- Email: [email protected]
A Case of Fibrillary Glomerulonephritis in a Patient With Hepatitis C Infection
NKF 2016 Spring Clinical Meetings Abstracts
Case Report 357 A CASE OF FIBRILLARY GLOMERULONEPHRITIS IN A PATIENT WITH HEPATITIS C INFECTION Tina Thomas, Srijita Mukherjee, Jean-Luc Franck, Anees Quyyumi Emory University Department of Medicine, Division of Nephrology, Atlanta, Georgia, USA Fibrillary glomerulonephritis remains an uncommon cause of glomerular disease accounting for 0.5 to 1 percent of native renal biopsies. This is a case of a 53yo African American lady with a history of hypertension that was initially found to have renal insufficiency with a creatinine of 1.6 mg/dL in 2012 which later progressed to 2.1 mg/dL by 7/2013. Urinalysis at that time showed microscopic hematuria with 13 rbcs and a 24-hour urine collection demonstrated proteinuria of 3680mg per day. At the same time, the patient was noted to have Hepatitis C positivity for which she was referred to Gastroenterology for treatment. Pt was treated with ribavirin and sofosbuvir for 6 months with complete response. During this time, patient had a renal biopsy in 9/2013 that was consistent with fibrillary glomerulonephritis with moderate interstitial fibrosis and tubular atrophy of 65%. HIV, ANA, ANCA, anti-GBM, cryoglobulin were all found to be negative. C3 and C4 were normal. Serum and urine protein electrophoresis showed no paraprotein. In 2/2015, the patient was started on Cellcept and Prednisone at which point the creatinine was 2.1mg/dL. By the time of the follow up visit in 5/2015, the patient’s creatinine had risen to 2.6mg/dL and then 3.2mg/dL by 7/2015. The decision was made at that time to switch immunosuppression to Rituximab. By 10/2015, the patient was still waiting for approval for Rituximab. The creatinine had progressed to 4.2mg/dL and then 4.9mg/dL by the end of the month. The patient is currently being evaluated for potential renal transplant. Fibrillary glomerulonephritis is a rare yet devastating renal disease and is associated with Hepatitis C though the exact prevalence is unknown. The outcome for these patients is frequently poor. Early diagnosis and initiation of treatment is the best chance at preserving maximal renal function. For this reason, fibrillary glomerulonephritis should be considered among other more common nephropathies in the differential diagnosis for patients with Hepatitis C and renal insufficiency.
358 DEVELOPMENT AND IMPLEMENTATION OF A PHARMACSITLED PATIENT EDUCATION AND PROVIDER COMMUNICATION SERVICE FOR MEDICATION-RELATED ANGIOEDEMA AND ANAPHYLAXIS REQUIRING HOSPITALIZATION: Andrew Ticcioni, Robert Mueller, Tahmeena Siddiqui Wheaton Franciscan – St. Joseph Campus, Milwaukee, WI, USA Angioedema and anaphylaxis are rare, but life-threatening reactions to medications. Avoiding repeat exposure to an agent that has caused such a reaction is paramount, but challenging in the face of limited patient comprehension, fragmented medical record systems, and the utilization of multiple providers. The high utilization of ACE inhibitors for chronic illnesses in combination with our hospital’s predominantly black patient population requires our facility to treat patients with angioedema quite regularly. Here we aim to describe the development and implementation of a pharmacist-led service that provides face-to-face patient education about these drug reactions in our community teaching hospital. Patients are identified by a daily report that captures admission diagnoses with key words related to both angioedema and anaphylaxis. A pharmacist reviews the patient record and provides bedside education to the patient about his/her medication-related reaction. Additionally, the pharmacist notifies the patient’s pharmacy, primary care provider and prescriber of the offending agent. Documentation of these activities is included in the patient’s permanent medical record. Healthcare facilities may consider starting a similar service to improve patient education and communication between medical providers. The inpatient pharmacist is well suited to coordinate these efforts.
A108
359 DECEIVED BY THE OBVIOUS: HIDING BEHIND THE CLOAK OF VACULITIS: Sumithra Tirunagaram1, Hannah Tiu2 1Marshfield Clinic, Marshfield, WI, USA 2 University of Michigan, Ann Arbor, MI, USA
Pregnancy in a Patient Wit and Circulating Anti-P
Immune complex formation leading to vasculitis and glomerulonephritis is a 1, known phenomenon of infective endocarditis (IE). Laith Al-Rabadi, MBBS, * Rivka A 70 year old man whose only complaints were weight loss, decreased Jennifer E. Ballard, MD,2,y Alan appetite and fatigue was seen initially at an outside facility. His physical exam 1 David was remarkable only for a purpuric rash on his medial thighs. He was later J. Salant, MD, transferred to our hospital for progressive renal failure after extensive work up was reported to be inconclusive. Serologies were repeated and mostly is decreased little information about pregnancy o unremarkable except for non-hemolytic anemia,There a slightly C3, positive rheumatoid factor with normal CCP especially Ab, and Typethose III with circulating autoantibod cryoglobulinemia. Skin biopsy was performed and showed leukocytoclastic autoantigen in primary MN. We present what vasculitis. He was initiated on high dose steroids with a concernwoman for a 39-year-old with PLA2R-associate cryoglobulinemic vasculitis. Thereafter patient quickly deteriorated and anasarca, hypoalbuminemia (albumin, 1.3-2. developed clinical signs of sepsis. Kidney biopsy showed diffuse proliferative revealed MN deposition. with staining for PLA2R, a necrotizing glomerulonephritis with crescentsopsy and immune complex She not respond to conservative therapy a Blood cultures were obtained and were positive fordid streptococcus viridans. Imaging revealed mitral valve endocarditis. His course weeks was complicated by Several after presentation, she was fou multi-organ failure requiring dialysis, CNS vasculitis subarachnoid further with immunosuppressive treatment. Protei hemorrhage, DIC, perforated viscus requiringCirculating exploratory laparotomy, R levels declined but w anti-PLA2enteral nutrition via PEG, ventilator support and eventual tracheostomy. Once the without proteinuria at birth or at her subseque patient was started on appropriate antibiotics, his renal function slowly improved and he eventually underwent mitralhad valvedetectable repair. Priorcirculating to our care, anti-PLA2R of imm low titers. Only trace amounts of IgG4 ant this patient neither had signs nor symptoms suspicious for an infectious etiology. discrepancy between anti-PLA2R levels in th This case serves as a reminder that mixed cryoglobulinemia and 67(5):775-778. ª 2016 by Am J Kidney Dis. glomerulonephritis can be a presentation of IE and that delay in the diagnosis due to its atypical presentation can lead to increased morbidity and mortality INDEX WORDS: Membranous nephropathy ( for patients.
receptor (PLA2R); autoantibody; placenta; ritu
P
regnant patients with autoimmune disease deliver newborns with a spectrum of cl manifestations due to the transplacental passa 360 circulating autoantibodies. Pregnant patients OPTIMIZING CKD DIAGNOSIS DELIVERY lupus orRoney myasthenia deliver babies 2, Eve Kerr1,3, gravis 1, Hannah Tiu1, Angela Fagerlin1,2,3 , Meghan Akinlolu Ojocan 1,2 Julie Wright Nunes1,2 1 Internalcorresponding Medicine, University ofdisease Michigan 2 Center for neonate. in the Neo Bioethics and Social Sciencesmembranous in Medicine 3 Veterans Administration Ann nephropathy (MN) not associated Arbor Center for Clinical Management Research, Ann Arbor, MI, USA congenital infection was first described in 199 There is limited knowledge about how providers inform patients of their CKD to timing the and passive of maternal diagnosis. We elicited providerattributed perspectives about languagetransfer used to discuss CKD diagnosis in order to designto an putative education intervention to optimize 3 More than a d bodies renal antigens. diagnosis messaging and improve patient CKD awareness. later, Debiec et al4 identified the first antigen inv Using root/cause analysis and quality function deployment we conducted in such asdoctors neutral semi-structured interviews. Primary care andcases nephrology were endopeptidase (NE metalloprotease present individually interviewed between January-October 2014. Interviewson werethe surface of the pod audiotaped, transcribed, and analyzed using contentinanalysis. and involved the proteolytic regulation of va Twenty four physicians completed interviews including 17 nephrologists and tive peptides. Debiec et al described a mother w 7 primary care providers. Four key themes emerged from 215 statements: 1) mutation preventing NEP expression who had fo Perspectives on informing patients about CKD diagnosis (37 statements) 2) Timing of diagnosis messaginganti-NEP (38 statements) 3) Language used antibodies dueto convey to fetomaternal alloi diagnosis (42 statements) andnization 4) Challenges in diagnosis delivery (143 from a previous miscarriage; these antib statements). 92% of physicians agreed patients should be informed of their were to cross the placenta cause subepit CKD, and 67% believed communication should occur early in the spectrumand of deposits inused thethefetal kidney of a subsequent the disease. Only 37.5% of providers said they words “Chronic Kidney Disease” specifically during diagnosis Communication nancy.communication. M-type phospholipase A2 receptor (PL challenges cited by providers included: perception that kidney disease is a was later identified as the major autoantigen fo difficult concept to explain, that lack of patient symptoms leads to 5 patient denial mary MN in adults. Little literature exists and low public awareness of CKD in general. Most providers felt patients should be informed of CKD, but timing and pregnancy outcomes in patients with nephrotic language during diagnosis messaging variable. from this study with no data ava dromearedue toInsights primary MN, offer modifiable targets for education interventions including an opportunity to about pregnancy in PLA 2R-associated disease create materials that help providers efficiently convey diagnosis information present what we believe to about a disease that may be perceived as inherently difficult to explain. be the first known ca pregnancy in a patient with PLA2R-associated who was seropositive for anti-PLA2R autoantib throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118
Case Report 357 A CASE OF FIBRILLARY GLOMERULONEPHRITIS IN A PATIENT WITH HEPATITIS C INFECTION Tina Thomas, Srijita Mukherjee, Jean-Luc Franck, Anees Quyyumi Emory University Department of Medicine, Division of Nephrology, Atlanta, Georgia, USA Fibrillary glomerulonephritis remains an uncommon cause of glomerular disease accounting for 0.5 to 1 percent of native renal biopsies. This is a case of a 53yo African American lady with a history of hypertension that was initially found to have renal insufficiency with a creatinine of 1.6 mg/dL in 2012 which later progressed to 2.1 mg/dL by 7/2013. Urinalysis at that time showed microscopic hematuria with 13 rbcs and a 24-hour urine collection demonstrated proteinuria of 3680mg per day. At the same time, the patient was noted to have Hepatitis C positivity for which she was referred to Gastroenterology for treatment. Pt was treated with ribavirin and sofosbuvir for 6 months with complete response. During this time, patient had a renal biopsy in 9/2013 that was consistent with fibrillary glomerulonephritis with moderate interstitial fibrosis and tubular atrophy of 65%. HIV, ANA, ANCA, anti-GBM, cryoglobulin were all found to be negative. C3 and C4 were normal. Serum and urine protein electrophoresis showed no paraprotein. In 2/2015, the patient was started on Cellcept and Prednisone at which point the creatinine was 2.1mg/dL. By the time of the follow up visit in 5/2015, the patient’s creatinine had risen to 2.6mg/dL and then 3.2mg/dL by 7/2015. The decision was made at that time to switch immunosuppression to Rituximab. By 10/2015, the patient was still waiting for approval for Rituximab. The creatinine had progressed to 4.2mg/dL and then 4.9mg/dL by the end of the month. The patient is currently being evaluated for potential renal transplant. Fibrillary glomerulonephritis is a rare yet devastating renal disease and is associated with Hepatitis C though the exact prevalence is unknown. The outcome for these patients is frequently poor. Early diagnosis and initiation of treatment is the best chance at preserving maximal renal function. For this reason, fibrillary glomerulonephritis should be considered among other more common nephropathies in the differential diagnosis for patients with Hepatitis C and renal insufficiency.
358 DEVELOPMENT AND IMPLEMENTATION OF A PHARMACSITLED PATIENT EDUCATION AND PROVIDER COMMUNICATION SERVICE FOR MEDICATION-RELATED ANGIOEDEMA AND ANAPHYLAXIS REQUIRING HOSPITALIZATION: Andrew Ticcioni, Robert Mueller, Tahmeena Siddiqui Wheaton Franciscan – St. Joseph Campus, Milwaukee, WI, USA Angioedema and anaphylaxis are rare, but life-threatening reactions to medications. Avoiding repeat exposure to an agent that has caused such a reaction is paramount, but challenging in the face of limited patient comprehension, fragmented medical record systems, and the utilization of multiple providers. The high utilization of ACE inhibitors for chronic illnesses in combination with our hospital’s predominantly black patient population requires our facility to treat patients with angioedema quite regularly. Here we aim to describe the development and implementation of a pharmacist-led service that provides face-to-face patient education about these drug reactions in our community teaching hospital. Patients are identified by a daily report that captures admission diagnoses with key words related to both angioedema and anaphylaxis. A pharmacist reviews the patient record and provides bedside education to the patient about his/her medication-related reaction. Additionally, the pharmacist notifies the patient’s pharmacy, primary care provider and prescriber of the offending agent. Documentation of these activities is included in the patient’s permanent medical record. Healthcare facilities may consider starting a similar service to improve patient education and communication between medical providers. The inpatient pharmacist is well suited to coordinate these efforts.
A108
359 DECEIVED BY THE OBVIOUS: HIDING BEHIND THE CLOAK OF VACULITIS: Sumithra Tirunagaram1, Hannah Tiu2 1Marshfield Clinic, Marshfield, WI, USA 2 University of Michigan, Ann Arbor, MI, USA
Pregnancy in a Patient Wit and Circulating Anti-P
Immune complex formation leading to vasculitis and glomerulonephritis is a 1, known phenomenon of infective endocarditis (IE). Laith Al-Rabadi, MBBS, * Rivka A 70 year old man whose only complaints were weight loss, decreased Jennifer E. Ballard, MD,2,y Alan appetite and fatigue was seen initially at an outside facility. His physical exam 1 David was remarkable only for a purpuric rash on his medial thighs. He was later J. Salant, MD, transferred to our hospital for progressive renal failure after extensive work up was reported to be inconclusive. Serologies were repeated and mostly is decreased little information about pregnancy o unremarkable except for non-hemolytic anemia,There a slightly C3, positive rheumatoid factor with normal CCP especially Ab, and Typethose III with circulating autoantibod cryoglobulinemia. Skin biopsy was performed and showed leukocytoclastic autoantigen in primary MN. We present what vasculitis. He was initiated on high dose steroids with a concernwoman for a 39-year-old with PLA2R-associate cryoglobulinemic vasculitis. Thereafter patient quickly deteriorated and anasarca, hypoalbuminemia (albumin, 1.3-2. developed clinical signs of sepsis. Kidney biopsy showed diffuse proliferative revealed MN deposition. with staining for PLA2R, a necrotizing glomerulonephritis with crescentsopsy and immune complex She not respond to conservative therapy a Blood cultures were obtained and were positive fordid streptococcus viridans. Imaging revealed mitral valve endocarditis. His course weeks was complicated by Several after presentation, she was fou multi-organ failure requiring dialysis, CNS vasculitis subarachnoid further with immunosuppressive treatment. Protei hemorrhage, DIC, perforated viscus requiringCirculating exploratory laparotomy, R levels declined but w anti-PLA2enteral nutrition via PEG, ventilator support and eventual tracheostomy. Once the without proteinuria at birth or at her subseque patient was started on appropriate antibiotics, his renal function slowly improved and he eventually underwent mitralhad valvedetectable repair. Priorcirculating to our care, anti-PLA2R of imm low titers. Only trace amounts of IgG4 ant this patient neither had signs nor symptoms suspicious for an infectious etiology. discrepancy between anti-PLA2R levels in th This case serves as a reminder that mixed cryoglobulinemia and 67(5):775-778. ª 2016 by Am J Kidney Dis. glomerulonephritis can be a presentation of IE and that delay in the diagnosis due to its atypical presentation can lead to increased morbidity and mortality INDEX WORDS: Membranous nephropathy ( for patients.
receptor (PLA2R); autoantibody; placenta; ritu
P
regnant patients with autoimmune disease deliver newborns with a spectrum of cl manifestations due to the transplacental passa 360 circulating autoantibodies. Pregnant patients OPTIMIZING CKD DIAGNOSIS DELIVERY lupus orRoney myasthenia deliver babies 2, Eve Kerr1,3, gravis 1, Hannah Tiu1, Angela Fagerlin1,2,3 , Meghan Akinlolu Ojocan 1,2 Julie Wright Nunes1,2 1 Internalcorresponding Medicine, University ofdisease Michigan 2 Center for neonate. in the Neo Bioethics and Social Sciencesmembranous in Medicine 3 Veterans Administration Ann nephropathy (MN) not associated Arbor Center for Clinical Management Research, Ann Arbor, MI, USA congenital infection was first described in 199 There is limited knowledge about how providers inform patients of their CKD to timing the and passive of maternal diagnosis. We elicited providerattributed perspectives about languagetransfer used to discuss CKD diagnosis in order to designto an putative education intervention to optimize 3 More than a d bodies renal antigens. diagnosis messaging and improve patient CKD awareness. later, Debiec et al4 identified the first antigen inv Using root/cause analysis and quality function deployment we conducted in such asdoctors neutral semi-structured interviews. Primary care andcases nephrology were endopeptidase (NE metalloprotease present individually interviewed between January-October 2014. Interviewson werethe surface of the pod audiotaped, transcribed, and analyzed using contentinanalysis. and involved the proteolytic regulation of va Twenty four physicians completed interviews including 17 nephrologists and tive peptides. Debiec et al described a mother w 7 primary care providers. Four key themes emerged from 215 statements: 1) mutation preventing NEP expression who had fo Perspectives on informing patients about CKD diagnosis (37 statements) 2) Timing of diagnosis messaginganti-NEP (38 statements) 3) Language used antibodies dueto convey to fetomaternal alloi diagnosis (42 statements) andnization 4) Challenges in diagnosis delivery (143 from a previous miscarriage; these antib statements). 92% of physicians agreed patients should be informed of their were to cross the placenta cause subepit CKD, and 67% believed communication should occur early in the spectrumand of deposits inused thethefetal kidney of a subsequent the disease. Only 37.5% of providers said they words “Chronic Kidney Disease” specifically during diagnosis Communication nancy.communication. M-type phospholipase A2 receptor (PL challenges cited by providers included: perception that kidney disease is a was later identified as the major autoantigen fo difficult concept to explain, that lack of patient symptoms leads to 5 patient denial mary MN in adults. Little literature exists and low public awareness of CKD in general. Most providers felt patients should be informed of CKD, but timing and pregnancy outcomes in patients with nephrotic language during diagnosis messaging variable. from this study with no data ava dromearedue toInsights primary MN, offer modifiable targets for education interventions including an opportunity to about pregnancy in PLA 2R-associated disease create materials that help providers efficiently convey diagnosis information present what we believe to about a disease that may be perceived as inherently difficult to explain. be the first known ca pregnancy in a patient with PLA2R-associated who was seropositive for anti-PLA2R autoantib throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118