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108: Calcemic Uremic Arteriolopathy -Wide Excision is a Promising Curative Approach: Case Report and Literature Review
A54
105 RENAL MANIFESTATIONS OF POEMS SYNDROME Gaurav Jain, Ivan Maya, Division of Nephrology, University of Alabama at Birmingham, Birmingham, AL. POEMS syndrome is a rare disorder associated with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and Skin changes. Renal involvement in POEMS syndrome is rare with unique biopsy findings and a frequent response to steroid treatment. This is a case report of a biopsy proven pauciimmune membranoproliferative glomerulonephritis in a Hispanic patient with POEMS. 41 y/o Hispanic female presented with dyspnea and generalized swelling. Exam revealed anasarca, extremity skin thickening with hyperpigmentation, hepatosplenomegaly and diffuse peripheral neuropathy. Labs: Hb/Hct 10/30; BUN/creatinine 30/1.5; total protein/albumin 6/2.1; UA: 2+protein, 5-10 red cells, no casts; 24 hour urine protein 338 mg; SPEP with M spike of 0.32g/dl; Urine/Serum IFE: IgG lambda chain. Lymph node biopsy revealed Castleman’s disease (angiofollicular hyperplasia, a non-clonal disease of the lymph nodes). EMG revealed sensory motor polyneuropathy. Diagnosis: POEMS syndrome with Castleman’s disease. Renal biopsy revealed lobulated glomeruli with mesangial hypercellularity and a “double contour” pattern; subendothelial electron dense deposits were present but no immune deposits were observed. Classic renal biopsy has been described as “pauciimmune membrano-proliferative” glomerulonephritis with absence of immune and light chain deposits, but with electron dense subendothelial deposits. The mechanism of renal involvement in POEMS is unclear, but increased levels of IL-6 with subsequent increase of vascular endothelial growth factor (VEGF) have been implicated. Moreover, the same pathophysiology mechanism has been theorized for Castleman’s disease. The increase of VEGF results in angiogenesis, capillary proliferation and endothelial hyperplasia in the glomerular vasculature, and it causes B-cell proliferation resulting in hyperplastic follicles and hence the enlarged lymph nodes. Interestingly, many patients have shown a dramatic improvement in renal function with steroid therapy along with decreasing IL-6 levels suggesting a central role of the proinflammatory state in the pathophysiology. Early recognition of renal involvement in POEMS syndrome is important to understand disease mechanisms and treatment options, including the role of steoids. The use of ACEI may play a role in controlling proteinuria in these patients.
106 DIAGNOSTIC IMAGING CLUES AND MANAGEMENT OF SUDDEN BLINDNESS AFTER RENAL TRANSPLANT. K.Janga, K. Sharma, D.Glicklich, S.Greenberg, VA.Tellis, SM.Greenstein, RS.Schechner .Montefiore Medical Center, Bronx NY, USA. A 48 yr old male admitted for new onset of rapid atrial fibrillation and a left upper lobe infiltrate. His history included a living related renal transplant x 2 (last was 30 days ago), PVD, CAD, HTN and DM.4 days later he developed sudden onset of blurry vision and pain in left eye. As the visual loss progressed, it was further complicated by increasing number of anterior chamber cells, hypopyon formation & posterior vitritis resulting in poor red reflex. This coincided with worsening diffuse infiltrates, cavitations with air crescent sign in left upper lobe for which he received intravitreous vancomycin and gentamycin. Screening for Circulating Galactomannan by ELISA was strongly positive for Aspergillus (90% specific). The brochoscopic lavage, brush washings, blood & eye cultures were negative for all fungal species. CT guided biopsy revealed hemorrhagic necrosis, inflammation and cultures grew Aspergillus fumigatus.The course was complicated by depression, severe malnutrition, anemia, clostridium difficle, uncontrolled BP and high sugars. He was successfully treated and discharged home on PO voriconazole. Aspergillus fumigatus is the most ubiquitous and colonized saprophytic fungi. Mortality in renal transplant is >50% in invasive aspergillosis. Most common cause of endophthalmitis is candida, but if due to aspergillus, it causes mostly blindness and results in enucleation.We report a rare case of endophthalmitis with Aspergillus fumigatus in a living related renal transplant patient. Preemptive antifungal therapy is recommended for COPD /Bronchiectasis in renal transplant subjects with azoles for 3 months. All type of fluids like eye aspirate, blood, urine, BAL lavage, CSF etc. have to be sent for PCR or antigen testing with least amount of suspicion. Weekly measurement of Voriconazole, Calcineurin inhibitors levels with LFTS and SMA-7 are necessary. PO voriconazole achieves therapeutic aqueous & vitreous levels in human eye and its activity spectrum appears to appropriately encompass the most frequently encountered mycotic endopthalmitis. Malnutrition was successfully treated in this rare case with PEG tube place.
NKF 2008 Spring Clinical Meetings Abstracts
107 A REVIEW OF THE EFFECT OF EPOETIN ALFA ON EXERCISE TOLERANCE AND PHYSICAL FUNCTIONING Jansen JP 1, Evans CE1, Guyot P1, Mayne TJ2;1Mapi Values, 2Amgen Inc. A systematic review of the published literature was conducted to evaluate the effect of Epoetin alfa on exercise tolerance and physical functioning in dialysis patients. A search was conducted in Medline and Cochrane databases and studies were included if they were casecontrol, cohort or cross-sectional, published in English, and included an estimate of the impact of Epoetin Alfa on exercise tolerance or physical functioning. Table 1: Percent Improvement from Baseline: Epoetin alfa Treated* Measure Minimum Maximum Range of p-values (# of studies) Improvement Improvement VO2 (10) <0.0005 to 15.1% 50.3% <0.05 Minutes <0.0005 to 17.8% 51.7% Walked (6) <0.05 Distance 7.9% 50.0% <0.001 to NS Walked (2) Physicianassessed 13.2% 24.7% <0.001 Karnofsky (3) Patient-reported 3.7% 18.4% 0.0001 to <0.01 Karnofsky (2) SIP Physical 19.2% 59.4% 0.0001 to NS Function (4) KDQ Physical 6.25% 44.4% <0.001 to <0.05 Symptoms (5) SF-36 Physical 8.86% <0.001 Functioning (1) * Three studies did not conduct statistical testing. Baseline compared to follow-up: Epoetin alfa treated patients; NS = not significant All published studies reviewed that conducted statistical testing reported significant improvements in exercise tolerance and physical functioning. Across studies of diverse design, Epoetin alfa is consistently associated with improvements in exercise tolerance and physical functioning.
108 CALCEMIC UREMIC ARTERIOLOPATHY -WIDE EXCISION IS A PROMISING CURATIVE APPROACH: CASE REPORT AND LITERATURE REVIEW. Deepak Jasuja ,Thomas Powell ,Alice Rocke and Donna Finegan , University of Pittsburgh, Pittsburgh, PA, USA
Calcemic Uremic Arteriolopathy (CUA) is a rare but devastating condition in patients with chronic renal insufficiency with mortality between 60-80 %. It is associated with increased phosphorus levels (>5.5 mg/dl), Calcium x Phosphorus product (>70 mg/dl), secondary hyperparathyroidism and a hypercoaguable state. Pathology involves calcification of media of small and subcutaneous arteries, intimal hyperplasia and intravascular thrombosis leading to necrosis and ulceration associated with severe pain and hyperaesthesia. Prompt diagnosis is vital to prevent sepsis and ischemic complications. Treatment options for this poorly defined condition are limited and include diet, binders, local wound care and parathyroidectomies in severe elevation of PTH (>500 pg/ml despite therapy). Newer approaches have been described including t-PA, hyperbaric oxygen and matrix metalloproteinases inhibitors. We present a 64-year-old white female with history of End Stage Renal disease and Obesity, on Peritoneal Dialysis for two years, who presented with extensive bilateral Ant. Abdominal wall necrotic ulcers. Diagnosis of CUA was made clinically, confirmed later by biopsy. Although the patient had been noncompliant and her phosphorus and PTH levels were elevated at 5.5 mg/dl and 230 pg/ml, respectively, her Calcium x Phosphorus product was never above 70. She was put on strict dietary regimen and binders and then underwent surgical debridement and wide excision of both ulcers (11.5 x 8 x 2 inches each) up to the level of fascia. She then underwent split thickness skin grafts over both wounds. After seven months of intensive wound care and multiple complications, she has achieved complete closure of the wounds. Surgical debridement has been earlier suggested to improve survival and prognosis (Weening et al / Kang et al). In the absence of trials validating the optimal therapeutic approach to this potentially fatal entity, we propose that wide excision accompanied by aggressive multidisciplinary care should be the first line therapy for Calcemic Uremic Arteriolopathy.
105 RENAL MANIFESTATIONS OF POEMS SYNDROME Gaurav Jain, Ivan Maya, Division of Nephrology, University of Alabama at Birmingham, Birmingham, AL. POEMS syndrome is a rare disorder associated with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and Skin changes. Renal involvement in POEMS syndrome is rare with unique biopsy findings and a frequent response to steroid treatment. This is a case report of a biopsy proven pauciimmune membranoproliferative glomerulonephritis in a Hispanic patient with POEMS. 41 y/o Hispanic female presented with dyspnea and generalized swelling. Exam revealed anasarca, extremity skin thickening with hyperpigmentation, hepatosplenomegaly and diffuse peripheral neuropathy. Labs: Hb/Hct 10/30; BUN/creatinine 30/1.5; total protein/albumin 6/2.1; UA: 2+protein, 5-10 red cells, no casts; 24 hour urine protein 338 mg; SPEP with M spike of 0.32g/dl; Urine/Serum IFE: IgG lambda chain. Lymph node biopsy revealed Castleman’s disease (angiofollicular hyperplasia, a non-clonal disease of the lymph nodes). EMG revealed sensory motor polyneuropathy. Diagnosis: POEMS syndrome with Castleman’s disease. Renal biopsy revealed lobulated glomeruli with mesangial hypercellularity and a “double contour” pattern; subendothelial electron dense deposits were present but no immune deposits were observed. Classic renal biopsy has been described as “pauciimmune membrano-proliferative” glomerulonephritis with absence of immune and light chain deposits, but with electron dense subendothelial deposits. The mechanism of renal involvement in POEMS is unclear, but increased levels of IL-6 with subsequent increase of vascular endothelial growth factor (VEGF) have been implicated. Moreover, the same pathophysiology mechanism has been theorized for Castleman’s disease. The increase of VEGF results in angiogenesis, capillary proliferation and endothelial hyperplasia in the glomerular vasculature, and it causes B-cell proliferation resulting in hyperplastic follicles and hence the enlarged lymph nodes. Interestingly, many patients have shown a dramatic improvement in renal function with steroid therapy along with decreasing IL-6 levels suggesting a central role of the proinflammatory state in the pathophysiology. Early recognition of renal involvement in POEMS syndrome is important to understand disease mechanisms and treatment options, including the role of steoids. The use of ACEI may play a role in controlling proteinuria in these patients.
106 DIAGNOSTIC IMAGING CLUES AND MANAGEMENT OF SUDDEN BLINDNESS AFTER RENAL TRANSPLANT. K.Janga, K. Sharma, D.Glicklich, S.Greenberg, VA.Tellis, SM.Greenstein, RS.Schechner .Montefiore Medical Center, Bronx NY, USA. A 48 yr old male admitted for new onset of rapid atrial fibrillation and a left upper lobe infiltrate. His history included a living related renal transplant x 2 (last was 30 days ago), PVD, CAD, HTN and DM.4 days later he developed sudden onset of blurry vision and pain in left eye. As the visual loss progressed, it was further complicated by increasing number of anterior chamber cells, hypopyon formation & posterior vitritis resulting in poor red reflex. This coincided with worsening diffuse infiltrates, cavitations with air crescent sign in left upper lobe for which he received intravitreous vancomycin and gentamycin. Screening for Circulating Galactomannan by ELISA was strongly positive for Aspergillus (90% specific). The brochoscopic lavage, brush washings, blood & eye cultures were negative for all fungal species. CT guided biopsy revealed hemorrhagic necrosis, inflammation and cultures grew Aspergillus fumigatus.The course was complicated by depression, severe malnutrition, anemia, clostridium difficle, uncontrolled BP and high sugars. He was successfully treated and discharged home on PO voriconazole. Aspergillus fumigatus is the most ubiquitous and colonized saprophytic fungi. Mortality in renal transplant is >50% in invasive aspergillosis. Most common cause of endophthalmitis is candida, but if due to aspergillus, it causes mostly blindness and results in enucleation.We report a rare case of endophthalmitis with Aspergillus fumigatus in a living related renal transplant patient. Preemptive antifungal therapy is recommended for COPD /Bronchiectasis in renal transplant subjects with azoles for 3 months. All type of fluids like eye aspirate, blood, urine, BAL lavage, CSF etc. have to be sent for PCR or antigen testing with least amount of suspicion. Weekly measurement of Voriconazole, Calcineurin inhibitors levels with LFTS and SMA-7 are necessary. PO voriconazole achieves therapeutic aqueous & vitreous levels in human eye and its activity spectrum appears to appropriately encompass the most frequently encountered mycotic endopthalmitis. Malnutrition was successfully treated in this rare case with PEG tube place.
NKF 2008 Spring Clinical Meetings Abstracts
107 A REVIEW OF THE EFFECT OF EPOETIN ALFA ON EXERCISE TOLERANCE AND PHYSICAL FUNCTIONING Jansen JP 1, Evans CE1, Guyot P1, Mayne TJ2;1Mapi Values, 2Amgen Inc. A systematic review of the published literature was conducted to evaluate the effect of Epoetin alfa on exercise tolerance and physical functioning in dialysis patients. A search was conducted in Medline and Cochrane databases and studies were included if they were casecontrol, cohort or cross-sectional, published in English, and included an estimate of the impact of Epoetin Alfa on exercise tolerance or physical functioning. Table 1: Percent Improvement from Baseline: Epoetin alfa Treated* Measure Minimum Maximum Range of p-values (# of studies) Improvement Improvement VO2 (10) <0.0005 to 15.1% 50.3% <0.05 Minutes <0.0005 to 17.8% 51.7% Walked (6) <0.05 Distance 7.9% 50.0% <0.001 to NS Walked (2) Physicianassessed 13.2% 24.7% <0.001 Karnofsky (3) Patient-reported 3.7% 18.4% 0.0001 to <0.01 Karnofsky (2) SIP Physical 19.2% 59.4% 0.0001 to NS Function (4) KDQ Physical 6.25% 44.4% <0.001 to <0.05 Symptoms (5) SF-36 Physical 8.86% <0.001 Functioning (1) * Three studies did not conduct statistical testing. Baseline compared to follow-up: Epoetin alfa treated patients; NS = not significant All published studies reviewed that conducted statistical testing reported significant improvements in exercise tolerance and physical functioning. Across studies of diverse design, Epoetin alfa is consistently associated with improvements in exercise tolerance and physical functioning.
108 CALCEMIC UREMIC ARTERIOLOPATHY -WIDE EXCISION IS A PROMISING CURATIVE APPROACH: CASE REPORT AND LITERATURE REVIEW. Deepak Jasuja ,Thomas Powell ,Alice Rocke and Donna Finegan , University of Pittsburgh, Pittsburgh, PA, USA
Calcemic Uremic Arteriolopathy (CUA) is a rare but devastating condition in patients with chronic renal insufficiency with mortality between 60-80 %. It is associated with increased phosphorus levels (>5.5 mg/dl), Calcium x Phosphorus product (>70 mg/dl), secondary hyperparathyroidism and a hypercoaguable state. Pathology involves calcification of media of small and subcutaneous arteries, intimal hyperplasia and intravascular thrombosis leading to necrosis and ulceration associated with severe pain and hyperaesthesia. Prompt diagnosis is vital to prevent sepsis and ischemic complications. Treatment options for this poorly defined condition are limited and include diet, binders, local wound care and parathyroidectomies in severe elevation of PTH (>500 pg/ml despite therapy). Newer approaches have been described including t-PA, hyperbaric oxygen and matrix metalloproteinases inhibitors. We present a 64-year-old white female with history of End Stage Renal disease and Obesity, on Peritoneal Dialysis for two years, who presented with extensive bilateral Ant. Abdominal wall necrotic ulcers. Diagnosis of CUA was made clinically, confirmed later by biopsy. Although the patient had been noncompliant and her phosphorus and PTH levels were elevated at 5.5 mg/dl and 230 pg/ml, respectively, her Calcium x Phosphorus product was never above 70. She was put on strict dietary regimen and binders and then underwent surgical debridement and wide excision of both ulcers (11.5 x 8 x 2 inches each) up to the level of fascia. She then underwent split thickness skin grafts over both wounds. After seven months of intensive wound care and multiple complications, she has achieved complete closure of the wounds. Surgical debridement has been earlier suggested to improve survival and prognosis (Weening et al / Kang et al). In the absence of trials validating the optimal therapeutic approach to this potentially fatal entity, we propose that wide excision accompanied by aggressive multidisciplinary care should be the first line therapy for Calcemic Uremic Arteriolopathy.