encapsulating peritoneal sclerosis

Patients usually present with abdominal symptoms such as altered bowel habits, nausea, vomiting, anorexia, and early satiety.{{cite journal |last1=Nakamoto |first1=Hidetomo |title=Encapsulating peritoneal sclerosis--a clinician's approach to diagnosis and medical treatment |journal=Peritoneal Dialysis International |date=April 2005 |volume=25 |issue=4 |pages=S30–S38 |doi=10.1177/089686080502504S05 |pmid=16300270|s2cid=18937047 }} In the early stages, these symptoms can be linked to signs of inflammation such as pyrexia and elevated CRP, and/or blood-stained ascites.{{cite journal |last1=Maruyama |first1=Yukio |last2=Nakayama |first2=Masaaki |title=Encapsulating peritoneal sclerosis in Japan |journal=Peritoneal Dialysis International |date=June 2008 |volume=28 |issue=3 |pages=S201–S204 |doi=10.1177/089686080802803s37 |pmid=18552256|s2cid=45478695 }}

Abdominal pain, fullness, overt bowel obstruction, and the presence of an abdominal mass are linked to the late stages of encapsulating peritoneal sclerosis. The intestines become gradually covered with a fibrous cocoon, which causes weight loss, malnutrition, bowel obstruction, ischemia and strangulation, infection, and death.

Encapsulating peritoneal sclerosis is typically observed in patients with end-stage renal disease (ESRD) receiving long-term peritoneal dialysis therapy. Dialysis fluid's high glucose content and acidic pH cause harm to the peritoneum.{{cite journal | last1=Moinuddin | first1=Zia | last2=Summers | first2=Angela | last3=Van Dellen | first3=David | last4=Augustine | first4=Titus | last5=Herrick | first5=Sarah E. | title=Encapsulating peritoneal sclerosisâ€"a rare but devastating peritoneal disease | journal=Frontiers in Physiology | publisher=Frontiers Media SA | volume=5 | date=January 5, 2015 | issn=1664-042X | doi=10.3389/fphys.2014.00470 | doi-access=free | page=470| pmid=25601836 | pmc=4283512 }} High glucose concentrations promote osmosis and diffusion gradients across the peritoneum, while low pH inhibits the production of harmful glucose degradation products (GDPs).{{cite journal |last1=Jörres |first1=A |last2=Topley |first2=N |last3=Gahl |first3=G M |title=Biocompatibility of peritoneal dialysis fluids |journal=The International Journal of Artificial Organs |date=February 1992 |volume=15 |issue=2 |pages=79–83 |doi=10.1177/039139889201500203 |pmid=1555880}} GDPs are created when peritoneal dialysis fluid is heated to sterilize it{{cite journal |last1=Wieslander |first1=A P |title=Cytotoxicity of peritoneal dialysis fluid — is it related to glucose breakdown products? |journal=Nephrology, Dialysis, Transplantation|date=June 1996 |volume=11 |issue=6 |pages= 958–959|pmid=8671949}} and these result in the production of advanced glycation end products (AGEs) when glucose is present.{{cite journal | last1=Mortier | first1=Siska | last2=De Vriese | first2=An S. | last3=Van de Voorde | first3=Johan | last4=Schaub | first4=Thomas P. | last5=Passlick-Deetjen | first5=Jutta | last6=Lameire | first6=Norbert H. | title=Hemodynamic Effects of Peritoneal Dialysis Solutions on the Rat Peritoneal Membrane | journal=Journal of the American Society of Nephrology | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=13 | issue=2 | year=2002 | issn=1046-6673 | doi=10.1681/asn.v132480 | pages=480–489| pmid=11805178 }} Less GDP-containing biocompatible solutions are now more frequently used, which lessens peritoneal damage.{{cite journal |last1=Boulange |first1=Eric |title=Peritoneal and systemic inflammation: the benefits of using biocompatible peritoneal dialysis fluids |journal=Peritoneal Dialysis International |date=2008 |volume=28 |issue=1 |pages=28–31 |doi=10.1177/089686080802800106 |pmid=18178944}}

Encapsulating peritoneal sclerosis can also occur in patients who are not on peritoneal dialysis but are suffering from other illnesses like endometriosis, sarcoidosis, peritoneal and intra-abdominal cancers, chronic peritoneal ascites, intraperitoneal chemotherapy, intraperitoneal exposure to particulate matter or disinfectant, abdominal surgery, intraperitoneal infections (tuberculosis), and beta-blocker administration.{{cite journal |last1=Pollock |first1=C A |title=Diagnosis and management of encapsulating peritoneal sclerosis |journal=Peritoneal Dialysis International |date=2001 |volume=21 |issue=3 |pages=S61–S66 |doi=10.1177/089686080102103S10 |pmid=11887865}}{{cite journal |last1=Kawanishi |first1=Hideki |last2=Moriishi |first2=Misaki |title=Epidemiology of encapsulating peritoneal sclerosis in Japan |journal=Peritoneal Dialysis International |date=April 2005 |volume=25 |issue=4 |pages=S14–S18 |pmid=16300268}}

The length of peritoneal dialysis treatment appears to be the primary risk factor for encapsulating peritoneal sclerosis development. The incidence of encapsulating peritoneal sclerosis rose with the length of peritoneal dialysis (PD) in an Australian survey; for patients on PD for more than 2, 5, 6, and 8 years, the rates were 1.9, 6.4, 10.8, and 19.4%, respectively.{{cite journal | last1=Rigby | first1=R. J. | last2=Hawley | first2=C. M. | title=Sclerosing peritonitis: the experience in Australia | journal=Nephrology Dialysis Transplantation | publisher=Oxford University Press (OUP) | volume=13 | issue=1 | date=January 1, 1998 | issn=0931-0509 | doi=10.1093/ndt/13.1.154 | pages=154–159| pmid=9481732 }}

Given that there is a high incidence of encapsulating peritoneal sclerosis shortly after renal transplantation, organ transplantation seems to increase the risk of developing this condition.{{cite journal | last1=Balasubramaniam | first1=Gowrie | last2=Brown | first2=Edwina A. | last3=Davenport | first3=Andrew | last4=Cairns | first4=Hugh | last5=Cooper | first5=Barbara | last6=Fan | first6=Stanley L. S. | last7=Farrington | first7=Ken | last8=Gallagher | first8=Hugh | last9=Harnett | first9=Patrick | last10=Krausze | first10=Sally | last11=Steddon | first11=Simon | title=The Pan-Thames EPS study: treatment and outcomes of encapsulating peritoneal sclerosis | journal=Nephrology Dialysis Transplantation | publisher=Oxford University Press (OUP) | volume=24 | issue=10 | date=February 11, 2009 | issn=1460-2385 | doi=10.1093/ndt/gfp008 | pages=3209–3215| pmid=19211652 }}

A frequent side effect of peritoneal dialysis, peritonitis is intricately linked to the development of encapsulating peritoneal sclerosis, with the frequency of episodes being correlated with the occurrence of encapsulating peritoneal sclerosis.{{cite journal | last1=Yamamoto | first1=Ryo | last2=Otsuka | first2=Yasushi | last3=Nakayama | first3=Masaaki | last4=Maruyama | first4=Yukio | last5=Katoh | first5=Naohiko | last6=Ikeda | first6=Masato | last7=Yamamoto | first7=Hiroyasu | last8=Yokoyama | first8=Keitaro | last9=Kawaguchi | first9=Yoshindo | last10=Matsushima | first10=Masato | title=Risk factors for encapsulating peritoneal sclerosis in patients who have experienced peritoneal dialysis treatment | journal=Clinical and Experimental Nephrology | publisher=Springer Science and Business Media LLC | volume=9 | issue=2 | date=June 24, 2005 | issn=1342-1751 | doi=10.1007/s10157-005-0349-8 | pages=148–152| pmid=15980950 | s2cid=26144928 }} Encapsulating peritoneal sclerosis has been specifically linked to recurrent peritonitis caused by bacterial contamination,{{cite journal | last1=Chew | first1=C. G. | last2=Clarkson | first2=A. R. | last3=Faull | first3=R. J. | title=Relapsing CAPD peritonitis with rapid peritoneal sclerosis due to Haemophilus influenzae | journal=Nephrology Dialysis Transplantation | publisher=Oxford University Press (OUP) | volume=12 | issue=4 | date=April 1, 1997 | issn=0931-0509 | doi=10.1093/ndt/12.4.821 | pages=821–822| pmid=9141024 }} specifically from Pseudomonas spp., Staphylococcus aureus, and specific fungal{{cite journal | last1=Flanigan | first1=Michael | last2=Anderson | first2=Dale | last3=Freeman | first3=Richard M. | title=Peritoneal dialysis complicated by fungal peritonitis and peritoneal fibrosis | journal=The American Journal of Medicine | publisher=Elsevier BV | volume=76 | issue=5 | year=1984 | issn=0002-9343 | doi=10.1016/0002-9343(84)91017-9 | page=A113| pmid=6720720 }} organisms.

Implicated triggers include systemic rheumatologic and inflammatory disorders,{{cite journal | last1=Ngô | first1=Yann | last2=Messing | first2=Bernard | last3=Marteau | first3=Philippe | last4=Nouël | first4=Olivier | last5=Pasquiou | first5=Alain | last6=Lavergne | first6=Anne | last7=Rambaud | first7=Jean Claude | title=Peritoneal sarcoidosis | journal=Digestive Diseases and Sciences | publisher=Springer Science and Business Media LLC | volume=37 | issue=11 | year=1992 | issn=0163-2116 | doi=10.1007/bf01299875 | pages=1776–1780| pmid=1425080 | s2cid=28350203 }}{{cite journal | last=Dabak | first=Resat | title=Encapsulating peritonitis and familial Mediterranean fever | journal=World Journal of Gastroenterology | publisher=Baishideng Publishing Group Inc. | volume=11 | issue=18 | year=2005 | pages=2844–2846 | issn=1007-9327 | doi=10.3748/wjg.v11.i18.2844 | doi-access=free | pmid=15884137 | pmc=4305931 }} dermoid cyst rupture,{{cite journal | last1=Fossey | first1=SJ | last2=Simson | first2=JNL | title=Sclerosing encapsulating peritonitis secondary to dermoid cyst rupture: a case report | journal=The Annals of the Royal College of Surgeons of England | publisher=Royal College of Surgeons of England | volume=93 | issue=5 | year=2011 | issn=0035-8843 | doi=10.1308/147870811x582495 | pages=e39–e40| pmid=21943446 | pmc=5827212 }} gynecologic neoplasms,{{cite journal | last1=Bahar | first1=Burak | last2=Hu | first2=Zhihong | last3=Szpaderska | first3=Anna | last4=Liotta | first4=Margaret | last5=Potkul | first5=Ronald K. | last6=Smith | first6=Donna | last7=Erşahin | first7=Çağatay | title=Fatal Case of Luteinized Thecoma With Sclerosing Peritonitis in a 40-Year-Old Woman | journal=International Journal of Gynecological Pathology | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=33 | issue=1 | year=2014 | issn=0277-1691 | doi=10.1097/pgp.0b013e31827d1a65 | pages=30–34| pmid=24300532 | s2cid=27140004 }}{{cite journal | last1=Walker | first1=Jane | last2=Moss | first2=Esther L. | last3=Ganesan | first3=Raji | last4=Hirschowitz | first4=Lynn | title=Sclerosing Peritonitis Associated With a Luteinized Adult Granulosa Cell Tumor | journal=International Journal of Gynecological Pathology | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=31 | issue=2 | year=2012 | issn=0277-1691 | doi=10.1097/pgp.0b013e3182307b28 | pages=141–144| pmid=22317869 }} endometriosis,{{cite journal | last1=Frigerio | first1=Luigi | last2=Taccagni | first2=Gian Luca | last3=Mariani | first3=Andrea | last4=Mangili | first4=Giorgia | last5=Ferrari | first5=Augusto | title=Idiopathic sclerosing peritonitis associated with florid mesothelial hyperplasia, ovarian fibromatosis, and endometriosis: A new disorder of abdominal mass | journal=American Journal of Obstetrics and Gynecology | publisher=Elsevier BV | volume=176 | issue=3 | year=1997 | issn=0002-9378 | doi=10.1016/s0002-9378(97)70581-7 | pages=721–722| pmid=9077640 }} organ transplantation,{{cite journal | last1=Lee | first1=Kyo Won | last2=Cho | first2=Chan Woo | last3=Lee | first3=Nuri | last4=Lee | first4=Sanghoon | last5=Kim | first5=Jong Man | last6=Choi | first6=Gyu-Seong | last7=Kwon | first7=Choon Hyuck David | last8=Joh | first8=Jae-Won | last9=Lee | first9=Suk-Koo | title=Encapsulating peritoneal sclerosis in liver transplant recipients: a report of 2 cases | journal=Annals of Surgical Treatment and Research | publisher=The Korean Surgical Society | volume=92 | issue=3 | year=2017 | pages=164–167 | issn=2288-6575 | doi=10.4174/astr.2017.92.3.164 | pmid=28289671 | pmc=5344807 }}{{cite journal | last1=Morrow | first1=Ellen H. | last2=Gallo | first2=Amy E. | last3=Melcher | first3=Marc L. | title=Sclerosing Peritonitis After Kidney Transplantation: A Not-So-Silky Cocoon | journal=Digestive Diseases and Sciences | publisher=Springer Science and Business Media LLC | volume=56 | issue=2 | date=November 10, 2010 | issn=0163-2116 | doi=10.1007/s10620-010-1471-3 | pages=307–310| pmid=21063775 | s2cid=22601942 }}{{cite journal | last1=Rumbo | first1=Carolina | last2=Zambernardi | first2=Agustina | last3=Cabanne | first3=Ana | last4=Rumbo | first4=Martin | last5=Gondolesi | first5=Gabriel | title=Sclerosing peritonitis, a rare complication after intestinal transplant. Report of one case successfully treated with adjustment of immunosuppression | journal=Pediatric Transplantation | publisher=Wiley | volume=17 | issue=5 | date=July 3, 2013 | pages=E125-9 | issn=1397-3142 | doi=10.1111/petr.12110 | pmid=23902605 | hdl=11336/23404 | s2cid=21314087 | hdl-access=free }} cirrhosis,{{cite journal | last1=Wakabayashi | first1=Hisao | last2=Okano | first2=Keiichi | last3=Suzuki | first3=Yasuyuki | title=Clinical Challenges and Images in GI | journal=Gastroenterology | publisher=Elsevier BV | volume=132 | issue=3 | year=2007 | issn=0016-5085 | doi=10.1053/j.gastro.2007.01.060 | pages=854–1210| pmid=17383416 }} mechanical or chemical intraperitoneal irritants,{{cite journal | last1=Takebayashi | first1=Katsushi | last2=Sonoda | first2=Hiromichi | last3=Shimizu | first3=Tomoharu | last4=Ohta | first4=Hiroyuki | last5=Ishida | first5=Mitsuaki | last6=Mekata | first6=Eiji | last7=Endo | first7=Yoshihiro | last8=Tani | first8=Tohru | last9=Tani | first9=Masaji | title=Successful surgical approach for a patient with encapsulating peritoneal sclerosis after hyperthermic intraperitoneal chemotherapy: a case report and literature review | journal=BMC Surgery | publisher=Springer Science and Business Media LLC | volume=14 | issue=1 | date=August 27, 2014 | issn=1471-2482 | doi=10.1186/1471-2482-14-57 | doi-access=free | page=57| pmid=25160862 | pmc=4149037 }}{{cite journal | last1=Sigaroudinia | first1=Mandana O. | last2=Baillie | first2=Colin | last3=Ahmed | first3=Shiban | last4=Mallucci | first4=Connor | title=Sclerosing encapsulating peritonitis—a rare complication of ventriculoperitoneal shunts | journal=Journal of Pediatric Surgery | publisher=Elsevier BV | volume=43 | issue=5 | year=2008 | issn=0022-3468 | doi=10.1016/j.jpedsurg.2008.01.019 | pages=e31–e33}}{{cite journal | last1=Arnadottir | first1=M. | last2=Jonasson | first2=J. 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S. | title=Encapsulating peritoneal sclerosis following a peritoneal foreign body reaction to Dacron fibres--a case report | journal=Clinical Kidney Journal | publisher=Oxford University Press (OUP) | volume=4 | issue=2 | date=February 11, 2011 | issn=2048-8505 | doi=10.1093/ndtplus/sfq202 | pages=107–109| pmid=25984126 | pmc=4421583 }} infection,{{cite journal | last1=Hsu | first1=Yung-Hsuen | last2=Hsia | first2=Ching-Chih | last3=Tsai | first3=Dong-Ming | last4=Tu | first4=Hsing-Yang | last5=Hung | first5=Kuan-Yu | last6=Huang | first6=Jenq-Wen | title=Development of Encapsulating Peritoneal Sclerosis Following Bacterial Peritonitis in a Peritoneal Dialysis Patient | journal=American Journal of Kidney Diseases | publisher=Elsevier BV | volume=55 | issue=1 | year=2010 | issn=0272-6386 | doi=10.1053/j.ajkd.2009.06.043 | pages=198–202| pmid=19782452 }}{{cite journal | last1=Tan | first1=J. | last2=Manickam | first2=R. | last3=Pisharam | first3=J. | last4=Telisinghe | first4=P. | last5=Chong | first5=V.H. | title=Mucormycosis—A Possible Trigger Pathogen for Encapsulating Peritoneal Sclerosis | journal=Peritoneal Dialysis International | publisher=SAGE Publications | volume=32 | issue=4 | year=2012 | issn=0896-8608 | doi=10.3747/pdi.2011.00241 | pages=479–481| pmid=22859845 | pmc=3524856 }} and medications.{{cite journal | last1=Eltringham | first1=W K | last2=Espiner | first2=H J | last3=Windsor | first3=C W O | last4=Griffiths | first4=D A | last5=Davies | first5=J D | last6=Baddeley | first6=H | last7=Read | first7=A E A | last8=Blunt | first8=R J | title=Sclerosing peritonitis due to practolol: A report on 9 cases and their surgical management | journal=British Journal of Surgery | publisher=Oxford University Press (OUP) | volume=64 | issue=4 | year=1977 | issn=0007-1323 | doi=10.1002/bjs.1800640402 | pages=229–235| pmid=856375 | s2cid=33419488 }}{{cite journal | last1=Nauen | first1=David W. | last2=Martin | first2=Allison | last3=Katz | first3=Aviva | last4=Cohen | first4=Debra | last5=Ranganathan | first5=Sarangarajan | title=A case of luteinizing thecoma with sclerosing peritonitis: Revisiting a link with anti-epileptic drugs | journal=Pediatric Blood & Cancer | publisher=Wiley | volume=54 | issue=3 | date=October 21, 2009 | issn=1545-5009 | doi=10.1002/pbc.22325 | pages=470–472| pmid=19847882 | s2cid=20286106 }}

Encapsulating peritoneal sclerosis is believed to happen in patients who have a predisposing condition if a peritoneal inflammatory process (inciting factor) takes place. This is known as the "two-hit" theory in the literature on peritoneal dialysis, according to which the non-inflammatory peritoneal sclerosis brought on by repeated dialysis treatments is the "first hit" or predisposing factor.{{cite journal | last1=Alston | first1=Helen | last2=Fan | first2=Stanley | last3=Nakayama | first3=Masaaki | title=Encapsulating Peritoneal Sclerosis | journal=Seminars in Nephrology | publisher=Elsevier BV | volume=37 | issue=1 | year=2017 | issn=0270-9295 | doi=10.1016/j.semnephrol.2016.10.010 | pages=93–102| pmid=28153198 }} This is corroborated by the fact that during peritoneal dialysis, the cumulative incidence of encapsulating peritoneal sclerosis rises significantly over time. A proinflammatory "second hit"{{cite journal |last1=Honda |first1=Kazuho |last2=Oda |first2=Hideaki |title=Pathology of encapsulating peritoneal sclerosis |journal=Peritoneal Dialysis International |date=April 2005 |volume=25 |issue=4 |pages=S19–S29 |doi=10.1177/089686080502504S04 |pmid=16300269|s2cid=10389752 }} initiates a series of proangiogenic [vascular endothelial growth factor (VEGF)], proinflammatory [transforming growth factor β1 (TGFβ1), interleukin-6 (IL-6), CCN2] cytokines.{{cite journal | last1=Abrahams | first1=Alferso C. | last2=Habib | first2=Sayed M. | last3=Dendooven | first3=Amélie | last4=Riser | first4=Bruce L. | last5=van der Veer | first5=Jan Willem | last6=Toorop | first6=Raechel J. | last7=Betjes | first7=Michiel G. H. | last8=Verhaar | first8=Marianne C. | last9=Watson | first9=Christopher J. E. | last10=Nguyen | first10=Tri Q. | last11=Boer | first11=Walther H. | title=Patients with Encapsulating Peritoneal Sclerosis Have Increased Peritoneal Expression of Connective Tissue Growth Factor (CCN2), Transforming Growth Factor-β1, and Vascular Endothelial Growth Factor | journal=PLOS ONE | publisher=Public Library of Science (PLoS) | volume=9 | issue=11 | date=November 10, 2014 | issn=1932-6203 | doi=10.1371/journal.pone.0112050 | doi-access=free | page=e112050| pmid=25384022 | pmc=4226475 | bibcode=2014PLoSO...9k2050A }}{{cite journal | last1=Lambie | first1=Mark R. | last2=Chess | first2=James | last3=Summers | first3=Angela M. | last4=Williams | first4=Paul Ford | last5=Topley | first5=Nicholas | last6=Davies | first6=Simon J. | title=Peritoneal inflammation precedes encapsulating peritoneal sclerosis: results from the GLOBAL Fluid Study | journal=Nephrology Dialysis Transplantation | publisher=Oxford University Press (OUP) | volume=31 | issue=3 | date=January 26, 2016 | issn=0931-0509 | doi=10.1093/ndt/gfv440 | pages=480–486| pmid=26908833 }} TGFβ1 stimulates peritoneal mesothelial cells to transdifferentiate into mesenchymal cells, which leads to the depletion of mesothelial cells,{{cite journal | last1=Braun | first1=N. | last2=Alscher | first2=D. M. | last3=Fritz | first3=P. | last4=Edenhofer | first4=I. | last5=Kimmel | first5=M. | last6=Gaspert | first6=A. | last7=Reimold | first7=F. | last8=Bode-Lesniewska | first8=B. | last9=Ziegler | first9=U. | last10=Biegger | first10=D. | last11=Wuthrich | first11=R. P. | last12=Segerer | first12=S. | title=Podoplanin-positive cells are a hallmark of encapsulating peritoneal sclerosis | journal=Nephrology Dialysis Transplantation | publisher=Oxford University Press (OUP) | volume=26 | issue=3 | date=August 13, 2010 | issn=0931-0509 | doi=10.1093/ndt/gfq488 | pages=1033–1041 | doi-access=free| pmid=20709739 }}{{cite journal | last1=Lopez-Anton | first1=Melisa | last2=Lambie | first2=Mark | last3=Lopez-Cabrera | first3=Manuel | last4=Schmitt | first4=Claus P. | last5=Ruiz-Carpio | first5=Vicente | last6=Bartosova | first6=Maria | last7=Schaefer | first7=Betti | last8=Davies | first8=Simon | last9=Stone | first9=Timothy | last10=Jenkins | first10=Robert | last11=Taylor | first11=Philip R. | last12=Topley | first12=Nicholas | last13=Bowen | first13=Timothy | last14=Fraser | first14=Donald | title=miR-21 Promotes Fibrogenesis in Peritoneal Dialysis | journal=The American Journal of Pathology | publisher=Elsevier BV | volume=187 | issue=7 | year=2017 | issn=0002-9440 | doi=10.1016/j.ajpath.2017.03.007 | pages=1537–1550 | doi-access=free| pmid=28495592 }} increased extracellular matrix component production [collagen type 1, alpha 1 (COL1A1)], and fibrogenesis, which forms a fibrocollagenous cocoon.{{cite journal | last1=Reimold | first1=Fabian R. | last2=Braun | first2=Niko | last3=Zsengellér | first3=Zsuzsanna K. | last4=Stillman | first4=Isaac E. | last5=Karumanchi | first5=S. Ananth | last6=Toka | first6=Hakan R. | last7=Latus | first7=Joerg | last8=Fritz | first8=Peter | last9=Biegger | first9=Dagmar | last10=Segerer | first10=Stephan | last11=Alscher | first11=M. Dominik | last12=Bhasin | first12=Manoj K. | last13=Alper | first13=Seth L. | title=Transcriptional Patterns in Peritoneal Tissue of Encapsulating Peritoneal Sclerosis, a Complication of Chronic Peritoneal Dialysis | journal=PLOS ONE | publisher=Public Library of Science (PLoS) | volume=8 | issue=2 | date=February 13, 2013 | issn=1932-6203 | doi=10.1371/journal.pone.0056389 | doi-access=free | page=e56389| pmid=23418565 | bibcode=2013PLoSO...856389R | pmc=3572070 }}

Encapsulating peritoneal sclerosis is diagnosed clinically, supported by radiography or laparotomy, and based on a constellation of clinical findings.

The non-specific laboratory results associated with encapsulating peritoneal sclerosis are linked to underlying infections, malnourishment, and inflammation.{{cite journal | last1=Cho | first1=R. | last2=Ghag | first2=D. | last3=Karim | first3=M. A. | last4=Lo | first4=C. | title=Encapsulating peritoneal sclerosis: surgery, sustained drug therapy and treatment of recurrence at 1 year | journal=BMJ Case Reports | publisher=BMJ | volume=2015 | issue=nov11 1 | date=November 11, 2015 | issn=1757-790X | doi=10.1136/bcr-2015-211490 | pages=bcr2015211490| pmid=26561223 | pmc=4654214 }} It has been demonstrated that dialysate from patients with encapsulating peritoneal sclerosis had higher levels of inflammatory cytokines than did peritoneal dialysis controls, sometimes up to years before the condition's clinical manifestation.{{cite journal | last1=Lopes Barreto | first1=Deirisa | last2=Struijk | first2=Dirk G. | last3=Krediet | first3=Raymond T. | title=Peritoneal Effluent MMP-2 and PAI-1 in Encapsulating Peritoneal Sclerosis | journal=American Journal of Kidney Diseases | publisher=Elsevier BV | volume=65 | issue=5 | year=2015 | issn=0272-6386 | doi=10.1053/j.ajkd.2014.10.022 | pages=748–753| pmid=25530106 }}{{cite journal | last1=Ahmad | first1=Sohail | last2=North | first2=Bernard V. | last3=Qureshi | first3=Ashfaq | last4=Malik | first4=Amir | last5=Bhangal | first5=Gurjeet | last6=Tarzi | first6=Ruth M. | last7=Brown | first7=Edwina A. | last8=Tam | first8=Frederick W. K. | title=CCL18 in peritoneal dialysis patients and encapsulating peritoneal sclerosis | journal=European Journal of Clinical Investigation | publisher=Wiley | volume=40 | issue=12 | date=November 18, 2010 | issn=0014-2972 | doi=10.1111/j.1365-2362.2010.02353.x | pages=1067–1073| pmid=20695883 | s2cid=24656350 }} Nevertheless, no biomarker has been discovered to help anticipate the onset of encapsulating peritoneal sclerosis.{{cite journal | last1=Goodlad | first1=Catriona | last2=Tam | first2=Frederick W.K. | last3=Ahmad | first3=Sohail | last4=Bhangal | first4=Gurjeet | last5=North | first5=Bernard V. | last6=Brown | first6=Edwina A. | title=Dialysate Cytokine Levels do not Predict Encapsulating Peritoneal Sclerosis | journal=Peritoneal Dialysis International | publisher=SAGE Publications | volume=34 | issue=6 | year=2014 | issn=0896-8608 | doi=10.3747/pdi.2012.00305 | pages=594–604| pmid=24584593 | pmc=4164403 }}

When distinguishing encapsulating peritoneal sclerosis from other causes of intestinal obstruction, imaging is frequently useful. Advanced encapsulating peritoneal sclerosis may be suggested by abdominal plain films with peritoneal calcification and dilated bowel loops with air-fluid levels. The small bowel follow-through may be characterized by delayed transit, distension near small bowel adhesions, and a "cauliflower" appearance due to peritoneal sclerosis-encapsulated bowel loop compression. Dilated loops of bowel may appear encased in a dense fibrous membrane or matted together and tethered posteriorly on ultrasonography.{{cite journal | last=Akbulut | first=Sami | title=Accurate definition and management of idiopathic sclerosing encapsulating peritonitis | journal=World Journal of Gastroenterology | publisher=Baishideng Publishing Group Inc. | volume=21 | issue=2 | year=2015 | pages=675–687 | issn=1007-9327 | doi=10.3748/wjg.v21.i2.675 | doi-access=free | pmid=25593498 | pmc=4292304 }} The appearance of a trilaminar colon wall may be due to intraperitoneal echogenic strands.{{cite journal | last1=Ti | first1=Joanna P. | last2=Al-Aradi | first2=Ali | last3=Conlon | first3=Peter J. | last4=Lee | first4=Michael J. | last5=Morrin | first5=Martina M. | title=Imaging Features of Encapsulating Peritoneal Sclerosis in Continuous Ambulatory Peritoneal Dialysis Patients | journal=American Journal of Roentgenology | publisher=American Roentgen Ray Society | volume=195 | issue=1 | year=2010 | issn=0361-803X | doi=10.2214/ajr.09.3175 | pages=W50–W54| pmid=20566781 }}

As of right now, the most extensively researched and widely used imaging method for encapsulating peritoneal sclerosis diagnosis is the CT scan. Small bowel loops are frequently connected by a thickened, encircling peritoneum, which is usually accompanied by proximal bowel dilatation. Increased mesenteric fat density, loculated ascites, and localized or diffuse peritoneal calcification are additional radiographic features.{{cite journal | last1=Stuart | first1=Sam | last2=Stott | first2=David | last3=Goode | first3=Antony | last4=Cash | first4=Charlotte J | last5=Davenport | first5=Andrew | title=Can radiological assessment of abdominal computerized scans diagnose encapsulating peritoneal sclerosis in long-term peritoneal dialysis patients? | journal=Nephrology | publisher=Wiley | volume=22 | issue=1 | date=December 22, 2016 | issn=1320-5358 | doi=10.1111/nep.12718 | pages=19–24| pmid=26730546 | s2cid=21844894 }} Complex loculations may indicate intra-abdominal hemorrhage, but if they contain gas, there is reason to suspect sepsis or a perforation.{{cite journal | last1=Upponi | first1=S. | last2=Butler | first2=A.J. | last3=Watson | first3=C.J.E. | last4=Shaw | first4=A.S. | title=Encapsulating peritoneal sclerosis—Correlation of radiological findings at CT with underlying pathogenesis | journal=Clinical Radiology | publisher=Elsevier BV | volume=69 | issue=1 | year=2014 | issn=0009-9260 | doi=10.1016/j.crad.2013.09.004 | pages=103–109| pmid=24209872 }} Elevated thickening or enhancement of the colon wall suggests transmural fibrosis or persistent inflammation. Although it hasn't been used as much for diagnosis, magnetic resonance imaging probably produces similar results. Benefits include better bowel encasement and peritoneal thickening delineation, as well as the avoidance of ionizing radiation.{{cite journal | last1=Jovani | first1=Manol | last2=Baticci | first2=Fabio | last3=Bonifacio | first3=Cristiana | last4=Omodei | first4=Paolo Dario | last5=Malesci | first5=Alberto | title=Abdominal cocoon or idiopathic encapsulating peritoneal sclerosis: Magnetic resonance imaging | journal=Digestive and Liver Disease | publisher=Elsevier BV | volume=46 | issue=2 | year=2014 | issn=1590-8658 | doi=10.1016/j.dld.2013.08.136 | pages=192–193| pmid=24055233 }}

The histologic results for encapsulating peritoneal sclerosis are not specific and can be confused with those for infectious peritonitis or simple peritoneal sclerosis.{{cite journal | last1=Brown | first1=Edwina A. | last2=Bargman | first2=Joanne | last3=van Biesen | first3=Wim | last4=Chang | first4=Ming-Yang | last5=Finkelstein | first5=Frederic O. | last6=Hurst | first6=Helen | last7=Johnson | first7=David W. | last8=Kawanishi | first8=Hideki | last9=Lambie | first9=Mark | last10=de Moraes | first10=Thyago Proença | last11=Morelle | first11=Johann | last12=Woodrow | first12=Graham | title=Length of Time on Peritoneal Dialysis and Encapsulating Peritoneal Sclerosis — Position Paper for ISPD: 2017 Update | journal=Peritoneal Dialysis International | publisher=SAGE Publications | volume=37 | issue=4 | year=2017 | issn=0896-8608 | doi=10.3747/pdi.2017.00018 | pages=362–374 | doi-access=free| pmid=28676507 | hdl=1854/LU-8529222 | hdl-access=free }} The mesothelial cell layer is microscopically denuded by fibrin deposition, fibroblast proliferation, and fibrocollagenous deposition. When inflammation is active, there may be an infiltration of inflammatory mononuclear cells. Encapsulating peritoneal sclerosis is distinguished from peritoneal sclerosis and peritonitis by the transmembrane glycoprotein podoplanin, which is present on peritoneal mesothelial cells and binds inflammatory cytokines.{{cite journal | last1=Braun | first1=Niko | last2=Fritz | first2=Peter | last3=Ulmer | first3=Christoph | last4=Latus | first4=Joerg | last5=Kimmel | first5=Martin | last6=Biegger | first6=Dagmar | last7=Ott | first7=German | last8=Reimold | first8=Fabian | last9=Thon | first9=Klaus-Peter | last10=Dippon | first10=Juergen | last11=Segerer | first11=Stephan | last12=Alscher | first12=M. Dominik | title=Histological Criteria for Encapsulating Peritoneal Sclerosis – A Standardized Approach | journal=PLOS ONE | publisher=Public Library of Science (PLoS) | volume=7 | issue=11 | date=November 7, 2012 | issn=1932-6203 | doi=10.1371/journal.pone.0048647 | doi-access=free | page=e48647| pmid=23144917 | pmc=3492493 | bibcode=2012PLoSO...748647B }}

Based on the clinical presentation, encapsulating peritoneal sclerosis is classified into four stages:

1. Pre-encapsulating peritoneal sclerosis stage: Mild ascites, no inflammation, and no symptoms.
2. Inflammatory stage: The patient's symptoms, which include intestinal swelling and partial encapsulation of the bowel, nausea and diarrhea. There is an exudation of fibrin and mild inflammation.
3. Encapsulation: Signs of intestinal blockage brought on by the encapsulation-causing fibrous cocoon. It may be linked to inflammation, ranging from mild to severe.
4. Chronic stage of ileus: The thickening of the encapsulating fibrous cocoon causes patients to experience complete bowel obstruction. At this point, there's not much, if any, inflammation.

There is currently no known way to stop encapsulating peritoneal sclerosis from developing, despite numerous studies pointing to potential causes. There has been much discussion regarding the "expiry date" for patients receiving peritoneal dialysis because the risk of developing encapsulating peritoneal sclerosis rises with the length of the treatment. Research from Japan has indicated that patients should switch to hemodialysis after the recommended 8-year safe period for continuing peritoneal dialysis.{{cite journal |last1=Kawanishi |first1=Hideki |last2=Kawaguchi |first2=Yoshindo |last3=Fukui |first3=Hiroyoshi |last4=Hara |first4=Shigeko |last5=Imada |first5=Akio |last6=Kubo |first6=Hitoshi |last7=Kin |first7=Masao |last8=Nakamoto |first8=Masahiko |last9=Ohira |first9=Seiji |last10=Shoji |first10=Takao |title=Encapsulating peritoneal sclerosis in Japan: a prospective, controlled, multicenter study |journal=American Journal of Kidney Diseases|date=2004 |volume=44 |issue=4 |pages=729–737 |doi=10.1016/S0272-6386(04)00953-9 |pmid=15384025}} Setting an expiration date, however, is not advised since it may worsen quality of life and raise the risk of complications for patients receiving hemodialysis via tunneled lines in cases where they had no symptoms while receiving peritoneal dialysis.{{cite journal |last1=Garosi |first1=Guido |last2=Paolo |first2=Nicola Di |last3=Sacchi |first3=Giovanni |last4=Gaggiotti |first4=Enzo |title=Sclerosing peritonitis: a nosological entity |journal=Peritoneal Dialysis International |date=February 2005 |volume=25 |issue=3 |pages=S110–S112 |doi=10.1177/089686080502503S28 |pmid=16048272}}

Treating the underlying cause of encapsulating peritoneal sclerosis is recommended whenever it is feasible. This entails switching from peritoneal dialysis to hemodialysis in the case of peritoneal dialysis.

The nutritional status should be evaluated upon encapsulating peritoneal sclerosis diagnosis. Encapsulating peritoneal sclerosis cannot be effectively treated with bowel rest or total parenteral nutrition (TPN) alone; however, making sure the patient receives enough nutrition is crucial. Enteral feeding is frequently not tolerated due to obstruction, necessitating TPN.{{cite journal |last1=Freitas |first1=Declan de |last2=Jordaan |first2=Antoinette |last3=Williams |first3=Rosalind |last4=Alderdice |first4=Jane |last5=Curwell |first5=Janet |last6=Hurst |first6=Helen |last7=Hutchison |first7=Alastair |last8=Brenchley |first8=Paul E C |last9=Augustine |first9=Titus |last10=Summers |first10=Angela M |title=Nutritional management of patients undergoing surgery following diagnosis with encapsulating peritoneal sclerosis |journal=Peritoneal Dialysis International |date=2008 |volume=28 |issue=3 |pages=271–276 |doi=10.1177/089686080802800314 |pmid=18474920|s2cid=40920566 }}

Many medications have been used to target the inflammatory component of encapsulating peritoneal sclerosis such as mammalian target of rapamycin (mTOR) inhibitors,{{cite journal | last1=Sud | first1=Rahul | last2=Garry | first2=Lorraine | last3=Spicer | first3=Stephen Timothy | last4=Allen | first4=Richard DM | last5=Eris | first5=Josette M | last6=Wyburn | first6=Kate | last7=Verran | first7=Deborah | last8=Cooper | first8=Caroline Louise | last9=Chadban | first9=Steve | title=A role for everolimus in post-transplant encapsulating peritoneal sclerosis: First case report | journal=Nephrology | publisher=Wiley | volume=19 | issue=S1 | date=March 14, 2014 | issn=1320-5358 | doi=10.1111/nep.12196 | pages=27–30| pmid=24460661 | s2cid=30166094 }} cyclosporine,{{cite journal | last1=Romagnoli | first1=J. | last2=Pedroso | first2=J.A. | last3=Paola Salerno | first3=M. | last4=Favi | first4=E. | last5=Spagnoletti | first5=G. | last6=Citterio | first6=F. | title=Posttransplant Encapsulating Peritoneal Sclerosis, Long-Term Success With Everolimus and Low-Dose CNI: A Case Report | journal=Transplantation Proceedings | publisher=Elsevier BV | volume=46 | issue=7 | year=2014 | issn=0041-1345 | doi=10.1016/j.transproceed.2014.07.060 | pages=2368–2370| pmid=25242790 }} mycophenolate mofetil,{{cite journal | last1=Huddam | first1=Bülent | last2=Başaran | first2=Murat | last3=Koçak | first3=Gülay | last4=Azak | first4=Alper | last5=Yalçın | first5=Funda | last6=Reyhan | first6=Nihan Haberal | last7=Duranay | first7=Murat | title=The use of mycophenolate mofetil in experimental encapsulating peritoneal sclerosis | journal=International Urology and Nephrology | publisher=Springer Science and Business Media LLC | volume=47 | issue=8 | date=July 10, 2015 | issn=0301-1623 | doi=10.1007/s11255-015-1015-z | pages=1423–1428| pmid=26159779 | s2cid=205215 }} colchicine,{{cite journal | last1=Bozkurt | first1=Devrim | last2=Bicak | first2=Selahattin | last3=Sipahi | first3=Savas | last4=Taskin | first4=Huseyin | last5=Hur | first5=Ender | last6=Ertilav | first6=Muhittin | last7=Şen | first7=Sait | last8=Duman | first8=Soner | title=The Effects of Colchicine on the Progression and Regression of Encapsulating Peritoneal Sclerosis | journal=Peritoneal Dialysis International | publisher=SAGE Publications | volume=28 | issue=5_suppl | year=2008 | issn=0896-8608 | doi=10.1177/089686080802805s11 | pages=53–57| pmid=19008543 }} corticosteroids, and azathioprine.{{cite journal | last1=Ceri | first1=Mevlut | last2=Unverdi | first2=Selman | last3=Dogan | first3=Mehmet | last4=Unverdi | first4=Hatice | last5=Karaca | first5=Gokhan | last6=Kocak | first6=Gulay | last7=Kurultak | first7=Ilhan | last8=Akbal | first8=Erdem | last9=Can | first9=Murat | last10=Duranay | first10=Murat | title=Effect of sirolimus on the regression of peritoneal sclerosis in an experimental rat model | journal=International Urology and Nephrology | publisher=Springer Science and Business Media LLC | volume=44 | issue=3 | date=April 15, 2012 | issn=0301-1623 | doi=10.1007/s11255-012-0167-3 | pages=977–982| pmid=22528580 | s2cid=26941363 }}

For patients who have already experienced significant fibrosis, immunosuppression might not be enough. A potent anti-fibrotic agent, tamoxifen is a selective estrogen receptor modulator (SERM) that inhibits TGF-β, a crucial cytokine in the fibrosis process.{{cite journal | last1=Loureiro | first1=Jesús | last2=Aguilera | first2=Abelardo | last3=Selgas | first3=Rafael | last4=Sandoval | first4=Pilar | last5=Albar-Vizcaíno | first5=Patricia | last6=Pérez-Lozano | first6=María Luisa | last7=Ruiz-Carpio | first7=Vicente | last8=Majano | first8=Pedro L. | last9=Lamas | first9=Santiago | last10=Rodríguez-Pascual | first10=Fernando | last11=Borras-Cuesta | first11=Francisco | last12=Dotor | first12=Javier | last13=López-Cabrera | first13=Manuel | title=Blocking TGF-β1 Protects the Peritoneal Membrane from Dialysate-Induced Damage | journal=Journal of the American Society of Nephrology | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=22 | issue=9 | year=2011 | issn=1046-6673 | doi=10.1681/asn.2010111197 | pages=1682–1695| pmid=21742730 | pmc=3171939 | hdl=10486/668378 | hdl-access=free }}

Because encapsulating peritoneal sclerosis surgery is a time-consuming, dangerous, and technical procedure, it should only be performed on patients who have not responded to conservative medical therapy and, if at all possible, in facilities with prior experience performing such procedures.{{cite journal | last1=Célicout | first1=Betty | last2=Levard | first2=Hugues | last3=Hay | first3=Jean-Marie | last4=Msika | first4=Simon | last5=Fingerhut | first5=Abe | last6=Pelissier | first6=Edouard | last7=French Associations for Surgical Research | first7=F | title=Sclerosing Encapsulating Peritonitis: Early and Late Results of Surgical Management in 32 Cases | journal=Digestive Surgery | publisher=S. Karger AG | volume=15 | issue=6 | year=1998 | issn=0253-4886 | doi=10.1159/000018681 | pages=697–702| pmid=9845640 | s2cid=46805116 }}{{cite journal | last1=Ulmer | first1=Christoph | last2=Braun | first2=Niko | last3=Rieber | first3=Fabian | last4=Latus | first4=Joerg | last5=Hirschburger | first5=Sandra | last6=Emmel | first6=Jens | last7=Alscher | first7=M. Dominik | last8=Steurer | first8=Wolfgang | last9=Thon | first9=Klaus-Peter | title=Efficacy and morbidity of surgical therapy in late-stage encapsulating peritoneal sclerosis | journal=Surgery | publisher=Elsevier BV | volume=153 | issue=2 | year=2013 | issn=0039-6060 | doi=10.1016/j.surg.2012.07.033 | pages=219–224| pmid=22981361 }}

Patients with encapsulating peritoneal sclerosis have a very high death rate, which ranges from 26% to 58%, and rises with the length of peritoneal dialysis.{{cite journal | last1=Brown | first1=Michaela C. | last2=Simpson | first2=Keith | last3=Kerssens | first3=Jan J. | last4=Mactier | first4=Robert A. | title=Encapsulating Peritoneal Sclerosis in the New Millennium | journal=Clinical Journal of the American Society of Nephrology | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=4 | issue=7 | year=2009 | issn=1555-9041 | doi=10.2215/cjn.01260209 | pages=1222–1229| pmid=19541815 | pmc=2709523 }} Malnutrition and sepsis are the most common causes of death among those with encapsulating peritoneal sclerosis.{{cite journal | last1=Jagirdar | first1=Rajesh M. | last2=Bozikas | first2=Andreas | last3=Zarogiannis | first3=Sotirios G. | last4=Bartosova | first4=Maria | last5=Schmitt | first5=Claus Peter | last6=Liakopoulos | first6=Vassilios | title=Encapsulating Peritoneal Sclerosis: Pathophysiology and Current Treatment Options | journal=International Journal of Molecular Sciences | publisher=MDPI AG | volume=20 | issue=22 | date=November 16, 2019 | issn=1422-0067 | doi=10.3390/ijms20225765 | doi-access=free | page=5765| pmid=31744097 | pmc=6887950 }}

Encapsulating peritoneal sclerosis occurs between 0.5% and 7.3% of the time worldwide, but in patients receiving peritoneal dialysis for 15 years or longer, the frequency can reach 17.2%.{{cite journal | last1=Johnson | first1=David W. | last2=Cho | first2=Yeoungjee | last3=Livingston | first3=Brian E.R. | last4=Hawley | first4=Carmel M. | last5=McDonald | first5=Stephen P. | last6=Brown | first6=Fiona G. | last7=Rosman | first7=Johan B. | last8=Bannister | first8=Kym M. | last9=Wiggins | first9=Kathryn J. | title=Encapsulating peritoneal sclerosis: incidence, predictors, and outcomes | journal=Kidney International | publisher=Elsevier BV | volume=77 | issue=10 | year=2010 | issn=0085-2538 | doi=10.1038/ki.2010.16 | pages=904–912 | doi-access=free| pmid=20375981 }} After nine years of peritoneal dialysis, the encapsulating peritoneal sclerosis risk rose to 8% in one study involving over 17,300 patients from Australia, New Zealand, and Scotland; however, when the competing risk of death was considered, the risk dropped to just 1.5%.{{cite journal | last1=Lambie | first1=Mark | last2=Teece | first2=Lucy | last3=Johnson | first3=David W | last4=Petrie | first4=Michaela | last5=Mactier | first5=Robert | last6=Solis-Trapala | first6=Ivonne | last7=Belcher | first7=John | last8=Bekker | first8=Hilary L | last9=Wilkie | first9=Martin | last10=Tupling | first10=Ken | last11=Phillips-Darby | first11=Louise | last12=Davies | first12=Simon J | title=Estimating risk of encapsulating peritoneal sclerosis accounting for the competing risk of death | journal=Nephrology Dialysis Transplantation | publisher=Oxford University Press (OUP) | volume=34 | issue=9 | date=February 28, 2019 | issn=0931-0509 | doi=10.1093/ndt/gfz034 | pages=1585–1591| pmid=30820552 | pmc=6735880 }}

• Peritonitis
• Peritoneal dialysis

{{reflist}}

• {{cite journal | last1=Singhal | first1=Manphool | last2=Krishna | first2=Satheesh | last3=Lal | first3=Anupam | last4=Narayanasamy | first4=Sabarish | last5=Bal | first5=Amanjit | last6=Yadav | first6=Thakur D. | last7=Kochhar | first7=Rakesh | last8=Sinha | first8=Saroj K. | last9=Khandelwal | first9=Niranjan | last10=Sheikh | first10=Adnan M. | title=Encapsulating Peritoneal Sclerosis: The Abdominal Cocoon | journal=RadioGraphics | publisher=Radiological Society of North America (RSNA) | volume=39 | issue=1 | year=2019 | issn=0271-5333 | doi=10.1148/rg.2019180108 | pages=62–77 | pmid=30526331 | s2cid=54470904 | ref=none}}
• {{cite journal | last1=Korte | first1=Mario R. | last2=Sampimon | first2=Denise E. | last3=Betjes | first3=Michiel G. H. | last4=Krediet | first4=Raymond T. | title=Encapsulating peritoneal sclerosis: the state of affairs | journal=Nature Reviews Nephrology | publisher=Springer Science and Business Media LLC | volume=7 | issue=9 | date=August 2, 2011 | issn=1759-5061 | doi=10.1038/nrneph.2011.93 | pages=528–538 | pmid=21808281 | s2cid=21841526 | ref=none}}

• [https://www.ncbi.nlm.nih.gov/books/NBK574525/ StatPearls]
• [https://www.kidneyfund.org/treatments/dialysis/peritoneal-dialysis Peritoneal dialysis] - American Kidney Fund

{{Medical resources

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{{Kidney disease|state=collapsed}}

Category:Renal dialysis

Category:Kidney diseases

Category:Rare diseases