NKF 2016 Spring Clinical Meetings Abstracts
Case Report 177
179
IMPROVING ANEMIA THERAPY IN HEMODIALYSIS PATIENTS. EFFICACY AND SAFETY OF ACTH TREATMENT IN GLOMERULAR DISEASES: A SYSTEMATIC REVIEW AND RESULTS OF A MULTICENTRE CLINICAL AUDIT. META-ANALYSIS. Wonngarm Kittanamongkolchai, MD1, Wisit William Kilgallon1, Amelia Fairburn-Beech1, António Sousa2, Carlos 21 2 3 3 Cheungpasitporn, MD1, Ladan Zand, MD11Division of Nephrology and Andrade , Erika Schümann , Thomas Ryzlewicz , Franz-Ferdinand Becker , 4 1 2 Hypertension, Mayo Clinic, Rochester, Minnesota, USA. 1, 1 1 Iain C Macdougall . Oxyless Ltd, London,MBBS, UK, FRP, Portalegre, Laith Al-Rabadi, * RivkaPortugal, Ayalon, MD, RamonThere G. Bonegio, MD, PhD, Background: is a growing evidence that Adrenocorticotropic 3 Via Medis, Riesa, Germany, 4King’s College Hospital,2,y London, UK. 3 4 hormone (ACTH) may be effective in treating Jennifer E. Ballard, MD, Alan M. Fujii, MD, Joel M. Henderson, MD, PhD, various forms of The aim of this investigation was to explore whether the use of1a novel 1 glomerular diseases. However, the efficacy and frequency of adverse David J. Salant, MD, and Laurence H. Beck Jr, MD, PhD bloodline (Oxyless), which reduces the contact between blood and air, could effects associated with the use of ACTH in glomerular diseases are improve the efficiency of Erythropoietin Stimulating Agent (ESA) therapy. unknown. A systematic review and meta-analysis of the literatures was Patients (n=110; >18 years, HD ≥ 3 months via AV fistulae) were entered performed. There littlesingle information pregnancy outcomes in patients withsetting, active participants membranousand nephropathy (MN),A literature into a 16-month open is label, crossover about audit following a 3 month Design, measurements: receptor (PLAEMBASE, major scholar and especially with circulating to M-typesearch phospholipase Run-In. Patients revertedthose to control bloodlines in the autoantibodies Crossover phase after was performedA2using MEDLINE, 2R), the Google Database Systematic Reviews from inceptioninthrough July treatment with Oxyless. Hemoglobin levels, iron sucrose autoantigen in primary(Hb) MN. We IV present whatand weESA believe to Cochrane be the first knownofcase of successful pregnancy 2015. Studies efficacy safetydeveloped of ACTH Treatment in doses wereareported. year prior to assessing pregnancy, the and patient 39-year-old woman with PLA2R-associated MN. In the18,
Pregnancy in a Patient With Primary Membranous Nephropathy and Circulating Anti-PLA R Antibodies: A Case Report
adults with glomerular diseases were included.
anasarca, hypoalbuminemia (albumin, 1.3-2.2 g/dL), and proteinuria (protein excretion, 29.2 g/d). Kidney biResults: Of the 343 identified citations, 18 evaluated the drug was seropositive for anti-PLA opsy revealed MN with staining for PLA2R, and the patientefficacy 2R autoantibodies. and 12 evaluated the adverse effects. The most common She did not respond to conservative therapy and was treated with intravenous doses membranous of 1 g each).nephropathy glomerular diseases rituximab evaluated (2 included (MN),pregnant primary and focalwas segmental glomerulosclerosis Several weeks after presentation, she was found to be 6 weeks closely followed up without(FSGS) and minimal change disease (MCD). Overall of complete further immunosuppressive treatment. Proteinuria remained with protein excretion in the 8- torate 12-g/d range.remission in MN was at 0-6 months, 69% baby at >6-12 90% at > 12-24 At80% 38 weeks, a healthy girlmonths, was born, Circulating anti-PLA2R levels declined but were still detectable. months and 95% beyond 24 months of follow up. Fifty percent of without proteinuria at birth or at her subsequent 6-month postnatal visit. At the time of delivery, the mother still primary FSGS and MCD patients treated with ACTH were in remission IgG4 subclasses, although at had detectable circulating anti-PLA2R of immunoglobulin G1 at 6(IgG1), months,IgG3, but theand relapse rate was high after ACTH discontinuation foundEvidence in cordof blood. Potential for thediseases was low titers. Only trace amounts of IgG4 anti-PLA2R were(17%). ACTH efficacy for reasons other glomerular scarce. was the common reported side effect (incidence fetal Edema circulation aremost discussed. discrepancy between anti-PLA2R levels in the maternal and rate (IR)Foundation, 0.10; 95% Inc. CI, 0.04-0.18; I2 52%; p 0.02) followed by Amdoses J Kidney 67(5):775-778. ª 8,2016 the National Kidney Mean ESA reduced Dis. by 33% (p<0.01) at month equal by to 1,829 2
insomnia (IR 0.08; 95% CI, 0.03-0.15; I 53%; p 0.02), mood swings IU/week/patient. IV iron dose did not change significantly. Patients of a (IR 0.07; 95% CI, 0.02-0.14, I2 57%; p 0.01) and hyperglycemia (IR shorter dialysis vintage (<4 years,Membranous n=35) showed anephropathy greater reduction in ESA INDEX WORDS: (MN); nephrotic syndrome; phospholipase A2 rate due to 0%; p 0.59). The dropout 0.07; 95% CI, pregnancy; 0.04-0.11; I2M-type usage (-42%, p<0.01) (PLA compared with patients of a longer vintage (>4 years, receptor (Ig7%, G)mostly subclass. 2R); autoantibody; placenta; rituximab; immunoglobulin adverse eventGwas due to edema and weight gain. n=31, -23%). By month 16 (Crossover), ESA doses increased to 83% of their Conclusions: ACTH therapy is a promising therapy for glomerular Run-In levels. diseases especially membranous nephropathy, primary FSGS and The audit design increases the confidence in the validity of the data. These MCD. It is overall well-tolerated and has a more favorable side effect results could have clinical and financial benefits for HD service delivery. profile than corticosteroids. regnant patients with autoimmune disease may CASE REPORT
P
1.5
1
.5
All-cause Mortality Hazard ratio
2
deliver newborns with a spectrum of clinical manifestations due to the transplacental passage of 178 circulating autoantibodies. Pregnant patients with RACIAL/ETHNIC DIFFERENCE IN MORTALITY lupus or myasthenia gravis can deliver babies with ASSOCIATED WITH SERUM POTASSIUM IN 1,2Kim 1; MAINTENANCE HEMODIALYSIS PATIENTS Taehee corresponding disease in the neonate. Neonatal Elani Streja1; Connie M. Rhee1; Yoshitsugu Obi1; Csaba P. Kalantar-Zadeh1. 1(MN) UC Irvine,not Orange, CA; Kovesdy2;;Kamyar membranous nephropathy associated with 2 Univ. of Tennessee Health Sciences Center, Memphis, TN; congenital infection was first described in 1990 and Hyperkalemia is a life threatening and likely to occur in CKD patients. Potassium and excretion are different attributed to theintake passive transfer ofamong maternal antirace/ethnicity. We examined racial/ethnic differences in serum 3 bodies to putative renal antigens. than a decade potassium among Whites, Blacks, and HispanicsMore undergoing 4 maintenance (HD), and whether thesesantigen racial/ethnicinvolved later, Debiechemodialysis et al identified the first differences were associated with mortality. in such cases as neutral a In a 5-year (1/2007-12/2011) cohort ofendopeptidase incident HD patients in(NEP), the US, we assessed the present racial/ethnicon differences in serum of potassium metalloprotease the surface the podocyte levels and mortality across Whites, Blacks, and Hispanics using andcoxinvolved proportional in the proteolytic regulation of vasoacup to 5 years. tivehazard peptides. Debiec et al described a mother with a We adjusted for mutation preventing NEP expression who had formed covariates including malnutritionanti-NEP antibodies due to fetomaternal alloimmuinflammation nization from a previous miscarriage; these antibodies markers as well as cardiovascular risk the placenta and cause subepithelial were to cross factors cachexia deposits the syndrome.in Mean (± fetal kidney of a subsequent pregSD) age of overall nancy. M-type phospholipase A2 serum receptor (PLA2R) cohort at baseline was 62.7±15.1 years and mean potassium 4.4±0.5 mEq/l. A totalas of 27,461 (26.9%) all-cause deaths waswaslater identified the major autoantigen for pri5 the black patients with serum were reported. Compared with mary MN in adults. Little literature exists about potassium >4.0 to ≤4.5 mEq/l as referent, white patients had highest mortality rate across all levels in case-mix and pregnancy outcomes inpotassium patients with nephrotic synMICS adjusted model. Hispanic patients with serum potassium drome due to primary MN, with no data available >3.6 mEq/l were associated with lowest mortality rate among races. Mortality rates in Hispanic along with rise in about pregnancy in patients PLAdecreased disease. We 2R-associated serum potassium levels. present what we believe to be the first known case of Even though Hispanics tended to be more hyperkalemic than Whites and Blacks patients whowith were initiating term HD pregnancy in ainpatient PLA2long R-associated MN treatment, the survival advantage for Hispanics even with high who was seropositive for anti-PLA autoantibodies 2R persisted. serum potassium levels over Whites and Blacks clearly throughout the course of her pregnancy. 3.6 =<
.6 >3
to
4.0 =<
>4
.0
4.5 5 .5 5.0 =< =< =< to to to .0 .5 >4 >5 baseline serum potassium (mEq/l)
Whites
Am J Kidney Dis. 2016;67(5):A1-A118
Blacks
.5 >5
Hispanics
A 39-year-old multiparous woman with morbid obesity presented for workup of severe nephrotic syndrome several months 180
before her current pregnancy. She had been treated for resistant ANGIOSARCOMA IN AN UNUSED ARTERIOVENOUS hypertension lower-extremity edemaPATIENT: during theMark past year, FISTULA OFand A RENAL TRANSPLANT but her proteinuria been overlooked. presentation, Kleman, M. Camilahad Bermudez, John Gray, At Geisinger Medicalserum creatinine level was 1.52 mg/dL (corresponding to estimated Center, Danville, PA, USA. glomerular filtration rate mL/min/1.73 m2 isascommonly calculated by Background: Edema in of an 46 arteriovenous fistula thecaused isotope-dilution spectrometry –traceable by thrombosis, mass aneurysm and infection. However,4-variable due to MDRD [Modification of Diet in Renal Disease] Study equathe increased risk of malignancy after transplantation, neoplasm should also be considered. ourg/dL; knowledge, there are few protein tion); serum albumin level,To1.5 and 24-hour urine cases reported in the excretion, 29.2 g. Theliterature. kidney biopsy specimen revealed features Case:ofOur patientMN is a 71 yearadditional old male status post living donor typical primary with strong staining for the transplant in 2011. He was induced with alemtuzumab and PLA 2R antigen within immune deposits (Fig S1). Many of the maintained ondeposits tacrolimus and mycophenolate with excellent subepithelial were completely surrounded by new allograft function. He presented to our institution with new onset basement membrane material (Fig S2), and 35% of the hypercalcemia, fatigue and weight loss. He was noted to have chronic and progressive edema and erythema on his arteriovenous fistula without clear laboratory signs of infection. 1 Fromupthe Department ofwas Medicine, Renal and DeWork for hypercalcemia remarkable for aSection, suppressed 3 partments of 2Obstetrics and Gynecology, Pediatrics,normal and 4PaiPTH, normal PTH-related peptide, normal phosphorus, 1,25- vitamin D and an unremarkable CT chest,University abdomen, pelvis thology and Laboratory Medicine, Boston Medical with IVBoston, contrast. He was treated with calcitonin and zoledronic Center, MA. * acid with improvement in his calcium Given presence of Current affiliation: Department of levels. Internal Medicine, Division a non-PTH related hypercalcemia his immunosuppressed of Nephrology, University of Utahand School of Medicine, Salt Lake status, City, UT.malignancy remained a concern. Duplex ultrasound of arteriovenous fistula showed a complex soft tissueand mass. y Current affiliation: Department of Obstetrics Gynecology, Vascular surgery resected it with pathology showing a high Medstar Washington Hospital Center, Washington, DC. grade angiosarcoma metastatic to axillary lymph nodes. His Received June 29, 2015. Accepted in revised form October 27, immunosuppression regimen was decreased to tacrolimus 2015. Originally onlineonDecember 29, 2015. monotherapy andpublished he was started radiation therapy and Address correspondence to Laurence H. Beck Jr, MD, PhD, paclitaxel adjuvant chemotherapy. Renal Section, X-504, 650 Albany the St, Boston, MAof02118. Discussion: This case highlights importance routineE-mail:
[email protected] examination of unused arteriovenous access in renal transplant � 2016 byInthe Kidney Foundation, Inc. recipients. thisNational population, consideration of malignancy as a cause of remote access pathology is warranted. 0272-6386 http://dx.doi.org/10.1053/j.ajkd.2015.10.031 775 A63