i naprawy tkanki łącznej po urazie. W jednej z serii przypadków obejmującej 92 pacjentów ze stwardniającym zapaleniem krezki, 38 pacjentów (41%) miało w przeszłości zabiegi chirurgiczne w obrębie jamy brzusznej, w tym cholecystektomię, appendektomię, histerektomię i kolektomię.2

Schorzenie to wiązano zarówno z ostrymi urazami jamy brzusznej, jak i przewlekłymi urazami powtarzalnymi (np. używanie pneumatycznego młota) oraz przewlekłym stanem zapalnym (np. utajone perforacje jelita krętego). Sugerowano również rolę rękawiczek chirurgicznych z pudrem jako potencjalnego czynnika wywołującego włóknienie jamy brzusznej, co może być prekursorem stwardniającego zapalenia krezki.23

Mechanizmy autoimmunologiczne

Rola procesów autoimmunologicznych w etiologii stwardniającego zapalenia krezki jest sugerowana przez wielu autorów.12 Choroba często współwystępuje z innymi schorzeniami autoimmunologicznymi, takimi jak toczeń rumieniowaty układowy, reumatoidalne zapalenie stawów, zapalenie tarczycy Riedla czy pierwotne stwardniające zapalenie dróg żółciowych.12

Osoby z rozpoznanym stwardniającym zapaleniem krezki często mają rodzinną historię chorób autoimmunologicznych lub sami cierpią na inne schorzenia o podłożu autoimmunologicznym.1 Dodatkowo, znacząca odpowiedź kliniczna na leki immunomodulujące w leczeniu tej choroby również wspiera hipotezę o autoimmunologicznym podłożu.1

Związek z chorobami związanymi z IgG4

W ostatnich latach zwrócono uwagę na potencjalny związek między stwardniającym zapaleniem krezki a chorobami związanymi z IgG4 (IgG4-related disease, IgG4-RD). IgG4-RD to grupa schorzeń charakteryzujących się tworzeniem guzopodobnych zmian w różnych narządach, naciekami z komórek plazmatycznych IgG4-dodatnich oraz podwyższonym stężeniem IgG4 w surowicy.12

Cechy histologiczne stwardniającego zapalenia krezki związanego z IgG4 (IgG4-RSM) obejmują co najmniej dwie z następujących zmian: naciek limfoplazmocytowy, włóknienie o układzie wrzecionowatym (storiform fibrosis) oraz zarostowe zapalenie żył. Kryterium immunologicznym jest stosunek komórek IgG4-dodatnich do IgG-dodatnich wynoszący ≥40%, przy czym ten wskaźnik jest uważany za drugorzędny w stosunku do obrazu histologicznego.12

Patogeneza chorób związanych z IgG4 pozostaje niejasna, ale sugeruje się obecność czynnika wyzwalającego, który stymuluje naciek komórek B. Komórki te następnie różnicują się w komórki plazmatyczne IgG4 w odpowiedzi na mediatory (IL-4 i IL-13) wydzielane przez limfocyty Th2. Komórki B i plazmablasty działają jako komórki prezentujące antygen dla limfocytów T CD4, które z kolei uwalniają substancje powodujące włóknienie.1

Potencjalny związek z procesami nowotworowymi

Jedną z największych kontrowersji dotyczących stwardniającego zapalenia krezki jest pytanie, czy choroba ta jest zespołem paraneoplastycznym.1 Stwardniające zapalenie krezki było opisywane w związku z różnymi nowotworami, takimi jak chłoniaki, przewlekła białaczka limfocytowa, szpiczak, rakoidy, nowotwory przewodu pokarmowego, rak nerki i rak płuca.12

Należy jednak zaznaczyć, że prawdziwy związek przyczynowy między stwardniającym zapaleniem krezki a nowotworami nie został jednoznacznie udowodniony. Badania sugerujące związek z nowotworami mają pewne ograniczenia, a przeglądy systematyczne i metaanalizy oraz badania kohortowe z dopasowanymi przypadkami podważyły takie powiązanie.1

Rola niedokrwienia i infekcji

Niedokrwienie i infekcje zostały zaproponowane jako potencjalne mechanizmy rozwoju stwardniającego zapalenia krezki, jednak dowody na poparcie tej hipotezy są ograniczone.1

Zmiany niedokrwienne opisywano w związku z wcześniejszą operacją, urazem jamy brzusznej, zakrzepicą krezkową i arteriopatią krezkową.1 Ponadto, niektóre badania sugerują związek z różnymi infekcjami, w tym dur brzuszny, czerwonka, gruźlica, kiła, malaria, grypa i gorączka reumatyczna.1

Chociaż infekcja została zaproponowana jako jeden z mechanizmów rozwoju stwardniającego zapalenia krezki, dostępne dowody w opublikowanych seriach przypadków lub dużych retrospektywnych przeglądach dokumentacji medycznej są bardzo ograniczone.1

Patologia i przebieg choroby

Stadia choroby

Stwardniające zapalenie krezki jest uważane za postępujący proces zapalny przechodzący przez trzy stadia, choć niektórzy autorzy uważają je za oddzielne jednostki chorobowe:12

  1. Lipodystrofia krezkowa (mesenteric lipodystrophy) – charakteryzuje się degeneracją tłuszczu krezkowego i obecnością makrofagów obładowanych tłuszczem
  2. Zapalenie tkanki tłuszczowej krezki (mesenteric panniculitis) – stan zapalny z naciekiem limfocytowym i obecnością grudek limfoidalnych
  3. Zapalenie krezki z retrakcją/stwardniające zapalenie krezki (retractile mesenteritis/sclerosing mesenteritis) – włóknienie, które może być związane z deformacją lub niedrożnością naczyń limfatycznych

Proces ten zostaje zapoczątkowany przez różnorodne bodźce, takie jak urazy termiczne lub chemiczne, zapalenie naczyń, awitaminoza, choroby autoimmunologiczne, zapalenie trzustki, wyciek żółci lub moczu, reakcje nadwrażliwości, a nawet infekcje bakteryjne.1

Obraz histopatologiczny

Diagnoza stwardniającego zapalenia krezki jest ustalana na podstawie badania histopatologicznego, które wykazuje kombinację następujących zmian:12

  • Martwica tkanki tłuszczowej
  • Przewlekłe zapalenie
  • Włóknienie
  • Makrofagi obładowane tłuszczem

W przypadku stwardniającego zapalenia krezki związanego z IgG4, dodatkowe cechy histologiczne obejmują naciek limfoplazmocytowy, włóknienie o układzie wrzecionowatym i zarostowe zapalenie żył.1

Makroskopowo obserwuje się pogrubienie krezki, które może być rozlane, pojedyncze guzkowate lub mnogie guzkowate. Histologicznie jest to mieszanka martwicy tłuszczu, przewlekłego zapalenia i włóknienia, które obejmują krezkę i tłuszcz podśluzówkowy jelita, podczas gdy błona śluzowa pozostaje nienaruszona.1

W prawie połowie przypadków najczęstszym znaleziskiem jest rozległe włóknienie z zapaleniem i niewielką martwicą tłuszczu, podczas gdy prawie jedna czwarta przypadków ma przeważający przewlekły stan zapalny, a pozostała część – makrofagi obładowane tłuszczem.1

Potencjalne mechanizmy molekularne

Zrozumienie patofizjologii stwardniającego zapalenia krezki pozostaje ograniczone. Zaproponowano kilka potencjalnych mechanizmów molekularnych związanych z rozwojem choroby:12

  • Obecność makrofagów rezydentnych w tkance tłuszczowej krezki
  • Transformacja miejscowych makrofagów w komórki piankowate, podobnie jak w procesie miażdżycy
  • Regulacja w górę receptora aktywowanego przez proliferatory peroksysomów gamma (PPAR-gamma) i ekspresji receptorów zmiatających
  • Potencjalny związek z zaburzeniami metabolizmu glukozy

Dane dotyczące tych mechanizmów są jednak ograniczone i spekulacyjne, a ich rzeczywista rola w patogenezie choroby wymaga dalszych badań.1

Implikacje kliniczne dla diagnostyki i leczenia

Zrozumienie patogenezy stwardniającego zapalenia krezki ma kluczowe znaczenie dla opracowania skutecznych strategii diagnostycznych i terapeutycznych. Choroba może powodować różnorodne objawy żołądkowo-jelitowe i systemowe, w tym ból brzucha, nudności i wymioty, biegunkę, utratę masy ciała i gorączkę.1

Diagnoza jest często trudna i zwykle opiera się na kombinacji badań obrazowych, serologicznych i histopatologicznych. Tomografia komputerowa jest najbardziej czułą metodą obrazowania do wykrywania zmian, ale ostateczne rozpoznanie wymaga potwierdzenia histopatologicznego.1

Nie ma konsensusu dotyczącego leczenia farmakologicznego pacjentów z objawowym stwardniającym zapaleniem krezki. Stosuje się różne leki przeciwzapalne, immunomodulujące i przeciwzwłóknieniowe:12

  • Glikokortykosteroidy (np. prednizon) – kontrolują stan zapalny
  • Leczenie hormonalne (np. tamoksyfen) – może spowolnić wzrost tkanki bliznowatej
  • Leki immunosupresyjne (azatiopryna, metotreksat, cyklofosfamid) – stosowane w przypadkach opornych na glikokortykosteroidy
  • Leczenie biologiczne (np. ustekinumab) – w przypadkach związanych z inhibitorami punktów kontrolnych układu immunologicznego

Pacjenci z większym komponentem zapalnym, z gorączką, utratą masy ciała i ogólnym złym samopoczuciem, wydają się najlepiej reagować na glikokortykosteroidy, stosowane samodzielnie lub w połączeniu z innymi lekami.1

W przypadkach, gdy tkanki bliznowate blokują przechodzenie pokarmu przez przewód pokarmowy, może być konieczne leczenie chirurgiczne.1 Nie ma jednak konsensusu, czy pacjenci wymagają leków immunosupresyjnych, a ze względu na małą liczbę pacjentów cierpiących na stwardniające zapalenie krezki, trudno jest przeprowadzić prospektywne i randomizowane badania.1

Kierunki przyszłych badań

Mimo postępów w zrozumieniu patofizjologii stwardniającego zapalenia krezki, wiele pytań pozostaje bez odpowiedzi. Potrzebne są dalsze badania w następujących obszarach:12

  • Dokładniejsze określenie etiologii i patogenezy choroby
  • Opracowanie jaśniejszych kryteriów diagnostycznych
  • Identyfikacja nieinwazyjnych markerów choroby
  • Ocena skuteczności leczenia farmakologicznego i chirurgicznego
  • Wyjaśnienie potencjalnego związku z procesami nowotworowymi
  • Badanie molekularnych szlaków związanych z rozwojem choroby

Dalsze gromadzenie doświadczeń klinicznych, lepsze zrozumienie patogenezy choroby oraz doskonalenie technik obrazowania pozwoli na opracowanie jaśniejszych kryteriów diagnostycznych i klinicznych, zawężenie poszukiwań diagnostycznych i, ostatecznie, poprawę i standaryzację leczenia.1

Ze względu na rzadkość występowania stwardniającego zapalenia krezki, jego szerokie spektrum kliniczne i ograniczone zrozumienie jego patofizjologii, leczenie opiera się głównie na doświadczeniu anegdotycznym i seriach przypadków.1 Konieczna jest dalsza starannie zaprojektowana współpraca między radiologami, patologami i klinicystami w opiece nad tymi pacjentami, co będzie miało zasadnicze znaczenie dla przyszłych wysiłków na rzecz zrozumienia tej choroby.1

Kolejne rozdziały

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #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%. […] The etiology of sclerosing mesenteritis is uncertain, but the disease has been associated with trauma, autoimmune disease, surgery, and malignancy. […] The etiopathogenesis of sclerosing mesenteritis remains unclear till now. A better understanding of the pathogenesis may eventually allow for more formal diagnostic criteria and eventually better management. […] At least four different pathologic processes have been proposed as etiologies of development of sclerosing mesenteritis including abdominal surgery/trauma, autoimmune phenomenon, paraneoplastic process, and ischemia/infection. […] Abdominal surgery has frequently been shown to be a precursor to the development of sclerosing mesenteritis.
  • #1 Sclerosing mesenteritis: a comprehensive clinical review
    https://atm.amegroups.org/article/view/20473/html
    Role of autoimmunity as an etiology of sclerosing mesenteritis has been purported by several authors. […] One of the single biggest debate pertaining to sclerosing mesenteritis has been the question of whether sclerosing mesenteritis is a paraneoplastic syndrome. […] Although infection has been proposed as one of the mechanisms of sclerosing mesenteritis development, there has been very little evidence provided in the published case series or large retrospective chart reviews.
  • #1 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
    The onset of SM is often associated with autoimmune conditions, such as IgG4 mediated sclerosing disorders, Lupus, rheumatoid arthritis, Riedel thyroiditis, and primary sclerosing cholangitis. […] Some authors have emphasized the link between SM and IgG4 disease, by showing the presence of IgG4 positive plasma cells in the context of SM; histologically, in these cases there is also the presence of storiform fibrosis and obliterative phlebitis.
  • #1 Sclerosing Mesenteritis: Definition, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/15284-sclerosing-mesenteritis
    People who get sclerosing mesenteritis often have a family history of autoimmune disease, and sometimes they have other autoimmune diseases themselves. […] Research suggests that people who’ve experienced inflammation from one of these causes are more likely to develop sclerosing mesenteritis. It’s as if their mesentery catches the inflammation and continues to keep it alive.
  • #1 Sclerosing Mesenteritis-Update on Diagnostic and Therapeutic Appr
    https://www.itmedicalteam.pl/articles/sclerosing-mesenteritisupdate-on-diagnostic-and-therapeutic-approach-108510.html
    The possible autoimmune etiology is supported by a significant clinical response to immunomodulatory drugs. […] The ischemic lesions are described correlated with the previous surgery, abdominal trauma, mesenteric thrombosis and mesenteric arteriopathy. […] Initially a degenerative process of mesenteric fat, followed by inflammation (panniculitis). Later, develops and fibrosis the retractable mesenteritis appears. […] The inflammatory process-fibrotic can affect the gastrointestinal lumen and the blood vessels and mesenteric lymph nodes, causing injury for mass effect, which may result in partial bowel obstruction, ischemia and ascites quilosa, justifying the diverse clinical gastrointestinal and systemic. […] The SM has been described in association with a number of conditions, including autoimmune thyroiditis, presumably of Riedel, primary sclerosing cholangitis, retroperitoneal fibrosis, and orbital pseudotumor.
  • #1 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) is a rare disease of the mesentery characterized by chronic inflammation and fibrosis of the mesentery which was first described by Jura as retractile mesenteritis in 1924 and the term sclerosing mesenteritis was given by Emory et al. The exact mechanism is unknown; however, several conditions have been associated with SM. The study by Sharma et al. on 192 cases showed that 28.6% of the cases had a history of abdominal trauma or prior surgery, 8.9% had a history of cancer and 5.7% had underlying autoimmune disease. Some authors have implicated the association of SM with IgG4 related disease. IgG4 related disease which is a fibroinflammatory condition is characterized by formation of tumor-like growth in multiple sites of the body and elevated IgG4 level in the serum. Other studies have shown the association of SM with carcinomas like breast, colorectal, gynecological and urological cancers and hematological malignancies like Non-Hodgkins Lymphoma and Multiple Myeloma. Sclerosing Mesenteritis is characterized by fat degeneration, necrosis, chronic inflammation, scarring and eventually fibrosis of the fatty tissue within the mesentery.
  • #1 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. […] In particular, if the immunologic and histologic characteristics are consistent with the IgG4-RD, it is classified as IgG4-RSM. The histologic features of IgG4-RSM include more than two of the followings: lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. The immunologic criterion is IgG4-positive/IgG-positive cells 40%, but this ratio is considered secondary in importance to the histological appearance.
  • #1 Mesenteritis esclerosante relacionada con IgG4, una entidad rara causante de dolor abdominal
    https://scielo.isciii.es/scielo.php?pid=S1130-01082018000300014&script=sci_arttext&tlng=en
    IgG4-related sclerosing mesenteritis is an inflammatory, fibrosing condition of an unknown origin. Cases have recently been reported with tissue infiltration by IgG4 plasma cells. This might be considered part of the IgG4-related disease spectrum, although further studies are required. […] The etiology is unknown but has been related to mechanisms such as abdominal trauma, surgery, autoimmunity, ischemia, infection and paraneoplastic syndrome. […] It was recently suggested that most sclerosing „idiopathic” conditions might belong within the IgG4-related disease spectrum. This is an immune-mediated systemic disorder characterized by the presence of lymphoplasmacytic infiltration rich in IgG4 plasma cells, storiform fibrosis, obliterative phlebitis and tissue eosinophilia. The pathogenesis remains unknown but the presence of a type of trigger has been suggested, which stimulates B-cell infiltration. These cells then differentiate into IgG4 plasma cells in response to mediators (IL-4 and IL-13) secreted by Th2 lymphocytes. B-cells and plasmablasts act as antigen-presenting cells to CD4 T-cells, which in turn release the substances that cause fibrosis.
  • #1 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 idiopathic disorder affecting mesentery, characterized by fat necrosis, chronic inflammation and fibrosis. […] Although the etiopathogenesis is still unclear, previous abdominal trauma or surgery, cancer, autoimmunity, infection, and medications have been linked to the development of SM. […] SM has been hypothesized to be a progressive inflammatory process, moving from mesenteric lipodystrophy to retractile mesenteritis, triggered by a wide variety of stimuli. […] The most common conditions associated with SM are previous abdominal trauma or surgery; in this context, SM might be the result of an abnormal response to healing and repair of connective tissue after trauma or surgery in genetically predisposed subjects. […] Moreover, SM has also been considered a paraneoplastic syndrome, since it is associated with different malignancies, such as lymphomas, chronic lymphocytic leukemia, myeloma, carcinoid, gastrointestinal cancers, renal cancer, and lung cancer.
  • #1 Sclerosing mesenteritis | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/sclerosing-mesenteritis-1?lang=us
    The mainstay of treatment is supportive, as the disease is typically self-limiting. If severe or protracted, medical therapy with corticosteroids, cyclophosphamide or azathioprine can be contemplated. Mesenteric panniculitis cannot be completely resected and surgery is of no benefit. […] While local lymphoma (in up to 15% of cases), and a more general association with malignancy of 37-56% have been suggested, 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. 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.
  • #1 Sclerosing Mesenteritis-Update on Diagnostic and Therapeutic Appr
    https://www.itmedicalteam.pl/articles/sclerosing-mesenteritisupdate-on-diagnostic-and-therapeutic-approach-108510.html
    Studies describe an intrinsic connection between the increase of serum IgG4 antibodies and the intestinal inflammatory process, suggesting a relationship between SM and IgG4-related diseases. […] The association with infections proved to be more evident in patients with personal history of infection with typhoid, dysentery, tuberculosis, syphilis, malaria, influenza and rheumatic fever. […] The major signs and symptoms appear to be associated with inflammation and the adjacent organ mass effect. […] The diagnosis is established by histopathologic study.
  • #1 Sclerosing mesenteritis | Radiology Reference Article | Radiopaedia.org
    https://radiopaedia.org/articles/sclerosing-mesenteritis-1?lang=us
    Sclerosing mesenteritis, also known as mesenteric panniculitis or retractile mesenteritis, is an uncommon idiopathic disorder characterized by chronic non-specific inflammation involving the adipose tissue of the bowel mesentery. It is often considered in the spectrum of autoimmune disease. […] The disease is said to pass through three stages, although some authors believe these to be separate entities: mesenteric lipodystrophy: degeneration of mesenteric fat, mesenteric panniculitis: inflammatory reaction, retractile mesenteritis / sclerosing mesenteritis: fibrosis, which may be associated with distortion or lymphatic obstruction. […] Histology demonstrates lipid-laden macrophages (mesenteric lipodystrophy), lymphocytic aggregates and lymphoid follicles (mesenteric panniculitis), variable amounts of fibrosis (retractile mesenteritis).
  • #1 A Clinical Review of Mesenteric Panniculitis – Gastroenterology & Hepatology
    https://www.gastroenterologyandhepatology.net/archives/april-2023/a-clinical-review-of-mesenteric-panniculitis/
    Also, MP has been proposed to be a progressive inflammatory process, starting from mesenteric lipodystrophy to retractile mesenteritis, triggered by a wide variety of stimuli, such as thermal or chemical injuries, vasculitis, avitaminosis, autoimmune disease, pancreatitis, bile or urine leakage, hypersensitivity reactions, and even bacterial infections. However, there is scarce evidence to demonstrate histologic progression of the disease.
  • #1 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 laboratory findings of SM are non-specific. Majority of the patients have elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). The study by Sharma et al. showed that 86.5% of patients had elevated CRP and 88.4% of the cases had elevated ESR. […] On pathology grossly, there is thickening of the mesentery which can be diffuse, single knotty or multiple knotty. Histologically there is a mixture of fat necrosis, chronic inflammation and fibrosis which involve the mesenteric and submucosal fat of the bowel and the mucosa remains intact. In almost half of the cases the most common finding is extensive fibrosis with inflammation and little fat necrosis while almost one quarter of the cases have predominant chronic inflammation and other quarter has lipid-laden foamy macrophages.
  • #1 A Clinical Review of Mesenteric Panniculitis – Gastroenterology & Hepatology
    https://www.gastroenterologyandhepatology.net/archives/april-2023/a-clinical-review-of-mesenteric-panniculitis/
    Although several hypotheses have been suggested, the underlying cause of MP remains unclear. Several case series have suggested potential etiologies, such as previous abdominal surgery, trauma, autoimmune diseases, chronic infection, or malignancies. However, most of these associations have been made in case reports and case series with no paired matched analysis and thus lack statistical significance. […] The histologic changes observed in the mesentery of patients with MP are a mixture of scattered lymphoplasmacytic infiltration, focal fibrosis, fat necrosis, and lipid-laden macrophages, which together lead to thickening and retraction of the mesentery with preservation of vessels, intestine, and/or lymph nodes. The understanding of the pathophysiology of MP remains limited. The presence of resident macrophages has been reported in mesenteric adipose tissue. It has been proposed that the transformation of local macrophages to foam cells might occur in MP similar to the process in the context of atherosclerosis, accompanied by upregulation of peroxisome proliferator-activated receptor–γ and scavenger receptor expression; although these data are speculative, they have been suggested as potential added molecular pathways linked to the pathophysiology of the disease.
  • #1 Sclerosing mesenteritis – UpToDate
    https://www.uptodate.com/contents/sclerosing-mesenteritis
    Sclerosing mesenteritis is a rare, non-neoplastic inflammatory and fibrotic disease that affects the mesentery. Sclerosing mesenteritis can affect the integrity of the gastrointestinal lumen and mesenteric vessels by a mass effect. Sclerosing mesenteritis can result in a variety of gastrointestinal and systemic manifestations, including abdominal pain, nausea and vomiting, diarrhea, weight loss, and fever. […] The pathogenesis of sclerosing mesenteritis remain unclear, although several mechanisms have been postulated. […] It is hypothesized that sclerosing mesenteritis results in genetically predisposed individuals who have abnormal responses to healing and repair of connective tissue in response to trauma. In a systematic review of the published literature, previous abdominal surgery or abdominal trauma was described in almost 30 percent of patients. In one series of 92 patients with sclerosing mesenteritis, 38 patients (41 percent) had prior abdominal surgery, including cholecystectomy, appendectomy, hysterectomy, and colectomy. Other studies, however, have not found an association with prior abdominal surgery or trauma. Sclerosing mesenteritis has been reported in association with both acute abdominal injury and chronic repetitive trauma (eg, pneumatic jackhammer) and inflammation (eg, occult ileal perforation). The use of powdered surgical gloves has also been implicated in the development of abdominal fibrosis as a precursor to sclerosing mesenteritis.
  • #1 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. […] A number of pharmaceutical agents have been used in an attempt to stabilize the disease. Glucocorticoids, azathioprine, methotrexate, colchicine, cyclophosphamide, and/or radiotherapy have been proposed as therapeutic options. The patients with a greater inflammatory component, with fever, weight loss, and general malaise appear to be the most receptive to glucocorticoids, alone or in combination.
  • #1 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 exact diagnosis is often difficult and is usually made by finding one of three major pathological features; fibrosis, chronic inflammation or fatty infiltration of the mesentery. […] The first stage involves mesenteric lipodystrophy in which a layer of foamy macrophages replaces the mesenteric fat. […] The final stage is retractile mesenteritis which is distinguished by collagen deposition and a diffuse presence of necrosis and fibrosis that contribute to tissue retraction. […] There is no consensus of opinion on medical treatment for symptomatic cases of SM. A variety of anti-inflammatory, immunomodulatory, and antifibrotic agents are used. […] In the first case of our series the combined use of corticosteroids and methotrexate against rheumatoid arthritis probably maintained SM in a tolerant state. […] Akram et al consider that the management of the disease should be based on severity and type of symptoms and not on CT findings.
  • #1 Sclerosing mesenteritis | Health Library | Memorial Health System
    https://www.mhsystem.org/health-library/con-20213811/
    Sclerosing mesenteritis is a condition in which tissue that holds the small intestines in place, called the mesentery, becomes inflamed and forms scar tissue. […] Sclerosing mesenteritis is rare, and its not clear what causes it. […] The cause of sclerosing mesenteritis is not known. […] Medicines for sclerosing mesenteritis are used to control inflammation. […] Corticosteroids such as prednisone control inflammation. […] Hormone treatments such as tamoxifen (Soltamox) may slow the growth of scar tissue. […] You may need surgery if the scar tissue blocks food from moving through your digestive tract.
  • #1 Treatment options for spontaneous and postoperative sclerosing mesenteritis
    https://www.wjgnet.com/1948-9366/full/v8/i11/761.htm
    Sclerosing mesenteritis is a rare pathology with only a few described cases in the literature. The etiology is unclear; however, several potential triggers, including abdominal surgery and abdominal trauma, have been discussed. […] The pathogenesis of sclerosing mesenteritis remains to be elucidated; however, it seems to be an inflammatory and immune response to local stimuli, such as abdominal surgery or trauma. Moreover, infections, autoimmune processes, malignancy, drugs, vasculitis, avitaminosis and hypersensitivity have been described as potential causes for sclerosing mesenteritis. […] The only evidence in the treatment of these patients is that, in the case of non-resolving bowel obstruction, surgery is needed. Because of the small number of patients suffering from sclerosing mesenteritis, prospective and randomized trials are difficult to perform. Therefore, it is still under investigation if patients need immunosuppressive agents.
  • #1 Sclerosing Mesenteritis: A Review Article | Insight Medical Publishing
    https://raredisorders.imedpub.com/articles/sclerosing-mesenteritis-a-review-article.php?aid=23602
    The histopathology associated with retractile mesenteritis consists mainly of fibrous tissue and collagen deposits, leading to scarring and retraction. […] Given the rarity of SM, its wide clinical spectrum, and the limited understanding of its pathophysiology, treatments are based on anecdotal experience and case series. […] Despite the rarity, an increasing number of SM diagnoses are being reported. […] The authors reported incidental radiographic finding of SM in a number of asymptomatic patients. […] In summary, SM is a difficult to diagnose disease that has the potential to be easily misdiagnosed. […] More research is required for investigation into superior diagnostic criteria, noninvasive markers of disease, and the efficacy of medical and surgical management.
  • #1 Sclerosing mesenteritis as a surgical problem: a review of the literature and own clinical observation
    https://ouci.dntb.gov.ua/en/works/4M052Ld7/
    The problem of studying the pathology of mesenteric fat of the small intestine in surgical practice has not yet received enough attention. […] Issues of etiology, pathogenesis, differential diagnosis of this disease and its possible connection with the paraneoplastic process currently continue to cause debate. […] Further accumulation of clinical experience, a better understanding of the pathogenesis of the disease, and improvement of imaging techniques will allow us to develop clearer diagnostic and clinical criteria, narrow the diagnostic search and, ultimately, improve and standardize treatment.
  • #1 Sclerosing Mesenteritis – PubMed
    https://pubmed.ncbi.nlm.nih.gov/30829677/
    Sclerosing mesenteritis is a rare non-neoplastic disorder characterized by fat necrosis, chronic inflammation, and fibrosis typically of the small bowel mesentery. […] While prior abdominal surgery or trauma, autoimmunity, infection, ischemia, and malignancy have been suggested to be involved in the pathogenesis of the disorder, it remains poorly understood. […] A careful use of terminology and communication between the radiologist, pathologist, and clinicians in the care of these patients will be essential to future efforts at understanding this disease.
  • #2 Sclerosing mesenteritis – UpToDate
    https://www.uptodate.com/contents/sclerosing-mesenteritis
    Sclerosing mesenteritis is a rare, non-neoplastic inflammatory and fibrotic disease that affects the mesentery. Sclerosing mesenteritis can affect the integrity of the gastrointestinal lumen and mesenteric vessels by a mass effect. Sclerosing mesenteritis can result in a variety of gastrointestinal and systemic manifestations, including abdominal pain, nausea and vomiting, diarrhea, weight loss, and fever. […] The pathogenesis of sclerosing mesenteritis remain unclear, although several mechanisms have been postulated. […] It is hypothesized that sclerosing mesenteritis results in genetically predisposed individuals who have abnormal responses to healing and repair of connective tissue in response to trauma. In a systematic review of the published literature, previous abdominal surgery or abdominal trauma was described in almost 30 percent of patients. In one series of 92 patients with sclerosing mesenteritis, 38 patients (41 percent) had prior abdominal surgery, including cholecystectomy, appendectomy, hysterectomy, and colectomy. Other studies, however, have not found an association with prior abdominal surgery or trauma. Sclerosing mesenteritis has been reported in association with both acute abdominal injury and chronic repetitive trauma (eg, pneumatic jackhammer) and inflammation (eg, occult ileal perforation). The use of powdered surgical gloves has also been implicated in the development of abdominal fibrosis as a precursor to sclerosing mesenteritis.
  • #2 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. Its etiology is unknown, the pathogenesis is obscure, while the pathological characteristics of the disease are unspecific. […] The pathophysiology of SM remains unknown. The pathogenic mechanism seems to be a non-specific response to a wide variety of stimuli. It has been reported on in one series in which 84% of patients had a history of previous abdominal trauma or surgery. […] Autoimmune and infective causes such as abdominal tuberculosis as well as vascular insufficiency (mesenteric thrombosis, mesenteric arteriopathy, previous surgery, trauma) and retained suture material have all been implicated as etiological factors. […] The disease is often asymptomatic and indeed most patients in any given series were incidentally identified during a CT examination.
  • #2 Sclerosing Mesenteritis: Definition, Types & Treatment
    https://my.clevelandclinic.org/health/diseases/15284-sclerosing-mesenteritis
    Sclerosing mesenteritis is a chronic inflammatory condition that affects your mesentery, which is part of your peritoneum. It causes fibrosis (scarring) of the tissues. The inflammation in this condition is idiopathic. It may be a type of autoimmune response. […] Sclerosing mesenteritis causes chronic inflammation of the mesentery. Over time, chronic inflammation causes scarring or fibrosis of the tissues, making them harden. […] It takes consistent inflammation over a long period of time to cause sclerosis. It happens in stages: first, the fat begins to break down, then different types of cells begin to infiltrate the tissue, and finally, scar tissue develops. So, by definition, sclerosing mesenteritis is a chronic condition and a progressive one. […] The exact cause is unknown, but healthcare providers suspect that it’s a type of autoimmune disease. That means the inflammation is an inappropriate or overreactive immune response.
  • #2 Sclerosing Mesenteritis-Update on Diagnostic and Therapeutic Appr
    https://www.itmedicalteam.pl/articles/sclerosing-mesenteritisupdate-on-diagnostic-and-therapeutic-approach-108510.html
    The possible autoimmune etiology is supported by a significant clinical response to immunomodulatory drugs. […] The ischemic lesions are described correlated with the previous surgery, abdominal trauma, mesenteric thrombosis and mesenteric arteriopathy. […] Initially a degenerative process of mesenteric fat, followed by inflammation (panniculitis). Later, develops and fibrosis the retractable mesenteritis appears. […] The inflammatory process-fibrotic can affect the gastrointestinal lumen and the blood vessels and mesenteric lymph nodes, causing injury for mass effect, which may result in partial bowel obstruction, ischemia and ascites quilosa, justifying the diverse clinical gastrointestinal and systemic. […] The SM has been described in association with a number of conditions, including autoimmune thyroiditis, presumably of Riedel, primary sclerosing cholangitis, retroperitoneal fibrosis, and orbital pseudotumor.
  • #2 Mesenteritis esclerosante relacionada con IgG4, una entidad rara causante de dolor abdominal
    https://scielo.isciii.es/scielo.php?pid=S1130-01082018000300014&script=sci_arttext&tlng=en
    IgG4-related sclerosing mesenteritis is an inflammatory, fibrosing condition of an unknown origin. Cases have recently been reported with tissue infiltration by IgG4 plasma cells. This might be considered part of the IgG4-related disease spectrum, although further studies are required. […] The etiology is unknown but has been related to mechanisms such as abdominal trauma, surgery, autoimmunity, ischemia, infection and paraneoplastic syndrome. […] It was recently suggested that most sclerosing „idiopathic” conditions might belong within the IgG4-related disease spectrum. This is an immune-mediated systemic disorder characterized by the presence of lymphoplasmacytic infiltration rich in IgG4 plasma cells, storiform fibrosis, obliterative phlebitis and tissue eosinophilia. The pathogenesis remains unknown but the presence of a type of trigger has been suggested, which stimulates B-cell infiltration. These cells then differentiate into IgG4 plasma cells in response to mediators (IL-4 and IL-13) secreted by Th2 lymphocytes. B-cells and plasmablasts act as antigen-presenting cells to CD4 T-cells, which in turn release the substances that cause fibrosis.
  • #2 IgG4-related sclerosing mesenteritis causing bowel obstruction: a case report | Surgical Case Reports | Full Text
    https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-016-0248-0
    Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. […] SM was recently reported to be closely related to IgG4-related disease (IgG4-RD), a systemic syndrome characterized by masses in various organs infiltrated by IgG4-positive plasma cells and high serum IgG4 concentrations. […] The pathogenic mechanism of SM seems to be a non-specific response to a wide variety of stimuli. […] SM may be an IgG4-RD, diseases that dramatically respond to corticosteroid treatment. […] Although the incidence of SM related to IgG4-RD has not been determined, SM was observed in 2 (4 %) of 57 patients with autoimmune pancreatitis and marked infiltration of IgG4-positive plasma cells was observed in 4 (33 %) of 12 patients with SM. […] The comprehensive diagnostic criteria for IgG4-RD require imaging and serum and histopathological examination.
  • #2 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) is a rare disease of the mesentery characterized by chronic inflammation and fibrosis of the mesentery which was first described by Jura as retractile mesenteritis in 1924 and the term sclerosing mesenteritis was given by Emory et al. The exact mechanism is unknown; however, several conditions have been associated with SM. The study by Sharma et al. on 192 cases showed that 28.6% of the cases had a history of abdominal trauma or prior surgery, 8.9% had a history of cancer and 5.7% had underlying autoimmune disease. Some authors have implicated the association of SM with IgG4 related disease. IgG4 related disease which is a fibroinflammatory condition is characterized by formation of tumor-like growth in multiple sites of the body and elevated IgG4 level in the serum. Other studies have shown the association of SM with carcinomas like breast, colorectal, gynecological and urological cancers and hematological malignancies like Non-Hodgkins Lymphoma and Multiple Myeloma. Sclerosing Mesenteritis is characterized by fat degeneration, necrosis, chronic inflammation, scarring and eventually fibrosis of the fatty tissue within the mesentery.
  • #2 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 exact diagnosis is often difficult and is usually made by finding one of three major pathological features; fibrosis, chronic inflammation or fatty infiltration of the mesentery. […] The first stage involves mesenteric lipodystrophy in which a layer of foamy macrophages replaces the mesenteric fat. […] The final stage is retractile mesenteritis which is distinguished by collagen deposition and a diffuse presence of necrosis and fibrosis that contribute to tissue retraction. […] There is no consensus of opinion on medical treatment for symptomatic cases of SM. A variety of anti-inflammatory, immunomodulatory, and antifibrotic agents are used. […] In the first case of our series the combined use of corticosteroids and methotrexate against rheumatoid arthritis probably maintained SM in a tolerant state. […] Akram et al consider that the management of the disease should be based on severity and type of symptoms and not on CT findings.
  • #2 Sclerosing Mesenteritis: A Review Article | Insight Medical Publishing
    https://raredisorders.imedpub.com/articles/sclerosing-mesenteritis-a-review-article.php?aid=23602
    Sclerosing mesenteritis is a rare chronic inflammatory condition characterized by mesenteric fibrosis. […] The etiology of sclerosis mesenteritis (SM) is unknown. […] The pathophysiology of SM is likewise unclear. Given the typical presence of foamy macrophages on histopathology, it has been hypothesized that molecular pathways involving foam cell formation, including upregulation of peroxisome proliferator-activated receptor-gamma (PPAR-gamma), may play a role. […] A recent small series of case reports further suggests a relationship between dysfunctional glucose metabolism and the molecular mechanism of SM. […] Chronic inflammation and fat necrosis have been identified in the adipocyte tissue of both populations. […] Diagnosis is dependent on histology, which is demonstrative of inflammation, fat necrosis, and fibrosis, with the latter being a very regular feature.
  • #2 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. […] A number of pharmaceutical agents have been used in an attempt to stabilize the disease. Glucocorticoids, azathioprine, methotrexate, colchicine, cyclophosphamide, and/or radiotherapy have been proposed as therapeutic options. The patients with a greater inflammatory component, with fever, weight loss, and general malaise appear to be the most receptive to glucocorticoids, alone or in combination.
  • #2 Sclerosing mesenteritis as a surgical problem: a review of the literature and own clinical observation
    https://ouci.dntb.gov.ua/en/works/4M052Ld7/
    The problem of studying the pathology of mesenteric fat of the small intestine in surgical practice has not yet received enough attention. […] Issues of etiology, pathogenesis, differential diagnosis of this disease and its possible connection with the paraneoplastic process currently continue to cause debate. […] Further accumulation of clinical experience, a better understanding of the pathogenesis of the disease, and improvement of imaging techniques will allow us to develop clearer diagnostic and clinical criteria, narrow the diagnostic search and, ultimately, improve and standardize treatment.
  • #3 Sclerosing Mesenteritis – PubMed
    https://pubmed.ncbi.nlm.nih.gov/30829677/
    Sclerosing mesenteritis is a rare non-neoplastic disorder characterized by fat necrosis, chronic inflammation, and fibrosis typically of the small bowel mesentery. […] While prior abdominal surgery or trauma, autoimmunity, infection, ischemia, and malignancy have been suggested to be involved in the pathogenesis of the disorder, it remains poorly understood. […] A careful use of terminology and communication between the radiologist, pathologist, and clinicians in the care of these patients will be essential to future efforts at understanding this disease.
  • #3 Sclerosing mesenteritis – UpToDate
    https://www.uptodate.com/contents/sclerosing-mesenteritis/print
    Sclerosing mesenteritis is a rare, non-neoplastic inflammatory and fibrotic disease that affects the mesentery. […] The pathogenesis of sclerosing mesenteritis remain unclear, although several mechanisms have been postulated. […] It is hypothesized that sclerosing mesenteritis results in genetically predisposed individuals who have abnormal responses to healing and repair of connective tissue in response to trauma. […] In a systematic review of the published literature, previous abdominal surgery or abdominal trauma was described in almost 30 percent of patients. […] Sclerosing mesenteritis has been reported in association with both acute abdominal injury and chronic repetitive trauma (eg, pneumatic jackhammer) and inflammation (eg, occult ileal perforation). […] The use of powdered surgical gloves has also been implicated in the development of abdominal fibrosis as a precursor to sclerosing mesenteritis.