Review of the literature and description of a case of sclerosing encapsulating peritonitis requiring home parenteral nutrition

We present a case of a 48 year old HIV patient, who had recurrent episodes of ascites since 2007. His history includes ischaemic heart disease, for which he was treated with atenolol from 2005 to 2007, and Type 2 diabetes; he was later started on propranolol 40  mg twice a day from 2007 for Didanosine-induced portal hypertension. Because of negative cultures and neutrophil count < 250 cells/μL, spontaneous bacterial peritonitis was excluded. However, some low grade-peritoneal irritation cannot be ruled out because his CRP varied from 24 to 258, during 2007 - 2009, without any other obvious inflammatory cause. He was finally diagnosed in July 2009 with sclerosing encapsulating peritonitis (SEP) based on clinical features of intestinal obstruction, histology and imaging, including computed tomography and magnetic resonance imaging. Propranolol was stopped in November 2009.  Because of the patient's severe intestinal obstruction, he was started on parenteral nutrition 2  L/day. Since then, his CRP has returned to normal levels and there is a great improvement of his clinical features. This case demonstrates beta-blockers as a potential cause of SEP, while the presence of some low-grade peritoneal inflammation leading to SEP is also very likely

Sclerosing encapsulating peritonitis: a case successfully treated with immunosuppression.

Perit Dial Int. 1999 Sep-Oct;19(5):478-81. Sclerosing encapsulating peritonitis: a case successfully treated with immunosuppression. Martins LS, Rodrigues AS, Cabrita AN, Guimaraes S. SourceDepartment of Nephrology, Hospital de Santo António, Porto, Portugal

Sclerosing encapsulating peritonitis after peritoneal dialysis

Patients with chronic renal failure in use of peritoneal dialysis (PD) are subject to various complications of the renal replacement therapy. We report a rare complication of PD in which the peritoneum, after years of contact with hypertonic dialysate, is gradually replaced by fibrous tissue. This patient had several complications after initiation of PD including a bacterial peritonitis, tertiary hyperparathyroidism (being treated with parathyroidectomy 2) and cholelithiasis (being treated with laparoscopic cholecystectomy). After 8 years of peritoneal dialysis was transferred to hemodialysis by decreasing ultrafiltration and episodes of intestinal sub-occlusion, being diagnosed as sclerosing encapsulating peritonitis (SEP). He is currently on corticotherapy with a significant reduction of symptoms and likely stabilization of the SEP.Pacientes com insuficiência renal crônica terminal em uso de diálise peritoneal (DP) estão sujeitos a diversas complicações da própria terapia de substituição renal. Relatamos uma complicação rara da DP na qual o peritôneo, após anos de contato com a substância hipertônica dialisante, é gradualmente substituído por tecido fibroso. O paciente em questão teve diversas intercorrências após o início da DP, incluindo uma peritonite bacteriana, hiperparatireoidismo terciário (sendo tratado com duas paratireoidectomias) e colelitíase (sendo tratado com colecistectomia videolaparoscópica). Após 8 anos de diálise peritoneal, foi transferido para hemodiálise por diminuição da ultrafiltração e episódios de suboclusão intestinal, sendo diagnosticado como peritonite esclerosante encapsulante (PEE). Atualmente, está em corticoterapia e com diminuição significativa dos sintomas e provável estabilização da PEE.Fundação Lusíada Faculdade de Ciências Médicas de SantosUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM)Universidade de São Paulo Faculdade de Medicina Hospital das ClínicasUNIFESP, EPMSciEL

Encapsulating peritoneal sclerosis

Neðst á síðunni er hægt að nálgast greinina í heild sinni með því að smella á hlekkinn View/OpenThe incidence of encapsulating peritoneal sclerosis in patients on peritoneal dialysis seems to be increasing worldwide. In Iceland, two cases of encapsulating peritoneal sclerosis have recently been diagnosed (cumulative incidence 1.6%). The patients followed a similar course; the disease was diagnosed in the wake of a bacterial peritonitis, steroid treatment was effective during the acute phase but eventually surgical treatment was needed and a successful enterolysis performed.Umlykjandi lífhimnuhersli (encapsulating peritoneal sclerosis) er alvarlegur fylgikvilli kviðskilunar. Nýgengi þess fer vaxandi á heimsvísu. Talið er að langtímanotkun ólífvænna skilvökva sé helsti orsakavaldurinn en sjúkdómurinn greinist oft eftir bráða lífhimnubólgu. Horfur hafa batnað með notkun lyfja og þróun skurðaðgerðar. Hér á landi hefur umlykjandi lífhimnuhersli greinst hjá tveimur af 124 kviðskilunarsjúklingum. Samanlagt nýgengi er þannig 1,6% sem er svipað og annars staðar. Sjúkrasaga sjúklinganna tveggja var lík; þeir veiktust báðir í kjölfar bráðrar lífhimnubólgu, svöruðu sykursterameðferð vel í bráðafasa sjúkdómsins en þurftu síðar á skurðaðgerð að halda þar sem smágirnið var frílagt (enterolysis). Aðgerð heppnaðist vel í báðum tilvikum

Familial Mediterranean fever in northwest of Iran (Ardabil): The first global report from Iran

Familial Mediterranean fever (FMF), which is the prototype of the hereditary periodic fever syndromes, is common in the countries around the Mediterranean Sea. Considering its geographical position in the northwest of Iran, with its population of Turkish origin and its vicinity to the Mediterranean Sea, the incidence of FMF should be high in Ardabil. The goal of this study was to introduce FMF as a disease with significant outbreak in this area. Based on the Tel-Hashomer criteria, patients suffering from FMF were collected from private clinics together with the medical records of adult and pediatric rheumatology clinics. Of 112 total patients determined, 74 were studied. All of the patients were interviewed and completed a questionnaire. Familial Mediterranean fever was common among children under 18 years (76%), and it was more common in males than females (M/F 1.17). Abdominal pain was the most common complaint (74%) and abdominal pain and fever (95% and 84%, respectively) were the main clinical symptoms. The average duration of pain was 12-72 hours and the average recovery (attack-free period) was from one week to one month (63.5%). The majority of the patients had hospital admission for diagnostic work-up (85%) and some (32%) had undergone surgical operation erroneously; 92% of the patients had taken medications with incorrect diagnosis; and 20% had positive familial history of FMF. Fifty percent of the patients' parents were first-degree relatives and in 59.5% delay in diagnosis was more than three years. It seems that FMF is more common in the Northwest of Iran than previously thought, although physicians are not familiar with it. The common age for manifestation of this disease is under 18 years and its presentation after the age of 40 years is very rare

Incidence and outcome of encapsulating peritoneal sclerosis

Background: Studies report variation in the incidence and outcomes of encapsulating peritoneal sclerosis (EPS). This study reports the incidence and outcome of EPS cases in a national cohort of peritoneal dialysis (PD) patients. Methods: The incident cohort of adult patients who started PD between 1 January 2000 and 31 December 2007 in Scotland (n = 1238) was identified from the Scottish Renal Registry. All renal units in Scotland identified potential EPS cases diagnosed from 1 January 2000 to 31 December 2014, by which point all patients had a minimum of 7 years follow-up from start of PD. Results: By 31 December 2014, 35 EPS cases were diagnosed in the 1238 patient cohort: an overall incidence of 2.8%. The incidence for subgroups with longer PD duration rises exponentially: 1.1% by 1 year, 3.4% by 3 years, 8.8% at 4 years, 9.4% at 5 years and 22.2% by 7 years. Outcomes are poor with mortality of 57.1% by 1 year after diagnosis. Survival analysis demonstrates an initial above-average survival in patients who later develop EPS, which plummets to well below average after EPS diagnosis. Conclusions: The incidence of EPS is reassuringly low provided PD exposure is not prolonged and this supports ongoing use of PD. However, continuing PD beyond 3 years results in an exponential rise in the risk of developing EPS and deciding whether this risk is acceptable should be made on an individual patient basis

Prednisolone inhibits hyperosmolarity-induced expression of MCP-1 via NF-κB in peritoneal mesothelial cells

The mechanism of peritoneal fibrosis in patients on continuous ambulatory peritoneal dialysis (CAPD) is poorly elucidated. We investigated the cellular mechanism of high-glucose-induced expression of monocyte chemoattractant protein-1 (MCP-1), which is important in recruiting monocytes into the peritoneum and progression of peritoneal fibrosis, and examined the inhibitory mechanism of glucocorticoids. Rat peritoneal mesothelial cells were cultured in high-glucose-containing medium and then analyzed for phosphorylation levels of p42/44 and p38 mitogen-activated protein (MAP) kinases (MAPK), MAPK or extracellular signal-regulated kinase kinase (MEK)1/2, c-Jun N-terminal kinase (JNK)1/2, and protein kinase C (PKC) by Western blotting. Expression of MCP-1 was examined by reverse transcription-polymerase chain reaction and enzyme-linked immunosorbent assay, respectively. DNA-binding activity of nuclear factor (NF)-κB was measured by electrophoretic mobility shift assay. High glucose increased MCP-1 mRNA and MCP-1 protein expression. Although glucose increased phosphorylation of MEK1/2, p42/44 MAPK, p38 MAPK, JNK1/2, and PKC, and DNA-binding activity of NF-κB, its effect on MCP-1 expression was suppressed only by PKC and NF-κB inhibitors. Mannitol caused a similar increase in PKC and NF-κB activation and MCP-1 synthesis. Prednisolone increased I-κB-α expression and inhibited glucose/mannitol-induced NF-κB DNA binding and MCP-1 expression without affecting PKC phosphorylation. The inhibitory effects of prednisolone on MCP-1 expression were reversed by mifepristone, a glucocorticoid receptor antagonist. Our results indicate that glucose induces MCP-1 mainly through hyperosmolarity by activating PKC and its downstream NF-κB, and that such effect was inhibited by prednisolone, suggesting the efficacy of prednisolone in preventing peritoneal fibrosis in patients on CAPD

HDAC1 inhibition by MS-275 in mesothelial cells limits cellular invasion and promotes MMT reversal

Peritoneal fibrosis is a pathological alteration of the peritoneal membrane occurring in a variety of conditions including peritoneal dialysis (PD), post-surgery adhesions and peritoneal metastases. The acquisition of invasive and pro-fibrotic abilities by mesothelial cells (MCs) through induction of MMT, a cell-specific form of EMT, plays a main role in this process. Aim of this study was to evaluate possible effects of histone deacetylase (HDAC) inhibitors, key components of the epigenetic machinery, in counteracting MMT observed in MCs isolated from effluent of PD patients. HDAC inhibitors with different class/isoform selectivity have been used for pharmacological inhibition. While the effect of other inhibitors was limited to a partial E-cadherin re-expression, MS-275, a HDAC1-3 inhibitor, promoted: (i) downregulation of mesenchymal markers (MMP2, Col1A1, PAI-1, TGFβ1, TGFβRI) (ii) upregulation of epithelial markers (E-cadherin, Occludin), (iii) reacquisition of an epithelial-like morphology and (iv) marked reduction of cellular invasiveness. Results were confirmed by HDAC1 genetic silencing. Mechanistically, MS-275 causes: (i) increase of nuclear histone H3 acetylation (ii) rescue of the acetylation profile on E-cadherin promoter, (iii) Snail functional impairment. Overall, our study, pinpointing a role for HDAC1, revealed a new player in the regulation of peritoneal fibrosis, providing the rationale for future therapeutic opportunities