- Email: [email protected]
CKD Report: A New Communication Tool
NKF 2016 Spring Clinical Meetings Abstracts
Case Report 1
3 PATIENT CENTERED CKD PRACTICE: PROPOSED MODEL. Pregnancy in a Patient With Primary Membranous Nephropathy Konstantin Abramov, Jeffrey Stoff. UMass Memorial Health Care, Worcester, MA, USA.A Case Report and Circulating Anti-PLA2R Antibodies: We designed a clinical program that applies principles of a patient
LOW-INTENSITY RITUXAMAB THERAPY FOR NEPHROTIC SYNDROME AND ADVANCED CKD IN SYSTEMIC LUPUS ERYTHEMATOSUS: ONE DOSE MAY NOT FIT ALL. Sohail Abdul Salim, Swetha Kanduri, Yougandhar V. Akula, Mohit Agarwal, Jack R. Lewin, Tibor Fülöp.
centered specialty practice (PCSP)1 to the management of CKD.
1 1 Laith Al-Rabadi, MBBS,1,* Rivka Ayalon, MD, Ramon G.a KDIGO-based Bonegio, MD, The model includes clinicalPhD, decision pathway to Aggressive treatment of Systemic Lupus Erythematosus 2,y(SLE) in face of 3 4 facilitate appropriate screening and referral, CKD stage-specific Jennifer E. Ballard, MD, Alan M. Fujii, MD, Joel M. Henderson, MD,and PhD, advanced Chronic Kidney Disease (CKD) and nephrotic syndrome reports for better communication between 1 (NS) 1 patients, PCPs and David J. Salant, MD, and Laurence H. Beck Jr, MD, PhD may represent therapeutic dilemma. The danger of nephrologists. An email hotline, which is staffed by renal fellows and
supervised by an attending nephrologist, augments access to renal immunosuppressive therapy ought to be counterbalanced by the risk services and supplements the CKD clinical pathway by providing of ongoing nephrotic-range proteinuria. A 45 y/o petite AA female (52 same-day advice to PCPs (see figure for the full list of (MN), components). information about outcomes in patients with active membranous nephropathy kg) with pastThere history is of little SLE and CKD presented forpregnancy further follow-up those with Prior circulating autoantibodies to M-type phospholipase A2 receptor (PLA2R), the major after long especially period of non-adherence. treatments included autoantigen in primary MN.and Weprednisone present what wehas believe to be the first known case of successful pregnancy in hydroxychloroquine (HCQ), methotrexate but she R-associated a therapy 39-year-old woman with PLA2to not been on for 10 yrs. She was admitted the inpatient MN. In the year prior to pregnancy, the patient developed anasarca, hypoalbuminemia (albumin, 1.3-2.2 g/dL), and proteinuria (protein excretion, 29.2 g/d). Kidney biservice due to suspected SLE flare-up with renal failure (serum R, g/dL). and the patient was seropositive for anti-PLA2R autoantibodies. revealed MNmg/dL) with staining for PLA creatinineopsy (Cr) between 4.4-7.4 and NS (albumin of23.4 Random urine predicted proteinuria 4 gm.was treated with intravenous rituximab (2 doses of 1 g each). Sheprotein/creatinine did not respond(UPC) to conservative therapy> and Renal biopsy revealed membranous (Class V) SLE with Several weeks after presentation, sheadvanced was found to be 6 weeks pregnant and was closely followed up without fibrosis (“80%") only ~50% glomeruli reported as completely scarred. remained with protein excretion in the 8- to 12-g/d range. further immunosuppressive treatment. Proteinuria This represented a difficult management dilemma. We proceeded with Circulating anti-PLA 2R levels declined but were still detectable. At 38 weeks, a healthy baby girl was born, 2 dose of IV rituximab (initial dose mg;orrepeated dose 750 mg 66-month postnatal visit. At the time of delivery, the mother still without proteinuria at500 birth at her subsequent weeks later). A month later, proteinuria persisted but count was of immunoglobulin G1 (IgG1), IgG3, and IgG4 subclasses, although at had detectable circulating anti-PLA2RCD19 suppressed to 0% (normal: 2-11) and absolute CD4ofcount wasanti-PLA R were found in cord blood. Potential reasons for the low titers. Only trace amounts IgG4 2 3 moderately suppressed at 199 mm (506-3142); hence, further discrepancy between anti-PLA2R levels in the maternal and fetal circulation are discussed. rituximab was deferred. On follow-up 1.5 years later, Cr decreased to Am J Kidney Dis. 67(5):775-778. ª 2016 by2 the National Kidney Foundation, Inc. ~4 mg/dL (estimated race-adjusted GFR 16-18 mL/min/1.73 m ), and random UPC near-normalized to 0.2 with normal serum albumin (4-4.6 INDEX WORDS: Membranous nephropathy (MN); nephrotic syndrome; pregnancy; M-type phospholipase A2 g/dL). She remained free of uremia or any clinical activity of SLE. In We intent to study the effect of PCSP on achieving the Triple Aim of receptor (PLA2R); autoantibody; placenta; rituximab; immunoglobulin G Healthcare (Ig G) subclass. the Institute for Improvement: better care for individuals summary, low-intensity rituximab therapy achieved complete and populations at lower per-capita cost2. remission of nephrotic syndrome. 1. https://www.acponline.org/advocacy/current_policy_papers/as sets/pcmh_neighbors.pdf 2. http://content.healthaffairs.org/content/27/3/759.full.html regnant patients with autoimmune disease may CASE REPORT
P
deliver newborns with a spectrum of clinical manifestations due to the transplacental passage of 2 circulating autoantibodies. Pregnant patients with CKD REPORT: A NEW COMMUNICATION TOOL lupus or myasthenia gravis can Memorial deliverHealth babies Konstantin Abramov, Jeffrey Stoff. UMass Care,with Worcester, MA, USA. corresponding disease in the neonate.1,2 Neonatal We implemented point-of-care CKD reports as a communication tool membranous nephropathy (MN) not associated with with the potential to improve CKD management and patient congenital infection was first described 1990 and engagement in a patient centered specialty practice or ain patient centered medical home. attributed to the passive transfer of maternal anti3 Patient identifiers, name of bodies to putative renal antigens. More than a decade nephrologist and date of last 4 later, Debiec et al identified theappointment, first antigen involved name of PCP. Latest GFR and CKD stage. a in such cases as neutral endopeptidase (NEP), Last 5 Cr and GFR values metalloprotease present on the surface of the podocyte Last 5 clinic blood pressures and involved in the proteolyticLast regulation of vasoacurine albumin/Cr, protein/Cr, bicarbonate, tive peptides. Debiec et al described a serum mother with a calcium, phosphorus (P), PTH, mutation preventing NEP expression who had formed vitamin D, potassium (K), anti-NEP antibodies due to fetomaternal alloimmuserum albumin, Hgb, HgbA1C. ACE/ARB, statin antibodies therapy. nization from a previous miscarriage; these Dietary instructions if either K were to cross the placenta and cause subepithelial or P are abnormal; reminders deposits in the fetal kidney of a subsequent pregabout stage specific referrals access,(PLA CKD 2R) nancy. M-type phospholipase (e.g. A2 vascular receptor education groups, transplant). wasData later identified as the major autoantigen for priis transferred weekly into 5 a CKD, which is in Excel format. MRN mary MN in adults. Little literature at the startabout of the must be entered to display reports on screen or to print exists clinic. Nephrologists reviewinreports during clinic often add handpregnancy outcomes patients withandnephrotic synwritten instruction, which patients keep at the end of the visit. drome due to primary MN, with no data available CKD reports appear to improve physician work flow and patient about pregnancy in PLA R-associated engagement. We are studying the 2effect of CKD reportsdisease. on clinical We outcomes, as well patient’s satisfaction andfirst understanding CKD. of present what weasbelieve to be the knownofcase Since the majority of CKD patients are managed by PCPs this tool may pregnancy in a patient with PLA R-associated MN have additional applications in primary care. 2 who was seropositive for anti-PLA2R autoantibodies throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118
A 39-year-old multiparous woman with morbid obesity presented for workup of severe nephrotic syndrome several months 4
before her current pregnancy. She had been treated for resistant PRES AS AN INITIAL PRESENTATION OF ESRD: Matthew hypertension andBrook lower-extremity edema during Abramson, Stony University Hospital, Stony Brookthe NY,past USA year, but PRES her proteinuria had been overlooked. Atwith presentation, serum Syndrome has been described in patients ESRD creatinine was mg/dL (corresponding to estimated undergoinglevel both PD and1.52 HD. Here, we describe a case of a patient with glomerular of 46 with mL/min/1.73 m2found as calculated no medical filtration history whorate presented blurred vision, to have by HTN, CKD Vmass and PRES Syndrome. –traceable 4-variable themalignant isotope-dilution spectrometry A 29 year-old El Salvadorian maleinpresented to the ED with MDRD [Modification of Diet Renal initially Disease] Study equa2 weeks of worsening and headaches. Patienturine was protein tion); serum albuminblurred level,vision 1.5 g/dL; and 24-hour controlling headaches with frequent NSAIDs. In the ED, BP was excretion, 29.2 g. The kidney biopsy specimen revealed features 248/143, and labetalol drip was started. Initial BUN/Cr was 83/10.8, typical of primary MN with additional strong staining for the and did not improve with IVF or diuresis. No other significant PLA within were immune deposits S1). Many of the 2R antigen electrolyte abnormalities present. 24-hour (Fig urine protein was 2.4g, subepithelial deposits were completely surrounded by with an output of 2L. EKG and ECHO demonstrated LVH. CT Head new basement membrane material S2), 35% of the showed mild nonspecific white matter(Fig disease. MRI and brain revealed abnormal signal within bilateral occipital lobes and cerebellar vermis, suggestive of PRES. Renal sonogram showed small kidneys with 1 increased cortical echogenicity. improved to the Section, 150s, and and patientDeFrom the Department of SBPs Medicine, Renal was transitioned to oral medications. Due to concern for persistent 4 partments of 2Obstetrics and Gynecology, 3Pediatrics, and Pavisual disturbance, ophthalmology was consulted, and hypertensive thology and Laboratory Medicine, Boston University Medical retinopathy was noted on exam; at this time, glucocorticoids were Center, Boston, decided not to beMA. administered. AVF was placed and patient was * Current affiliation: of Internal Medicine, Division discharged. He presentedDepartment to clinic for routine follow-up 2 weeks later of with Nephrology, University of Utah School of Medicine, resolution of vision changes. BUN/Cr slightly worsened toSalt Lake 102/9.5. City, UT. Secondary etiologies of HTN and rheumatologic workup were y negative. Current affiliation: Department of Obstetrics and Gynecology, The patient was then Hospital lost to follow-up. HeWashington, returned 7 months Medstar Washington Center, DC.later to clinic with 2June weeks29, of hand morning nausea and vomiting. BP 27, Received 2015.tremors, Accepted in revised form October was 210/120. He was sent to the ED; BUN/Cr was 158/21. Due to 2015. Originally published online December 29, 2015. uremic symptoms, he was started on HD, and eventually discharged Address correspondence to Laurence H. Beck Jr, MD, PhD, with outpatient dialysis follow-up established. Renal Section, X-504, 650 Albany St, Boston, MA 02118. PRES Syndrome is becoming more frequently described as case E-mail: [email protected] reports, usually due to HTN, and it is important to recognize PRES � 2016 by the that National Foundation, Inc.will often early, considering reversalKidney of the underlying etiology reverse the syndrome. 0272-6386
http://dx.doi.org/10.1053/j.ajkd.2015.10.031 775 A19
Case Report 1
3 PATIENT CENTERED CKD PRACTICE: PROPOSED MODEL. Pregnancy in a Patient With Primary Membranous Nephropathy Konstantin Abramov, Jeffrey Stoff. UMass Memorial Health Care, Worcester, MA, USA.A Case Report and Circulating Anti-PLA2R Antibodies: We designed a clinical program that applies principles of a patient
LOW-INTENSITY RITUXAMAB THERAPY FOR NEPHROTIC SYNDROME AND ADVANCED CKD IN SYSTEMIC LUPUS ERYTHEMATOSUS: ONE DOSE MAY NOT FIT ALL. Sohail Abdul Salim, Swetha Kanduri, Yougandhar V. Akula, Mohit Agarwal, Jack R. Lewin, Tibor Fülöp.
centered specialty practice (PCSP)1 to the management of CKD.
1 1 Laith Al-Rabadi, MBBS,1,* Rivka Ayalon, MD, Ramon G.a KDIGO-based Bonegio, MD, The model includes clinicalPhD, decision pathway to Aggressive treatment of Systemic Lupus Erythematosus 2,y(SLE) in face of 3 4 facilitate appropriate screening and referral, CKD stage-specific Jennifer E. Ballard, MD, Alan M. Fujii, MD, Joel M. Henderson, MD,and PhD, advanced Chronic Kidney Disease (CKD) and nephrotic syndrome reports for better communication between 1 (NS) 1 patients, PCPs and David J. Salant, MD, and Laurence H. Beck Jr, MD, PhD may represent therapeutic dilemma. The danger of nephrologists. An email hotline, which is staffed by renal fellows and
supervised by an attending nephrologist, augments access to renal immunosuppressive therapy ought to be counterbalanced by the risk services and supplements the CKD clinical pathway by providing of ongoing nephrotic-range proteinuria. A 45 y/o petite AA female (52 same-day advice to PCPs (see figure for the full list of (MN), components). information about outcomes in patients with active membranous nephropathy kg) with pastThere history is of little SLE and CKD presented forpregnancy further follow-up those with Prior circulating autoantibodies to M-type phospholipase A2 receptor (PLA2R), the major after long especially period of non-adherence. treatments included autoantigen in primary MN.and Weprednisone present what wehas believe to be the first known case of successful pregnancy in hydroxychloroquine (HCQ), methotrexate but she R-associated a therapy 39-year-old woman with PLA2to not been on for 10 yrs. She was admitted the inpatient MN. In the year prior to pregnancy, the patient developed anasarca, hypoalbuminemia (albumin, 1.3-2.2 g/dL), and proteinuria (protein excretion, 29.2 g/d). Kidney biservice due to suspected SLE flare-up with renal failure (serum R, g/dL). and the patient was seropositive for anti-PLA2R autoantibodies. revealed MNmg/dL) with staining for PLA creatinineopsy (Cr) between 4.4-7.4 and NS (albumin of23.4 Random urine predicted proteinuria 4 gm.was treated with intravenous rituximab (2 doses of 1 g each). Sheprotein/creatinine did not respond(UPC) to conservative therapy> and Renal biopsy revealed membranous (Class V) SLE with Several weeks after presentation, sheadvanced was found to be 6 weeks pregnant and was closely followed up without fibrosis (“80%") only ~50% glomeruli reported as completely scarred. remained with protein excretion in the 8- to 12-g/d range. further immunosuppressive treatment. Proteinuria This represented a difficult management dilemma. We proceeded with Circulating anti-PLA 2R levels declined but were still detectable. At 38 weeks, a healthy baby girl was born, 2 dose of IV rituximab (initial dose mg;orrepeated dose 750 mg 66-month postnatal visit. At the time of delivery, the mother still without proteinuria at500 birth at her subsequent weeks later). A month later, proteinuria persisted but count was of immunoglobulin G1 (IgG1), IgG3, and IgG4 subclasses, although at had detectable circulating anti-PLA2RCD19 suppressed to 0% (normal: 2-11) and absolute CD4ofcount wasanti-PLA R were found in cord blood. Potential reasons for the low titers. Only trace amounts IgG4 2 3 moderately suppressed at 199 mm (506-3142); hence, further discrepancy between anti-PLA2R levels in the maternal and fetal circulation are discussed. rituximab was deferred. On follow-up 1.5 years later, Cr decreased to Am J Kidney Dis. 67(5):775-778. ª 2016 by2 the National Kidney Foundation, Inc. ~4 mg/dL (estimated race-adjusted GFR 16-18 mL/min/1.73 m ), and random UPC near-normalized to 0.2 with normal serum albumin (4-4.6 INDEX WORDS: Membranous nephropathy (MN); nephrotic syndrome; pregnancy; M-type phospholipase A2 g/dL). She remained free of uremia or any clinical activity of SLE. In We intent to study the effect of PCSP on achieving the Triple Aim of receptor (PLA2R); autoantibody; placenta; rituximab; immunoglobulin G Healthcare (Ig G) subclass. the Institute for Improvement: better care for individuals summary, low-intensity rituximab therapy achieved complete and populations at lower per-capita cost2. remission of nephrotic syndrome. 1. https://www.acponline.org/advocacy/current_policy_papers/as sets/pcmh_neighbors.pdf 2. http://content.healthaffairs.org/content/27/3/759.full.html regnant patients with autoimmune disease may CASE REPORT
P
deliver newborns with a spectrum of clinical manifestations due to the transplacental passage of 2 circulating autoantibodies. Pregnant patients with CKD REPORT: A NEW COMMUNICATION TOOL lupus or myasthenia gravis can Memorial deliverHealth babies Konstantin Abramov, Jeffrey Stoff. UMass Care,with Worcester, MA, USA. corresponding disease in the neonate.1,2 Neonatal We implemented point-of-care CKD reports as a communication tool membranous nephropathy (MN) not associated with with the potential to improve CKD management and patient congenital infection was first described 1990 and engagement in a patient centered specialty practice or ain patient centered medical home. attributed to the passive transfer of maternal anti3 Patient identifiers, name of bodies to putative renal antigens. More than a decade nephrologist and date of last 4 later, Debiec et al identified theappointment, first antigen involved name of PCP. Latest GFR and CKD stage. a in such cases as neutral endopeptidase (NEP), Last 5 Cr and GFR values metalloprotease present on the surface of the podocyte Last 5 clinic blood pressures and involved in the proteolyticLast regulation of vasoacurine albumin/Cr, protein/Cr, bicarbonate, tive peptides. Debiec et al described a serum mother with a calcium, phosphorus (P), PTH, mutation preventing NEP expression who had formed vitamin D, potassium (K), anti-NEP antibodies due to fetomaternal alloimmuserum albumin, Hgb, HgbA1C. ACE/ARB, statin antibodies therapy. nization from a previous miscarriage; these Dietary instructions if either K were to cross the placenta and cause subepithelial or P are abnormal; reminders deposits in the fetal kidney of a subsequent pregabout stage specific referrals access,(PLA CKD 2R) nancy. M-type phospholipase (e.g. A2 vascular receptor education groups, transplant). wasData later identified as the major autoantigen for priis transferred weekly into 5 a CKD, which is in Excel format. MRN mary MN in adults. Little literature at the startabout of the must be entered to display reports on screen or to print exists clinic. Nephrologists reviewinreports during clinic often add handpregnancy outcomes patients withandnephrotic synwritten instruction, which patients keep at the end of the visit. drome due to primary MN, with no data available CKD reports appear to improve physician work flow and patient about pregnancy in PLA R-associated engagement. We are studying the 2effect of CKD reportsdisease. on clinical We outcomes, as well patient’s satisfaction andfirst understanding CKD. of present what weasbelieve to be the knownofcase Since the majority of CKD patients are managed by PCPs this tool may pregnancy in a patient with PLA R-associated MN have additional applications in primary care. 2 who was seropositive for anti-PLA2R autoantibodies throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118
A 39-year-old multiparous woman with morbid obesity presented for workup of severe nephrotic syndrome several months 4
before her current pregnancy. She had been treated for resistant PRES AS AN INITIAL PRESENTATION OF ESRD: Matthew hypertension andBrook lower-extremity edema during Abramson, Stony University Hospital, Stony Brookthe NY,past USA year, but PRES her proteinuria had been overlooked. Atwith presentation, serum Syndrome has been described in patients ESRD creatinine was mg/dL (corresponding to estimated undergoinglevel both PD and1.52 HD. Here, we describe a case of a patient with glomerular of 46 with mL/min/1.73 m2found as calculated no medical filtration history whorate presented blurred vision, to have by HTN, CKD Vmass and PRES Syndrome. –traceable 4-variable themalignant isotope-dilution spectrometry A 29 year-old El Salvadorian maleinpresented to the ED with MDRD [Modification of Diet Renal initially Disease] Study equa2 weeks of worsening and headaches. Patienturine was protein tion); serum albuminblurred level,vision 1.5 g/dL; and 24-hour controlling headaches with frequent NSAIDs. In the ED, BP was excretion, 29.2 g. The kidney biopsy specimen revealed features 248/143, and labetalol drip was started. Initial BUN/Cr was 83/10.8, typical of primary MN with additional strong staining for the and did not improve with IVF or diuresis. No other significant PLA within were immune deposits S1). Many of the 2R antigen electrolyte abnormalities present. 24-hour (Fig urine protein was 2.4g, subepithelial deposits were completely surrounded by with an output of 2L. EKG and ECHO demonstrated LVH. CT Head new basement membrane material S2), 35% of the showed mild nonspecific white matter(Fig disease. MRI and brain revealed abnormal signal within bilateral occipital lobes and cerebellar vermis, suggestive of PRES. Renal sonogram showed small kidneys with 1 increased cortical echogenicity. improved to the Section, 150s, and and patientDeFrom the Department of SBPs Medicine, Renal was transitioned to oral medications. Due to concern for persistent 4 partments of 2Obstetrics and Gynecology, 3Pediatrics, and Pavisual disturbance, ophthalmology was consulted, and hypertensive thology and Laboratory Medicine, Boston University Medical retinopathy was noted on exam; at this time, glucocorticoids were Center, Boston, decided not to beMA. administered. AVF was placed and patient was * Current affiliation: of Internal Medicine, Division discharged. He presentedDepartment to clinic for routine follow-up 2 weeks later of with Nephrology, University of Utah School of Medicine, resolution of vision changes. BUN/Cr slightly worsened toSalt Lake 102/9.5. City, UT. Secondary etiologies of HTN and rheumatologic workup were y negative. Current affiliation: Department of Obstetrics and Gynecology, The patient was then Hospital lost to follow-up. HeWashington, returned 7 months Medstar Washington Center, DC.later to clinic with 2June weeks29, of hand morning nausea and vomiting. BP 27, Received 2015.tremors, Accepted in revised form October was 210/120. He was sent to the ED; BUN/Cr was 158/21. Due to 2015. Originally published online December 29, 2015. uremic symptoms, he was started on HD, and eventually discharged Address correspondence to Laurence H. Beck Jr, MD, PhD, with outpatient dialysis follow-up established. Renal Section, X-504, 650 Albany St, Boston, MA 02118. PRES Syndrome is becoming more frequently described as case E-mail: [email protected] reports, usually due to HTN, and it is important to recognize PRES � 2016 by the that National Foundation, Inc.will often early, considering reversalKidney of the underlying etiology reverse the syndrome. 0272-6386
http://dx.doi.org/10.1053/j.ajkd.2015.10.031 775 A19