encapsulating peritoneal sclerosis ( eps ) is a rare but devastating complication of peritoneal dialysis ( pd ) .
herein , we report a case of a patient undergoing 12 years of pd who developed eps and calciphylaxis simultaneously . we also provide a comprehensive discussion about the association between eps and calciphylaxis .
moreover , although tamoxifen is used in eps due to its inhibition of fibroblast - transforming growth factor beta ( tgf ) production , it may worsen the calciphylaxis due to a hypercoagulable state .
encapsulating peritoneal sclerosis ( eps ) is a rare but devastating complication of peritoneal dialysis ( pd ) . in this condition , the peritoneum is covered with fibrotic encapsulations , which leads to bowel obstruction ( 1 ) .
the risk factors for eps include a history of more than 8 years of peritoneal dialysis ( pd ) , repeated peritonitis , and higher concentrations of glucose in the dialysate . unfortunately , there are no reliable markers for this disease , nor any clear pathogenesis or curative treatments ( 2 ) . according to ridvan yavuz ( 3 ) ,
calciphylaxis is a severe vascular complication in patients with end - stage renal disease ( esrd ) .
one - year survival is reported to be 45% - 80% ( 4 ) . to the best of our knowledge
, the association between eps and calciphylaxis has not been discussed , nor has the use of tamoxifen in this context . herein , we report a case of a patient undergoing 12 years of pd , who developed eps and calciphylaxis simultaneously
our patient was a 65-year - old man who received pd for 12 years due to a 20-year history of type 2 diabetes mellitus ( dm ) .
he was under long - term follow - up at taichung veterans general hospital in taichung , taiwan .
he presented with poor appetite and a weight loss of 3 kg in 6 months , starting in january 2015 .
his pd regimen was 1.5%*2l*2 + 2.5%*2l*2 + icodextrin*2l*1 and the amount of daily ultrafiltration was 1000 ml .
blood urea nitrogen was 91 u / l and serum creatinine was 13.1 mg / dl . even while taking three tablets of calcium acetate ( 667 mg ) with each meal , his calcium level was 8.8 mg / dl , phosphate was 7.7 mg / dl , and intact parathyroid hormone was up to 675 pg / ml .
he experienced peritonitis three times during the pd period ( all cultures grew staphylococcus aureus ) . due to no apparent cause for his poor nutrition and poor appetite , we performed abdominal computed tomography ( ct ) to rule out eps . unsurprisingly , ct disclosed diffuse thin calcifications of the serosal surface of the small intestinal loops ( arrow , figure 1c ) .
we reviewed the patient s kidney , ureter , and bladder ( kub ) x - ray from three months before the ct , and noted eps in the wall of small intestine ( arrow , figure 1a ) .
therefore , eps was diagnosed by the radiologists due to cachexia , very low serum albumin , low clearance of the peritoneum , and diffuse calcifications of the intestinal serosal surface .
moreover , there was abdominal dissection with diffuse calciphylaxis ( arrowhead in figure 1a , arrow in figure 1b ) , as well as skin ulcers over the lower extremities . for the diffuse calciphylaxis , we changed to sevelamer , two tablets per meal . for the eps ,
the pd was changed to hemodialysis with low - calcium dialysate and exchange of 2 l of dialysate every two weeks .
however , questions were raised about the use of tamoxifen for eps , as this seems to be contraindicated in a patient in a state of calciphylaxis .
this patient had many risk factors for eps , including high - concentration glucose in the dialysate , a long period of pd ( 8 months ) , and repeated peritonitis .
he also had risk factors for calciphylaxis ( 4 ) , such as a severely dysregulated calcium - phosphorus metabolism , a 32-year history of dm ( 5 ) , type a dissection , a dialysis period of more than 6 - 7 years ( 6 ) , hypoalbuminemia ( 7 ) , and long - term prescription of high - dose calcium - based phosphate binders ( 8) .
( 9 ) , this is the second study mentioning a relationship between eps and calciphylaxis .
fetuin - a and matrix gla protein ( mgp ) can both inhibit calcification , but they are stabilized by serum albumin ( 10 ) . in other words ,
eps - related hypoalbuminemia will cause lower levels of fetuin - a and mgp , causing further vascular calcification .
in addition to identify the similar pathogeneses of eps and calciphylaxis , we also questioned and opposed the use of tamoxifen for eps in patients with calciphylaxis because tamoxifen produces estrogenic - like effects , which could cause venous thromboembolism ( 11 ) .
experience with the use of tamoxifen in eps due to its inhibition of the production of fibroblast - transforming growth factor beta ( tgf ) has grown , and such use is widely considered suitable .
the side effects are generally well - tolerated , such as nausea or hot flushes in women .
however , we believe it is another story when it comes to the use of tamoxifen in eps patients with superimposed calciphylaxis . calciphylaxis is mostly due to an imbalance between calcium and phosphate . according to harris et al .
( 12 ) , a hypercoagulable state indeed plays an important role in the pathogenesis of calciphylaxis ; for example , 38% of calciphylaxis patients had decreased protein c levels and 43% had decreased levels of protein s ( 12 ) .
first , it increases the influx of calcium into platelets , leading to platelet activation , and it acts synergistically with other platelet agonists ( 13 ) .
second , it increases factor viii , factor ix , and von willebrand s factor , while it decreases antithrombin , total protein s , protein c , and plasminogen activator inhibitor-1 ( 14 ) .
its pro - coagulation character superimposed on pre - existing calciphylaxis will worsen tissue ischemia and necrosis .
eps will exacerbate calciphylaxis , and the use of tamoxifen in patients with eps and calciphylaxis should be reconsidered . in the absence of evidence from
randomized controlled trials for the use of tamoxifen in eps , along with its known adverse effects on calciphylaxis , we suggest that tamoxifen should not be used in patients with both eps and calciphylaxis .
eps will exacerbate calciphylaxis , and the use of tamoxifen in patients with eps and calciphylaxis should be reconsidered . in the absence of evidence from
randomized controlled trials for the use of tamoxifen in eps , along with its known adverse effects on calciphylaxis , we suggest that tamoxifen should not be used in patients with both eps and calciphylaxis .
 introductionencapsulating peritoneal sclerosis ( eps ) is a rare but devastating complication of peritoneal dialysis ( pd ) . 
 tamoxifen has been generally well - tolerated , even without randomized controlled trials.case presentationherein , we report a case of a patient undergoing 12 years of pd who developed eps and calciphylaxis simultaneously . 
 we also provide a comprehensive discussion about the association between eps and calciphylaxis . 
 moreover , although tamoxifen is used in eps due to its inhibition of fibroblast - transforming growth factor beta ( tgf ) production , it may worsen the calciphylaxis due to a hypercoagulable state.conclusionswe suggest avoiding the use of tamoxifen for eps in patients with superimposed calciphylaxis . 
Introduction
Case Presentation
Conclusions
1. Introduction
2. Case Presentation
3. Discussion
3.1. Conclusions