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Sclerosing Cavity Linings:

If you are a patient or member of a family with a patient and are worried about the future, read the following extremely carefully & realize that NO ONE can predict the future for a single, particular patient. The best the field of medicine can do is about like an odds maker in any gambling activity. An educated guess is given. So, don't forget The Almighty & prayer (MIRACLES).

This topic was brought to my attention in the summer of 2017. Scarring, sclerosis, and fibrosis are synonyms and can be adjectives or nouns. The associated noun for the abdominal area could be "peritonitis"...or one could say peritoneal scarring, sclerosis, and fibrosis. The three (3) human cavity mesothelial linings are the ones (1) around the lungs (pleura), (2) around the heart (pericardial), and (3) around the intestines, liver, pancreas, spleen and female organs (peritoneal). In those cavities, the lining is normally extremely thin & transparent and covers all organs & the chest wall, the pericardial sac wall, and abdominal cavity wall. Keep in mind that people are different. Most mesothelial-lined serosal cavities are highly resistant to sclerosing reactions (especially generalized scarring reactions), but some cavities scar much more easily. UNFORTUNATELY, the only write-ups you will see on the internet are about devastating cases no matter what percentage of patients are predicted to do worse.

  • References: As to how often devastating cases of EPS are seen, a survey of 18 surgical centers in 5 countries...covering a period averaging 16 years...discovered 32 cases EPS as noted below with findings impressive enough to entitle ENCAPSULATING PERITONEAL SCLEROSIS [EPS]: DEFINITION, ETIOLOGY, DIAGNOSIS, AND TREATMENT by Kawaguchi, et. al., Sept. 2000, vol. 20., suppl. 4 of Peritoneal Dialysis International, pages S43-S55, HERE [URL = https://www.researchgate.net/publication/12228871_Encapsulating_peritoneal_sclerosis_Definition_etiology_diagnosis_and_treatment_International_Society_for_Peritoneal_Dialysis_Ad_Hoc_Committee_on_Ultrafiltration_Management_in_Peritoneal_Dialysis]). This study almost certainly did NOT include cases of fibrosis (sclerosis) that were less impressive than to provoke useage of the adjective, "encapsulated" (as in EPS).
  • Jeffrey RB, Jr, Imaging of the Peritoneal Cavity, Curr. Opin Radiol. 1991 June, vol. 3 #3, pages 471-473.
  • Kim KK, et. al., Ileal Obstruction Caused by Idiopathic Sclerosing Encapsulating Peritonitis, Abdom. Imaging Jan-Feb 1999, vol 24 #1, pages 82-84.
  • Abboud BN, et. al., Idiopathic Sclerosing Encapsulating Peritonitis: Abdominal Cocoon. World J. Gastroenterology, 2012 May 7, vol 18 (URL = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3342596/ HERE).
  • Solak L, et. al., Abdominal Cocoon Syndrome: Preoperative Diagnosic Criteria, Good Clinical Outcome with Medical Treatment and Review of the Literature; Turk J. Gastroenterology, 2012 vol 23 #6, pages 776-779 (URL = https://www.ncbi.nlm.nih.gov/pubmed/23864454 HERE).
  • Frederiksen JK, et. al., IgG4-Related Disease: a Reminder for Practicing Pathologists, Nov. 2017 p.1476-1483, Arch. Path. & Lab. Med., vol. 141 #11.

Prognosis: When lesser cases are encountered in practice, the question may come up as to "What is the prognosis for the future?"...Is my future at risk & how much risk?" The answer is that the risk of progression to EPS is vanishingly rare.

The various possible causitive conditions can be limited to one cavity or could involve the other two. Generally speaking, the medical field has three broad categories of causes of diseases: (1) idiopathic (meaning that there is no scientifc agreement on a cause...sometimes the word "primary" is used rather than "idiopathic"). EXTREMELY importantly, prognosis and treatment outcomes depend on how widespread the process is in one or more of the THREE cavities. That is, there is a wide spectrum for interpretation and worries about prognosis. In any one or two or all three cavities, the scarring (sclerosis) can focal (small patches or bands) vs. broadly patchy or segmental (as in a band around only one area of intestine) or diffuse (when this extent is reached, one will see descriptive terms such as "encapsulating sclerosis" used).

Physicians: In addition to the primary care physician, a gastroenterologist might be involved if abdominal pains or nausea & vomiting. A surgeon may have to deal with anything that seems like intestinal obstruction. A rheumatologist may well be involved, especially when the case some not straight-forward as to cause.

Lab work: when working up cavity scarring, one would likely see all sorts of routine lab testing plus ESR & CRP (inflammation markers), IgG subclass 4, and IgG subclass 4 special stain in a reference lab on any surgically removed tissue from within the abdomin. In the absence of an easy, straightforward diagnosis, the autoimmune screening test for ANA is likely to be ordered (casting "a broad net" for a diagnostic lead in an uncertain situation), and it will be the fortunate patient who benefits from HIGHLY expert autoimmune testing which can define confusing causes of ANA positivity (such as the DSF70 variant which almost never are indicative of real diusease unless there are some additional ENA positivities...we use a 15 ENAs panel; any additional ENAs then define the disease). FMF genetic testing may be done.

  1. Idiopathic/primary, unknown-etiology sclerosing (pleuritis, pericarditis, peritonitis): four of the 32 cases. No blame could be pinpointed for 4 cases.
  2. Secondary sclerosing (pleuritis, pericarditis, peritonitis)(a possible "blame" cause is found):
    • Peritoneal-dialysis-associated: in renal failure patients on this type of dialysis (4 of the 32 cases).
    • Malignancy-associated: this might be cancer-associated (paraneoplastic) or by direct cancer involvement (direct involvement by cancer would be seen in the pathology frozen section or final pathology exam).
    • Medication-associated: beta blockers were associated with 4 of 32 cases reported in 2000.
    • Infectious-associated: this cause follows generalized peritonitis (TB & other agents) & would usually have proportionately elevated ESR & CRP); this was 4 of those 32 cases.
    • Sugery-associated: post-operative scarring & adhesions and different patients are more susceptible & others less so. CRP may be elevated in the attack phase of some surgery cases and may be the only acute phase reactant elevated (ESR may be only slightly elevated unless there is an infectious component).Nineteen (19) of those 32 cases (59%) reported in 2000 had had prior surgery. It appears that some folks (rare cases) have as-yet-unexplainable, amazingly impressive, personal, individual, constitutional serosal sensitivity & reactivity to the organ manipulation during surgery!
    • Familial Mediterranean Fever Associated: One in 1000 persons have FMF worldwide, HERE (URL = https://rarediseases.info.nih.gov/diseases/6421/familial-mediterranean-fever ). There is usually (1) a positive family history of FMF and (2) periodic unexplained fevers. Genomic testing will identify 99 out of 100 cases (that one other due to a rare gene variant). Unless there is considerable STRONG evidence in favor of FMF, negative genomic sequencing tends to exclude FMF as the cause. The genomic test info at our reference lab in July 2017, HERE (URL = http://ltd.aruplab.com/Tests/Pub/2002658). CRP is elevated in the attack phase of all FMF cases and may be the only acute phase reactant elevated (ESR may be only slightly elevated).
    • Endometriosis-associated: usually patchy and only in pelvic peritoneal cavity and only in females. Elevations of ESR &/or CRP are unlikely. But, endometriosis or other less extensive ovarion lesions are said to be able to provoke enough reaction to cause a case of EPS (though none were collected in the above study).
    • Liver cirrhosis-with-ascites associated: Four of those 32 cases fit here.
    • All other causes: sarcoidosis, pariculate matter into the cavity space (talc, asbestos, silica); peritoneal shunts and/or chemical lavage; peritoneal chemotherapy; certain GI diseases; abdominal radiation (tends to have localized effects). Could have some CRP elevation by ESR elevation doubtful.
  3. Systemic sclerosis (scleroderma) syndrome: This syndrome most often affects skin but is variable and can scar internal organs (all due to a build-up of collagen connective tissue. Our specialized auto-immune lab testing plus radiology studies help lead to a correct diagnosis, HERE (URL = https://rarediseases.info.nih.gov/diseases/9748/systemic-scleroderma). That very specialized autoimmune testing, if "positive", has a high specificity (true positives) but a low sensitivity (many “false negs”)—a characteristic of the “Systemic Sclerosis panel" (which tests for some markers not included in our 15 agent ENA profile).
  4. Ormond's Disease: Otherwise known as "retroperitoneal fibrosis" (URL = https://rarediseases.info.nih.gov/diseases/9568/retroperitoneal-fibrosis), this is scarring deep to the cavity linings and focused toward the back so that it affects the kidney outflow of urine and the flow of blood & lymphatic vessels. About 33% of these cases have IgG subclass 4 positive plasma cells (HERE [URL = https://www.ncbi.nlm.nih.gov/pubmed/27125330]) with serum elevation & prominent mix of lymphocytes & plasma cells by H&E, & are diagnosed as "IgG4-related retroperitoneal fibrosis", while 66% are idiopathic.
  5. Mesenteric fibrosis: Otherwise known as sclerosing mesenteritis, this scarring primarily affects the abdominal mesentery onto which all of the small intestine is attached; it has an array of causes, HERE (URL = https://www.uptodate.com/contents/sclerosing-mesenteritis)(one cause of which can be an autoimmune disease...therefore ANA testing might be part of any abdominal cavity fibrosis workup).



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(posted 28 July 2017; additions 1 August 2017 & 2 Dec. 2017)