encapsulated peritoneal sclerosis ( eps ) is a rare and potentially serious complication of long - term peritoneal dialysis ( pd ) treatment [ 1 , 2 ] . in 1980 , gandhi et al .   reported the earliest description of sclerotic thickening of the peritoneal membrane among patients on long - term ( 3 months to 4 years ) maintenance pd . the authors implicated history of multiple episodes of peritonitis , low ph , and hypertonic dextrose as important contributing factors for the development of eps . in another study , denis et al . the incidence of eps among patients on pd is relatively low and has been estimated at 1.54.6 cases per 1,000 patients yearly , with significant geographical disparities . despite its rarity , the timely diagnosis of eps is important given the associated poor outcome . preoperative diagnosis depends on a high index of suspicion with attention to subtle , yet characteristic clinical features . we report the first case , to date , in al - ain , united arab emirates . months later , emphasizing the need for a better awareness of such a rare entity . a 40-year - old male emirati patient with a known history of hypertension , psoriasis and end - stage renal disease , presented to the emergency department because of progressive , generalized , dull abdominal pain with distention , and severe constipation . however , the pain was not radiating to the back and there was no associated vomiting , fever , or diarrhea . he had been on automated pd prescription ( 6 cycles , 2-liter exchanges of 1.36 alternating with 3.38% dextrose ) for the past 6 months . previously , he had been maintained on chronic ambulatory peritoneal dialysis 2-liter exchanges of 1.5% dextrose for a number of years . recently , he had evidence of ultra - filtration membrane failure with declining drain volumes , hence , the change to automated pd , after which he showed good improvement . prior to hospitalization , his spouse noticed turbidity in the pd fluid , but no other problems were reported . past clinical history was significant for end - stage renal disease of unknown etiology for over 20 years and hypertension . his current medications include : nifedipine 60 mg od , atenolol 25 mg od , caco3 1.2 g tid , and omeprazole 20 mg od . physical examination revealed a mildly distressed , unwell looking gentleman who was afebrile ( 36.8c ) , blood pressure 144/86 mm hg , pulse 72/min , and respiratory rate 18 breaths / min . cardiovascular examination was unremarkable with normal heart sounds , regular rhythm , and no murmur . lungs were clear and abdominal examination showed a distended abdomen with left lower quadrant tenderness and rebound , but normal bowel sounds . there was evidence of a previous surgical scar and the pd exit site had no redness or discharge . laboratory investigations revealed a white blood cell count of 11,000  10/l with 85% neutrophils and 8.2% lymphocytes . l , serum sodium : 135 mmol / l , potassium : 4.1 mmol / l , chloride : 94 mmol / l , bicarbonate : 26 mmol / l , and glucose : 6.4 mmol / l ; all levels were within normal limits . abdominal ct scan showed evidence of peritonitis with paralytic ileus and a thickened wall of the sigmoid colon ( fig . the patient was started on cefazolin ( 1 g / ip daily ) and gentamicin ( 40 mg / ip daily ) as per ispd guidelines 2000 . he continued to exhibit temperature spikes during his first 2 days in hospital with continued abdominal pain and turbid pd fluid . on day 5 , the surgical team , who had been monitoring the patient since admission , elected to perform an exploratory laparotomy and remove the pd catheter . was then switched to hemodialysis via left av fistula . however , despite removing the pd catheter , he remained febrile with continued abdominal pain and distention . after consultation with infectious disease physicians , he was switched to meropenem 1 g / day following hemodialysis . nonetheless , his fever and abdominal distention persisted unabated for over 3 months . he further underwent multiple treatment courses of antibiotics and antifungal agents including piperacillin - tazobactam , meropenem , tigecycline , voriconazole , caspofungin , metronidazole , vancomycin , and levofloxacin with no significant improvement . throughout the course of hospitalization overall , his general condition deteriorated during his hospital stay , he lost approximately 10% of his body weight , and his serum albumin decreased to 32 mmol / l . of note is that the culture of various specimens including blood , pd fluid , stool , and clostridium difficile antigen and toxin remained negative throughout his hospitalization . entertaining a wide spectrum of differential diagnoses to include tuberculous peritonitis , autoimmune disease , and familial mediterranean fever , the following investigations were performed : quantiferon - tb and other serological investigations , namely ana , anca , c3 , c4 , cmv , hepatitis b , c and hiv , were also negative . after removal of the pd catheter , the wound was healing well and wound cultures were negative with the exception of one which grew non - albicans candida , hence , the antifungal treatment . subsequently , the patient was prescribed a trial anti - tuberculosis treatment regimen consisting of isoniazid ( 300 mg / day ) , rifampicin ( 600 mg / day ) , moxifloxacin ( 400 mg / day ) , ethambutol ( 1,200 mg post hd ) and pyridoxine ( 50 mg / day ) . he was discharged home ( 4.5 months later ) to continue on thrice weekly hd . however , due to intolerance , he stopped anti - tuberculosis medications 2 weeks after discharge . despite not taking his medication regularly , his condition slowly improved , and he became afebrile after 4 weeks with complete resolution of all his symptoms . since then , the patient has had two similar presentations with abdominal pain secondary to subacute small bowel obstructions that responded well to supportive treatment with hydration , nasogastric drainage , and pain control . laboratory investigations revealed a white blood cell count of 11,000  10/l with 85% neutrophils and 8.2% lymphocytes . serum c - reactive protein was high at 344 mg / l , serum sodium : 135 mmol / l , potassium : 4.1 mmol / l , chloride : 94 mmol / l , bicarbonate : 26 mmol / l , and glucose : 6.4 mmol / l ; all levels were within normal limits . abdominal ct scan showed evidence of peritonitis with paralytic ileus and a thickened wall of the sigmoid colon ( fig . the patient was started on cefazolin ( 1 g / ip daily ) and gentamicin ( 40 mg / ip daily ) as per ispd guidelines 2000 . he continued to exhibit temperature spikes during his first 2 days in hospital with continued abdominal pain and turbid pd fluid . on day 5 , the surgical team , who had been monitoring the patient since admission , elected to perform an exploratory laparotomy and remove the pd catheter . the patient was then switched to hemodialysis via left av fistula . however , despite removing the pd catheter , he remained febrile with continued abdominal pain and distention . serum c - reactive protein level remained > 200 mg / l . after consultation with infectious disease physicians , he was switched to meropenem 1 g / day following hemodialysis . nonetheless , his fever and abdominal distention persisted unabated for over 3 months . he further underwent multiple treatment courses of antibiotics and antifungal agents including piperacillin - tazobactam , meropenem , tigecycline , voriconazole , caspofungin , metronidazole , vancomycin , and levofloxacin with no significant improvement . throughout the course of hospitalization overall , his general condition deteriorated during his hospital stay , he lost approximately 10% of his body weight , and his serum albumin decreased to 32 mmol / l . of note is that the culture of various specimens including blood , pd fluid , stool , and clostridium difficile antigen and toxin remained negative throughout his hospitalization . entertaining a wide spectrum of differential diagnoses to include tuberculous peritonitis , autoimmune disease , and familial mediterranean fever , the following investigations were performed : quantiferon - tb and other serological investigations , namely ana , anca , c3 , c4 , cmv , hepatitis b , c and hiv , were also negative . after removal of the pd catheter , the wound was healing well and wound cultures were negative with the exception of one which grew non - albicans candida , hence , the antifungal treatment . subsequently , the patient was prescribed a trial anti - tuberculosis treatment regimen consisting of isoniazid ( 300 mg / day ) , rifampicin ( 600 mg / day ) , moxifloxacin ( 400 mg / day ) , ethambutol ( 1,200 mg post hd ) and pyridoxine ( 50 mg / day ) . he was discharged home ( 4.5 months later ) to continue on thrice weekly hd . however , due to intolerance , he stopped anti - tuberculosis medications 2 weeks after discharge . despite not taking his medication regularly , his condition slowly improved , and he became afebrile after 4 weeks with complete resolution of all his symptoms . since then , the patient has had two similar presentations with abdominal pain secondary to subacute small bowel obstructions that responded well to supportive treatment with hydration , nasogastric drainage , and pain control . eps , though uncommon , is a potentially lethal complication of pd with unclear pathogenesis . known risk factors for eps include long duration of pd , exposure to high glucose concentrations in dialysis solution , and severe bacterial peritonitis . also , a decline in ultrafiltration in high transporters is an early indicator of eps development . our patient demonstrated high - risk characteristics for eps , with long exposure to maintenance pd for almost 8 years , and a history of using high glucose concentration in dialysate in the recent past but , interestingly , with no prior history of peritonitis . in addition , the patient had evidence of ultrafiltration failure prior to his presentation with the need to change to automated pd and the use of hypertonic pd fluid . more importantly , our patient had atypical clinical features of eps including , but not limited to , abdominal pain , nausea , vomiting , and intestinal obstruction . taken together , our patient appears to have had several known risk factors , all of which should have led to a timely diagnosis and better treatment outcome . eps is characterized by a progressive intra - abdominal inflammation resulting in sheets of fibrous tissue that encapsulate , bind , and constrict the viscera , thus restricting bowel movement and function . the hallmark pathological features of eps include peritoneal thickening and calcification , loculated ascites , narrowing bowel lumen with adherent loops , and encapsulation by fibrous cocoon . laboratory findings include elevated c - reactive protein and tgf1 as well as decreased hemoglobin , serum albumin , and ca-125 levels . ct scan is also the most helpful tool , though less definitive than laparoscopy and biopsy . overall , three out of the following criteria are diagnostic of eps , namely peritoneal enhancement , thickening , calcifications , bowel tethering , dilatation , and fluid loculation / septation . when reviewed in retrospect , our patient possessed four of the above characteristic features on ct scan as shown in fig . nonetheless , there was a significant delay in diagnosing eps in this patient ; in fact , the case was diagnosed once the acute phase had completely resolved . the patient was thus exposed to prolonged and multiple courses of antibiotics rather than appropriately targeted treatment . current therapy for eps focuses on the use of immune suppressive and antifibrotic agents such as tamoxifen , prednisone , azathioprine , or mycophenolate mofetil [ 10 , 11 , 12 , 13 ] . however , the data on the latter two is weak , whereas tamoxifen and steroids are advocated as useful in prevention as well as treatment of eps . experimental data suggest that rosiglitazone may also be useful in halting the progression and even causing regression of eps . raas blockade appears to reduce cell proliferation and fibrosis , which can prevent eps progression and might play a role in its prevention through improvement of high transporter characteristics and uf failure . other agents with a potential protective effect include thalidomide , everolimus and a number of other experimental agents . however , there are no randomized trials to clearly establish their utility in the prevention of eps . to date , no definitive surgical treatment for eps has been characterized due to limited clinical experience . however , when the latter is needed , complete enterolysis of intestinal adhesions without enterectomy has shown a successful improvement of up to 94.2% . recent data suggest that perionectomy and enterolysis ( peel ) , though a complex procedure , is a promising treatment option that may be performed with low mortality and acceptable morbidity . it should be noted that whilst surgery can reverse intestinal obstruction , it does not improve peritoneal deterioration ; thus , such procedures , though relatively successful , may lead to heightened recurrence rates of eps . undoubtedly , specific risk factors must be recognized early enough , especially among asymptomatic patients to avoid complications . such measures include avoidance of long durations of pd ( > 7 years ) and continuous assessment of pet to detect occurrence of high transporter characteristics . furthermore , the use of biocompatible dialysis solution has been advocated to reduce the occurrence of eps . expiry date , implying pd discontinuation according to a pre - specified time , even before eps is suspected , has been suggested . such an approach , however , was recently challenged by cornelis and oreopoulos , who proposed the development of an eps scoring system based on clinical , laboratory , and radiological features along with preventative treatment , namely tamoxifen and/or prednisone at a specified threshold . eps is a rare complication of pd that may lead to devastating clinical consequences . though it can occur in patients on pd , it may present long after pd has been discontinued , such as in the posttransplant period . hence , eps diagnosis depends on a high index of suspicion coupled with several laboratory and radiological tests . most importantly , early identification and recognition of its subtle features is key for prevention .
encapsulating peritoneal sclerosis ( eps ) is a rare complication of peritoneal dialysis ( pd ) with devastating consequences . 
 although it can occur among patients on pd , it may present long after pd has been discontinued ; thus , its diagnosis depends on a high index of suspicion . 
 we present a rare case of eps in the united arab emirates with a protracted course and delays in recognizing the condition until after resolution of the acute attack . 
 this case highlights the need for early identification of risk factors as well as the subtle features of eps for appropriate diagnosis and perhaps even prevention ; this being worth a ton of cure . 
 a review of the literature including the latest evidence - based treatment options is also highlighted .