- Email: [email protected]
A Randomized Controlled Trial of Taurolidine Heparin Versus Taurolidine Urokinase Lock to Improve Catheter Function in Hemodialysis
NKF 2016 Spring Clinical Meetings Abstracts
Case Report 225
227
DIGITAL ISCHEMIA: A RARE PRESENTATION OF GRANULOMATOSIS WITH POLYANGITIS
MY BONES ARE KILLING ME! Pregnancy in a Patient With Primary Membranous Nephropathy Azeem Mohammed, Matthew Diamond, Nardos Belayneh, Augusta,University, Augusta, GA USA Circulating Anti-PLA2R Antibodies: A Case Report Ritu Modi, Reshmaand Shah, Rahim Dhanani, Sunil Pokharel, Introduction: We present the case of a patient diagnosed with
Krishnakumar Hongalgi, Rafia Chaudhry, Adam Austin, Syed S 1, Haqqie Albany Medical College, Albany MBBS, NY Laith Al-Rabadi, * Rivka
depression and suicidal ideation because of her chronic bone pain from
1 Ayalon, MD, Ramon G.Chronic Bonegio, PhD,1 disease that hypophosphatemia. pain isMD, a debilitating 2,y 3 4 compounds illness. Unmasking occult causes of chronic Granulomatosis with polyangitis (GPA) is a small vasculitis Jennifer E. Ballard, MD,vesselAlan M. Fujii, MD, Joelpsychiatric M. Henderson, MD, PhD, pain is arduous but leads to significant with multi-organ involvement including RPGN and cutaneous1 1 improvement in the patient’s David J. Salant, MD, and Laurence H. Beck Jr, MD, PhD quality of life. vasculitis, involving dermal and subcutaneous small vessels (venules
and less commonly arterioles). We report a rare case of GPA presenting with digital ischemia and dry gangrene.
Case description: The patient is a 50 year old African American female with no previous psychiatric history who was admitted to the psychiatry a suicidal nephropathy attempt that she attributed to There is littlefemale information about pregnancy outcomes in patients withservice activeafter membranous (MN), A 72 year old Caucasian presented with left hand ischemia arthralgia A and receptor recurrent fractures for the (PLA2R), theprevious major18months. those with circulating autoantibodies to M-typeunbearable phospholipase 2 involving especially 2nd to 5th digit that evolved into dry gangrene of distal 1/3rd At outside facilities, the patient was treated for fibromyalgia then for autoantigen in primary MN. We what distal we believe to be the first known case ofrickets successful in We were of digits. Angiogram revealed a diminutive rightpresent brachial artery presumed hypophosphatemic with nopregnancy improvement. to the elbow, with sluggish flow in the radial and ulnar arteries, year prior pregnancy, the patient developed a 39-year-old woman with PLA2R-associated MN. In theconsulted for toevaluation of incidental hypophosphatemia. Labs terminating in the proximal forearm. There was no evidence of g/dL), and proteinuria indicated serum phosphorous 1.2 mg/dL (trend Kidney revealed biphosphorous anasarca, hypoalbuminemia (albumin, 1.3-2.2 (protein excretion, 29.2 g/d). thrombosis, after which intra-arterial nitroglycerin resulted in improved < 2seropositive mg/dL over thefor past 4 months), serum PTH 183 ng/mL, serum was anti-PLA opsy revealed MN with staining for PLA2R, and the patientlevel 2R autoantibodies. blood flow through the radial artery. Work up revealed a positive c1, 25(OH)D 16 pg/mL, urine phosphate 700 mg/dL and fractional She did not respond to conservative therapy and was treated with intravenous rituximab (2 doses of 1 g each). ANCA (titer: 5120) and anti-proteinase 3 (titer: 1117), with p-ANCA excretion of phosphate 50%. Given the profound renal phosphate Several weekslevels, after BUN presentation, she wascreatinine found to be 6 weeks pregnant and was followed up withoutsyndrome. (titer: <20), normal C3/C4 44 mg/dl and serum wasting, we investigated theclosely possibility of a paraneoplastic 2.0 mg/dl,further urine showing 3+ protein and blood. Renal biopsy immunosuppressive treatment. Proteinuria remained with protein the23 8- level to 12-g/d Further work up excretion revealed a inFGF of 364range. RU/mL and an demonstrated pauci-immune crescentic glomerulonephritis, consistent octreotide showed a a right proximal mesenchymal At scan 38 weeks, healthy babyfibular girl was born, tumor. Circulating anti-PLA 2R levels declined but were still detectable. with GPA.without She wasproteinuria treated with pulse Solu-Medrol, oral prednisone6-month postnatal After a wide the of tumor, serumthe phosphate at birth or at her subsequent visit.excision At the of time delivery, motherand stillPTH levels and IV Cytoxan x 1 dose (later transitioned to rituximab due to normalized. Patient reported satisfaction with resolution of her of immunoglobulin G1 (IgG1), IgG3, and IgG4 subclasses, although at had detectable circulating anti-PLA 2R degree profound leukopenia) with clinical improvement in the of hand arthralgia. found in cord blood. Potential reasons for theamong the lowalso titers. Only trace amounts of IgG4 anti-PLA2R were Discussion: ischemia and stabilization of serum creatinine, Tumor-induced osteomalacia (TIO) is counted in the maternal and fetalof circulation are discussed. discrepancy between anti-PLA2R levels ranks endocrine neoplasms with a striking presentation and, when Cutaneous manifestations occur in 20% of patients with GPA; resected,Foundation, a dramatic andInc. satisfying resolution. The patient presents with J Kidney 2016 ofby the National Kidney however, aAm literature searchDis. did 67(5):775-778. not reveal any priorªreports myalgias, arthralgia, fatigue and recurrent fractures. Biochemical involvement of a medium sized vessel, i.e. medial muscular artery and studies are consistent with an acquired renal phosphate wasting acute digital ischemia associated with GPA. Thisnephropathy case highlights(MN); the nephrotic syndrome; pregnancy; M-type phospholipase A2 INDEX WORDS: Membranous syndrome, leading us to diagnose occult malignancy. Resection of unique finding of critical limb ischemia possibly associated with GPA, receptor (PLA R); autoantibody; placenta; rituximab; immunoglobulin G) subclass. 2 tumor leads G to (Ig rapid correction of electrolyte abnormalities and which requires a high index of suspicion and prompt diagnosis for significant improvement in quality of life. This case demonstrates the appropriate immunosuppressive therapy to prevent further ischemia, importance of carefully investigating the cause of unexpected gangrene and vasculitic involvement. pathologic fractures in the setting of chronic bone pain, as a reversible pathology may be present. regnant patients with autoimmune disease may
P
deliver newborns with a spectrum of clinical manifestations due to the transplacental passage of 226 circulating autoantibodies. Pregnant patients with A RANDOMIZED CONTROLLED TRIAL OF TAUROLIDINE lupus or myasthenia gravis can deliverLOCK babies HEPARIN VERSUS TAUROLIDINE UROKINASE TO with IMPROVE CATHETER FUNCTION corresponding disease in theIN HEMODIALYSIS. neonate.1,2 Neonatal Zafar Mohammad, Abdullah Hamad, Ahmad Hamdy, Prim Chandra, membranous nephropathy (MN) notFadwa associated with Tarek Abdul- latif, Rania Ibrahim, Tarek Fouda, Al-Ali. Fahad Bin Jassim Kidney Center,was Hamad General Hospital, Doha, Qatar. and congenital infection first described in 1990 The use of tunneled catheters in hemodialysis is one of the leading attributed to theandpassive of (BFR) maternal anticauses of morbidity mortality. transfer Blood flow rate is a surrogate 3 outcome a markerrenal of catheter malfunction. We compared bodies to and putative antigens. More than athedecade effects of two catheter solutions (Taurolidine citrate with later, Debiec et (Taurolock/Hep) al4 locking identified the Taurolidine first antigen heparin 500 units versus citrate involved with 25000 units regarding BFR and Kt/v. in Urokinase such cases as (Taurolock/U) neutral endopeptidase (NEP), a This is a prospective randomized controlled trial in which all patients metalloprotease present on the surface of the podocyte who were undergoing ambulatory regular hemodialysis in Qatar with andtunneled involved theincluded. proteolytic regulation of vasoaccatheterinwere All patients were randomized to Taurolock/Hep or Taurolock/U on 1:1 basis using computertivereceive peptides. Debiec et al described a mother with a generated program. Patients were followed for 6 months. BFR in all mutation preventing NEP expression who had formed sessions, monthly Kt/V were monitored. Sub-analysis was done for BFR sessions post Taurolock/U versusalloimmuthe anti-NEP antibodies due(BFR-Taurolock/U) to fetomaternal comparable sessions in Taurolock/Hep group (BFR-Taurolock/Hep). nization from a previous miscarriage; these antibodies 177 participated in the study. 93 Patients in Taurolock/Hep and 84 in the Taurolock/U. catheter age, BFRcause and venous pressures were to cross Age, the sex, placenta and subepithelial before study, use of antiplatelet or warfarin and co-morbidities did not deposits in the fetal kidney of a subsequent differ between the two groups. There was no significant differencepregin mean BFR in all sessions between the twoA groups (273+/-7 ml/min nancy. M-type phospholipase (PLAin2R) 2 receptor versus 281+/-8 ml/min in Taurolock/U) (pValue 0.15). wasTaurolock/Hep later identified as the major autoantigen BFR-Taurolock/U was significantly higher compared to BFR- for pri5 Taurolock/Hep most of the 24 week study period. Monthly was mary MN ininadults. Little literature existsKt/vabout slightly higher in the Taurolock/U but was not statistically significant pregnancy outcomes in patients with nephrotic syn(1.22 versus 1.11) (pValue >0.05). There were no reported serious drome primary MN, withduring nostudy. data available adversedue eventstorelated to treatment reported In apregnancy randomized controlled trial comparing Taurolock/Hep to about in PLA disease. We 2R-associated Taurolock/U catheter lock solution, we found no statistically significant present what we believe to be the first known case difference in overall BFR or monthly Kt/v. Sub analysis showed that of BFR-Taurolock/U higher than BFR-Taurolock/Hep. Although this pregnancy in a was patient with PLA2R-associated MN difference was statistically significant but was not clinically important who(only was seropositive for anti-PLA R autoantibodies 2 13 ml/min difference). throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118
CASE REPORT
A 39-year-old multiparous woman with morbid obesity presented for workup of severe nephrotic syndrome several months 228
before her current pregnancy. She had been treated for resistant TEARS OF JOY? EXTRAGLANDULAR SJӦGREN SYNDROME hypertension lower-extremity edema during the past year, LEADING TOand CRYOGLOBULIN-ASSOCIATED but her proteinuria had been overlooked. GLOMERULONEPHRITIS AND MYOSITIS At presentation, serum Javier Monserrate, Jennifer Jaynes, Steven Foster, Roberto Collazocreatinine level was 1.52 mg/dL (corresponding to estimated 2 Maldonado.filtration Methodist rate Dallas System, Dallas,mTX, glomerular ofHealth 46 mL/min/1.73 asUSA. calculated by Sjögren’s syndrome is an autoimmune inflammatory disorder thecharacterized isotope-dilution spectrometry –traceable 4-variable by chronic mass inflammation of exocrine glandular tissues, MDRD [Modification of Diet in Renal Disease] mainly the lacrimal and salivary glands. However, almost anyStudy organ equation); serum level, 1.5 urineRenal protein system can bealbumin affected, including theg/dL; kidneysand and24-hour muscle tissue. involvement usually presents as tubulointerstitial nephritis or excretion, 29.2 g. The kidney biopsy specimen revealed features glomerulonephritis. this report present a case of a 59 y/o African typical of primaryInMN with we additional strong staining for the American man with a past medical history of Chronic Kidney Disease PLA R antigen within immune deposits (Fig S1). 2 (CKD) Stage III, Hepatitis C (HCV), Hepatitis B (HBV), andMany of the subepithelial deposits were completely surrounded by new Hypertension who presented with severe myalgia and Acute Kidney Injury (AKI) Stage III, without sicca symptoms. On admission, basement membrane material (Fig S2), and 35% of the laboratories showed a creatinine was 3.4 mg/dl (baseline of 1.5 mg/dl), active urinary sediment with hematuria (RBCs 10-25/HPF) and subnephrotic proteinuria. Creatine phosphokinase (CPK) levels were 1 From theSerologic Department of Medicine, Renal Type Section, and De1149 U/L. workup was positive for elevated 2 2 3 Cryoglobulins, undetectable and C4 levels, C3 levels of 77 mg/dl, along partments of Obstetrics Gynecology, Pediatrics, and 4Pawith elevated of SS-A Ro 60 kDa antibodies RF of 111.9Medical thology and titers Laboratory Medicine, Bostonand University IU/ml. HCV and HBV titers were non-detectable. The kidney biopsy Center, Boston, MA. revealed mesangial hypercellularity with increased matrix material; * Current affiliation: of Internal Immunofluorescence wasDepartment positive for IgG, IgM, C3q,Medicine, Lambda andDivision ofkappa Nephrology, of Utah of granular Medicine, Salt Lake staining inUniversity a global peripheral andSchool mesangial pattern. There was focal positive staining within the tubules for IgA, kappa and City, UT. y lambda. Electron microscope evaluationof revealed podocyte Current affiliation: Department Obstetrics andeffacement, Gynecology, mesangial deposits, along with mesangial interposition and prominent Medstar Washington Hospital Center, Washington, DC. subendothelial electron dense deposits. The diagnosis of myositis with Received June 29, 2015. Accepted in revised form October 27, Cryoglobulin-associated membranoproliferative glomerulonephritis 2015. Originally online December 29,The 2015. (MPGN) secondarypublished to Sjögren’s syndrome was made. patient was aggressively treated with systemic plasmapheresis andMD, PhD, Address correspondence to steroids, Laurence H. Beck Jr, rituximab, which resulted650 in normalization of CPK levels Renal Section, X-504, Albany St, Boston, MA and 02118. E-mail: resolution of the AKI. It is therefore important for the nephrologist to [email protected] remain aware that myositis and cryoglobulin-associated MPGN can be � 2016 by the National Kidney Foundation, Inc. initial manifestations of Sjogren syndrome.
0272-6386 http://dx.doi.org/10.1053/j.ajkd.2015.10.031
775 A75
Case Report 225
227
DIGITAL ISCHEMIA: A RARE PRESENTATION OF GRANULOMATOSIS WITH POLYANGITIS
MY BONES ARE KILLING ME! Pregnancy in a Patient With Primary Membranous Nephropathy Azeem Mohammed, Matthew Diamond, Nardos Belayneh, Augusta,University, Augusta, GA USA Circulating Anti-PLA2R Antibodies: A Case Report Ritu Modi, Reshmaand Shah, Rahim Dhanani, Sunil Pokharel, Introduction: We present the case of a patient diagnosed with
Krishnakumar Hongalgi, Rafia Chaudhry, Adam Austin, Syed S 1, Haqqie Albany Medical College, Albany MBBS, NY Laith Al-Rabadi, * Rivka
depression and suicidal ideation because of her chronic bone pain from
1 Ayalon, MD, Ramon G.Chronic Bonegio, PhD,1 disease that hypophosphatemia. pain isMD, a debilitating 2,y 3 4 compounds illness. Unmasking occult causes of chronic Granulomatosis with polyangitis (GPA) is a small vasculitis Jennifer E. Ballard, MD,vesselAlan M. Fujii, MD, Joelpsychiatric M. Henderson, MD, PhD, pain is arduous but leads to significant with multi-organ involvement including RPGN and cutaneous1 1 improvement in the patient’s David J. Salant, MD, and Laurence H. Beck Jr, MD, PhD quality of life. vasculitis, involving dermal and subcutaneous small vessels (venules
and less commonly arterioles). We report a rare case of GPA presenting with digital ischemia and dry gangrene.
Case description: The patient is a 50 year old African American female with no previous psychiatric history who was admitted to the psychiatry a suicidal nephropathy attempt that she attributed to There is littlefemale information about pregnancy outcomes in patients withservice activeafter membranous (MN), A 72 year old Caucasian presented with left hand ischemia arthralgia A and receptor recurrent fractures for the (PLA2R), theprevious major18months. those with circulating autoantibodies to M-typeunbearable phospholipase 2 involving especially 2nd to 5th digit that evolved into dry gangrene of distal 1/3rd At outside facilities, the patient was treated for fibromyalgia then for autoantigen in primary MN. We what distal we believe to be the first known case ofrickets successful in We were of digits. Angiogram revealed a diminutive rightpresent brachial artery presumed hypophosphatemic with nopregnancy improvement. to the elbow, with sluggish flow in the radial and ulnar arteries, year prior pregnancy, the patient developed a 39-year-old woman with PLA2R-associated MN. In theconsulted for toevaluation of incidental hypophosphatemia. Labs terminating in the proximal forearm. There was no evidence of g/dL), and proteinuria indicated serum phosphorous 1.2 mg/dL (trend Kidney revealed biphosphorous anasarca, hypoalbuminemia (albumin, 1.3-2.2 (protein excretion, 29.2 g/d). thrombosis, after which intra-arterial nitroglycerin resulted in improved < 2seropositive mg/dL over thefor past 4 months), serum PTH 183 ng/mL, serum was anti-PLA opsy revealed MN with staining for PLA2R, and the patientlevel 2R autoantibodies. blood flow through the radial artery. Work up revealed a positive c1, 25(OH)D 16 pg/mL, urine phosphate 700 mg/dL and fractional She did not respond to conservative therapy and was treated with intravenous rituximab (2 doses of 1 g each). ANCA (titer: 5120) and anti-proteinase 3 (titer: 1117), with p-ANCA excretion of phosphate 50%. Given the profound renal phosphate Several weekslevels, after BUN presentation, she wascreatinine found to be 6 weeks pregnant and was followed up withoutsyndrome. (titer: <20), normal C3/C4 44 mg/dl and serum wasting, we investigated theclosely possibility of a paraneoplastic 2.0 mg/dl,further urine showing 3+ protein and blood. Renal biopsy immunosuppressive treatment. Proteinuria remained with protein the23 8- level to 12-g/d Further work up excretion revealed a inFGF of 364range. RU/mL and an demonstrated pauci-immune crescentic glomerulonephritis, consistent octreotide showed a a right proximal mesenchymal At scan 38 weeks, healthy babyfibular girl was born, tumor. Circulating anti-PLA 2R levels declined but were still detectable. with GPA.without She wasproteinuria treated with pulse Solu-Medrol, oral prednisone6-month postnatal After a wide the of tumor, serumthe phosphate at birth or at her subsequent visit.excision At the of time delivery, motherand stillPTH levels and IV Cytoxan x 1 dose (later transitioned to rituximab due to normalized. Patient reported satisfaction with resolution of her of immunoglobulin G1 (IgG1), IgG3, and IgG4 subclasses, although at had detectable circulating anti-PLA 2R degree profound leukopenia) with clinical improvement in the of hand arthralgia. found in cord blood. Potential reasons for theamong the lowalso titers. Only trace amounts of IgG4 anti-PLA2R were Discussion: ischemia and stabilization of serum creatinine, Tumor-induced osteomalacia (TIO) is counted in the maternal and fetalof circulation are discussed. discrepancy between anti-PLA2R levels ranks endocrine neoplasms with a striking presentation and, when Cutaneous manifestations occur in 20% of patients with GPA; resected,Foundation, a dramatic andInc. satisfying resolution. The patient presents with J Kidney 2016 ofby the National Kidney however, aAm literature searchDis. did 67(5):775-778. not reveal any priorªreports myalgias, arthralgia, fatigue and recurrent fractures. Biochemical involvement of a medium sized vessel, i.e. medial muscular artery and studies are consistent with an acquired renal phosphate wasting acute digital ischemia associated with GPA. Thisnephropathy case highlights(MN); the nephrotic syndrome; pregnancy; M-type phospholipase A2 INDEX WORDS: Membranous syndrome, leading us to diagnose occult malignancy. Resection of unique finding of critical limb ischemia possibly associated with GPA, receptor (PLA R); autoantibody; placenta; rituximab; immunoglobulin G) subclass. 2 tumor leads G to (Ig rapid correction of electrolyte abnormalities and which requires a high index of suspicion and prompt diagnosis for significant improvement in quality of life. This case demonstrates the appropriate immunosuppressive therapy to prevent further ischemia, importance of carefully investigating the cause of unexpected gangrene and vasculitic involvement. pathologic fractures in the setting of chronic bone pain, as a reversible pathology may be present. regnant patients with autoimmune disease may
P
deliver newborns with a spectrum of clinical manifestations due to the transplacental passage of 226 circulating autoantibodies. Pregnant patients with A RANDOMIZED CONTROLLED TRIAL OF TAUROLIDINE lupus or myasthenia gravis can deliverLOCK babies HEPARIN VERSUS TAUROLIDINE UROKINASE TO with IMPROVE CATHETER FUNCTION corresponding disease in theIN HEMODIALYSIS. neonate.1,2 Neonatal Zafar Mohammad, Abdullah Hamad, Ahmad Hamdy, Prim Chandra, membranous nephropathy (MN) notFadwa associated with Tarek Abdul- latif, Rania Ibrahim, Tarek Fouda, Al-Ali. Fahad Bin Jassim Kidney Center,was Hamad General Hospital, Doha, Qatar. and congenital infection first described in 1990 The use of tunneled catheters in hemodialysis is one of the leading attributed to theandpassive of (BFR) maternal anticauses of morbidity mortality. transfer Blood flow rate is a surrogate 3 outcome a markerrenal of catheter malfunction. We compared bodies to and putative antigens. More than athedecade effects of two catheter solutions (Taurolidine citrate with later, Debiec et (Taurolock/Hep) al4 locking identified the Taurolidine first antigen heparin 500 units versus citrate involved with 25000 units regarding BFR and Kt/v. in Urokinase such cases as (Taurolock/U) neutral endopeptidase (NEP), a This is a prospective randomized controlled trial in which all patients metalloprotease present on the surface of the podocyte who were undergoing ambulatory regular hemodialysis in Qatar with andtunneled involved theincluded. proteolytic regulation of vasoaccatheterinwere All patients were randomized to Taurolock/Hep or Taurolock/U on 1:1 basis using computertivereceive peptides. Debiec et al described a mother with a generated program. Patients were followed for 6 months. BFR in all mutation preventing NEP expression who had formed sessions, monthly Kt/V were monitored. Sub-analysis was done for BFR sessions post Taurolock/U versusalloimmuthe anti-NEP antibodies due(BFR-Taurolock/U) to fetomaternal comparable sessions in Taurolock/Hep group (BFR-Taurolock/Hep). nization from a previous miscarriage; these antibodies 177 participated in the study. 93 Patients in Taurolock/Hep and 84 in the Taurolock/U. catheter age, BFRcause and venous pressures were to cross Age, the sex, placenta and subepithelial before study, use of antiplatelet or warfarin and co-morbidities did not deposits in the fetal kidney of a subsequent differ between the two groups. There was no significant differencepregin mean BFR in all sessions between the twoA groups (273+/-7 ml/min nancy. M-type phospholipase (PLAin2R) 2 receptor versus 281+/-8 ml/min in Taurolock/U) (pValue 0.15). wasTaurolock/Hep later identified as the major autoantigen BFR-Taurolock/U was significantly higher compared to BFR- for pri5 Taurolock/Hep most of the 24 week study period. Monthly was mary MN ininadults. Little literature existsKt/vabout slightly higher in the Taurolock/U but was not statistically significant pregnancy outcomes in patients with nephrotic syn(1.22 versus 1.11) (pValue >0.05). There were no reported serious drome primary MN, withduring nostudy. data available adversedue eventstorelated to treatment reported In apregnancy randomized controlled trial comparing Taurolock/Hep to about in PLA disease. We 2R-associated Taurolock/U catheter lock solution, we found no statistically significant present what we believe to be the first known case difference in overall BFR or monthly Kt/v. Sub analysis showed that of BFR-Taurolock/U higher than BFR-Taurolock/Hep. Although this pregnancy in a was patient with PLA2R-associated MN difference was statistically significant but was not clinically important who(only was seropositive for anti-PLA R autoantibodies 2 13 ml/min difference). throughout the course of her pregnancy. Am J Kidney Dis. 2016;67(5):A1-A118
CASE REPORT
A 39-year-old multiparous woman with morbid obesity presented for workup of severe nephrotic syndrome several months 228
before her current pregnancy. She had been treated for resistant TEARS OF JOY? EXTRAGLANDULAR SJӦGREN SYNDROME hypertension lower-extremity edema during the past year, LEADING TOand CRYOGLOBULIN-ASSOCIATED but her proteinuria had been overlooked. GLOMERULONEPHRITIS AND MYOSITIS At presentation, serum Javier Monserrate, Jennifer Jaynes, Steven Foster, Roberto Collazocreatinine level was 1.52 mg/dL (corresponding to estimated 2 Maldonado.filtration Methodist rate Dallas System, Dallas,mTX, glomerular ofHealth 46 mL/min/1.73 asUSA. calculated by Sjögren’s syndrome is an autoimmune inflammatory disorder thecharacterized isotope-dilution spectrometry –traceable 4-variable by chronic mass inflammation of exocrine glandular tissues, MDRD [Modification of Diet in Renal Disease] mainly the lacrimal and salivary glands. However, almost anyStudy organ equation); serum level, 1.5 urineRenal protein system can bealbumin affected, including theg/dL; kidneysand and24-hour muscle tissue. involvement usually presents as tubulointerstitial nephritis or excretion, 29.2 g. The kidney biopsy specimen revealed features glomerulonephritis. this report present a case of a 59 y/o African typical of primaryInMN with we additional strong staining for the American man with a past medical history of Chronic Kidney Disease PLA R antigen within immune deposits (Fig S1). 2 (CKD) Stage III, Hepatitis C (HCV), Hepatitis B (HBV), andMany of the subepithelial deposits were completely surrounded by new Hypertension who presented with severe myalgia and Acute Kidney Injury (AKI) Stage III, without sicca symptoms. On admission, basement membrane material (Fig S2), and 35% of the laboratories showed a creatinine was 3.4 mg/dl (baseline of 1.5 mg/dl), active urinary sediment with hematuria (RBCs 10-25/HPF) and subnephrotic proteinuria. Creatine phosphokinase (CPK) levels were 1 From theSerologic Department of Medicine, Renal Type Section, and De1149 U/L. workup was positive for elevated 2 2 3 Cryoglobulins, undetectable and C4 levels, C3 levels of 77 mg/dl, along partments of Obstetrics Gynecology, Pediatrics, and 4Pawith elevated of SS-A Ro 60 kDa antibodies RF of 111.9Medical thology and titers Laboratory Medicine, Bostonand University IU/ml. HCV and HBV titers were non-detectable. The kidney biopsy Center, Boston, MA. revealed mesangial hypercellularity with increased matrix material; * Current affiliation: of Internal Immunofluorescence wasDepartment positive for IgG, IgM, C3q,Medicine, Lambda andDivision ofkappa Nephrology, of Utah of granular Medicine, Salt Lake staining inUniversity a global peripheral andSchool mesangial pattern. There was focal positive staining within the tubules for IgA, kappa and City, UT. y lambda. Electron microscope evaluationof revealed podocyte Current affiliation: Department Obstetrics andeffacement, Gynecology, mesangial deposits, along with mesangial interposition and prominent Medstar Washington Hospital Center, Washington, DC. subendothelial electron dense deposits. The diagnosis of myositis with Received June 29, 2015. Accepted in revised form October 27, Cryoglobulin-associated membranoproliferative glomerulonephritis 2015. Originally online December 29,The 2015. (MPGN) secondarypublished to Sjögren’s syndrome was made. patient was aggressively treated with systemic plasmapheresis andMD, PhD, Address correspondence to steroids, Laurence H. Beck Jr, rituximab, which resulted650 in normalization of CPK levels Renal Section, X-504, Albany St, Boston, MA and 02118. E-mail: resolution of the AKI. It is therefore important for the nephrologist to [email protected] remain aware that myositis and cryoglobulin-associated MPGN can be � 2016 by the National Kidney Foundation, Inc. initial manifestations of Sjogren syndrome.
0272-6386 http://dx.doi.org/10.1053/j.ajkd.2015.10.031
775 A75