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Abstract

J.P. Garvey, C.M. Brown, S.H. Chotirmall, A.M. Dorman, P.J. Conlon and J.J. Walshe

Departments of Nephrology, Transplantation and Pathology, Beaumont Hospital, Dublin, Ireland
Background: Acute interstitial nephritis (AIN) secondary to trimethoprim-sulfamethoxazole (TMP-SMX) is well documented as a cause of acute renal failure in native kidneys. TMP-SMX is the standard prophylactic agent against pneumocystis carinii (PCP) used in the early post-transplant period, however, it has to date only been indirectly associated with AIN in renal allografts. Design, setting, participants and measurements: We describe eleven renal transplant patients with acute allograft dysfunction in whom a transplant biopsy demonstrated primary histopathologic features of allergic AIN, all of whom were receiving TMP-SMX in addition to other medications known to cause AIN. Results: All cases occurred within 1 month of transplantation and accounted for 2.12% (11/518) of the total number of transplant biopsies performed during the study period. However, this figure increased to 10.1% (11/109) when those biopsies performed for early allograft dysfunction (< 1 month) were taken into account. After discontinuation of TMP- SMX alone, all patients had an immediate improvement in serum creatinine with excellent long term allograft function – mean improvement of serum creatinine from 465 µmol/l to 136 µmol/l at last follow-up (range 15 – 55 months). Conclusions: AIN secondary to TMP-SMX, although an uncommon cause of allograft dysfunction over the study period, accounted for over 10% of cases of allograft dysfunction within the first month of transplantation. Therefore, a high degree of clinical suspicion for TMP-SMX-induced AIN is warranted when confronted with early acute allograft dysfunction.Correspondence to:
Prof. J.J. Walshe
Dept. of Nephrology and Transplantation
Beaumont Hospital,
Dublin 9, Ireland
Email: josephwalshe@beaumont.ie

Abstract

W.M. Michels¹,², D.C. Grootendorst², K. Rozemeijer², F.W. Dekker² and R.T. Krediet¹

¹Division of Nephrology, Department of Medicine, Academic Medical Center, University of Amsterdam, and ²Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands
Background: Measuring GFR using exogenous markers without a bladder catheter, errors can be easily made due to incomplete urine collection. The aim was to quantify agreement for ¹²⁵I-iothalamate GFR measurements with and without a correction for inaccurate urine collection using ¹³¹I-hippuran. Methods: The last available GFR measurement of adult patients was included. GFR was measured in two subsequent clearance periods with ¹²⁵I-iothalamate by the standard method with correction for inaccurate urine collections using ¹³¹I-hippuran. The uncorrected and corrected GFR measurements were compared within and between the time periods for each individual patient. To study the agreement between both methods, intraclass correlation coefficients (ICC) were calculated and Bland-Altman plots, with accompanying accuracies and precisions, were used. Cohen’s kappa was calculated to analyze the agreement of both methods for classifying patients according to the stages of chronic kidney disease (CKD). Results: For the 332 stable included patients, the mean GFR of the uncorrected measurements was 77.8 ml/min (34.7) and the mean GFR of the corrected measurements was 81.0 ml/min (34.9). The ICC was 0.80 for the uncorrected measurements with an accuracy of 7.3 ml/min and a precision of 21.7 ml/min. For the corrected GFR measurements the ICC was 0.98, with an accuracy of 2.1 ml/min and a precision of 6.5 ml/min. Comparison between the methods showed an ICC of 0.95, an accuracy of 3.2 and a precision of 11.0. In total, 86% of the patients were classified similarly into CKD stages with both methods, overall Cohen’s kappa was 0.81. Conclusion: Agreement was better for GFR measurements corrected for inaccurate urine collections. Therefore, GFR measurements with ¹²⁵I-iothalamate should be corrected for inaccurate urine collections using ¹³¹I-hippuran. Without such correction the GFR is easily underestimated which may lead to overtreatment.Correspondence to:
W.M. Michels, MD
Academic Medical Center, Division of Nephrology
PO BOX 22660,
1100 DD Amsterdam, The Netherlands
Email: w.m.michels@amc.uva.nl

Abstract

D.-C. Fischer¹, R. Nissel¹, A. Puhlmann¹, A. Mitzner², M. Tieß³, R. Schmidt² and D. Haffner¹

¹Department of Pediatrics, University Children’s Hospital Rostock, ²Department of Nephrology, University of Rostock, and ³Health Care Center, Dialysis Association North, Rostock, Germany
Chronic kidney disease (CKD) is highly associated with an increased cardiovascular morbidity and mortality and is also a state of growth hormone (GH) resistance. We examined the impact of a short-term treatment with rhGH on circulating markers of cardiovascular risk in nonmalnourished CKD patients in a single-center, nonrandomized pilot study. Patients with stable CKD Stage 3 – 5 and age- and sex-matched healthy controls (n = 15 each) received a 7-day treatment with rhGH (1.33 mg/m² body surface area per day, approximately 30 µg/kg). Prior to onset of rhGH therapy, at the end of the treatment period and at the end of a 7-day wash-out period blood was drawn to assess changes in circulating markers of cardiovascular risk. At time of enrolment CKD patients showed elevated serum concentrations of phosphate, calcium × phosphate product, PTH, fibroblast growth factor-23 (FGF-23), triglycerides, leptin and homocysteine compared to controls. In patients and controls rhGH treatment induced an increase in circulating insulin-like growth factor I (IGF-I), and the molar ratio of IGF-I/IGF binding protein 3 as well as an elevation of glucose, insulin, and triglycerides, whereas serum urea was decreased. In CKD patients, rhGH treatment raised concentrations of leptin, whereas LDL-cholesterol, homocysteine, phosphate, and 25-hydroxyvitamin D were significantly reduced. In controls, but not in CKD patients, rhGH raised 1,25-dihydroxy-vitamin D₃ serum levels, which were even more elevated at the end of the wash-out period. In conclusion, short-term treatment with rhGH in CKD patients affects not only insulin and glucose metabolism but also affects serum lipid profile, i.e., LDL-cholesterol, leptin and homocysteine. Long-term trials are required to evaluate the impact of rhGH on cardiovascular morbidity and mortality.Correspondence to:
D.-C. Fischer, PhD
Department of Pediatrics
University Children’s Hospital
Ernst-Heydemann-Straße 8
18057 Rostock, Germany
Email: dagmar-christiane.fischer@med.uni-rostock.de

Abstract

S. Choi, D. Lee, K.-H. Jeong, J.-Y. Moon, S.H. Lee, T.-W. Lee and C.-G. Ihm

Department of Nephrology, College of Medicine, Kyung-Hee University, Seoul, South Korea
Background: The prognostic relevance of clinical and histological features on renal outcome has not been assessed in patients with IgA nephropathy (IgAN) treated with the combination therapy of steroid and angiotensin receptor blockers (ARB). Methods: A prospective trial of a combination of steroid and ARB was performed in 50 patients with IgAN, proteinuria and serum creatinine levels < 2 mg/dl. Results: Over a mean follow-up period of 4 years, the combination therapy reduced proteinuria and hematuria and improved renal function in most patients. The mean change in estimated GFR (eGFR) was + 0.30 ± 0.74 ml/min/1.73 m2/month. Forty-three patients (86%) exhibited stable renal function and 7 patients (14%) reached the primary end point of a ³ 20% decrease in eGFR from baseline levels. Between the nonprogressive and progressive patients, there were significant differences in the levels of urine protein/ creatinine excretion ratio (PCR) at baseline and throughout the follow-up period as well as baseline eGFR and degree of glomerular crescents (p < 0.05). Forty (80%) and 24 patients (48%) had a urine PCR < 1 and < 0.3 g/g, respectively, at their last follow-up. Renal survival was better in patients who had initial urine PCR < 3 g/g as well as final PCR < 1 g/g. Regression analysis revealed that the final urine PCR and age were critical determinants of slope of the eGFR by both univariate and multivariate analyses. However, eGFR, pathologic findings, systolic BP, proteinuria, and body mass index at the initial presentation were not predictive of slope. Conclusion: Our results indicate that achieving a low urinary protein excretion is the main determinant for the good outcome in patients treated with combination therapy.Correspondence to:
C.-G. Ihm, MD
Kyung-hee Univ. Medical Center
Hoegi-dong, Dongdaemun-gu
Seoul, 130-702, Korea
Email: cgihm@yahoo.co.kr

Abstract

B.J. Lim, S.W. Hong and H.J. Jeong

Department of Pathology, Yonsei University College of Medicine, Seoul, Korea
Background: IgG nephropathy is one of the most recently described glomerulopathies, which frequently overlaps with C1q nephropathy. To clarify this entity, we evaluated renal biopsy cases with IgG deposits as a sole or predominant immunoglobulin. Methods: Fourteen cases demonstrating IgG as a predominantly deposited immunoglobulin in patients without infectious or autoimmune diseases between 1997 and 2008 were studied. Twelve patients had glomerular disease in the native kidney and the other 2 were renal allograft recipients. Results: Clinical presentation was microscopic hematuria, proteinuria, or both and nephrotic syndrome was observed in 3 patients. Segmental glomerulosclerosis was observed in 4 patients and mesangial hypercellularity was present in 7. Tubulointerstitial changes were not evident except for allograft biopsies. On immunofluorescence, mesangial or capillary wall IgG deposits were present in all cases, and C1q was observed in 11 cases in a similar pattern with IgG, co-dominant in 5 cases and dominant in 1. Conclusions: Since a significant overlap is frequently observed in these two rare conditions, we suggest a tentative diagnosis of IgG/C1q nephropathy in such cases.Correspondence to:
H.J. Jeong, MD
Department of Pathology, Yonsei University
College of Medicine
134 Shinchon-dong, Seodaemun-gu
Seoul 120-752, Korea
Email: jeong10@yuhs.ac

Abstract

R. Dikow¹, P. Quentmeier¹, V. Schwenger¹, R. Waldherr², K. Andrassy¹, E. Ritz¹ and M. Zeier¹

¹Department of Nephrology and ²Department of Pathology, University Hospital of Heidelberg, Heidelberg, Germany
The treatment of idiopathic membranous nephropathy (MN) with nephrotic syndrome comprises immunosuppressive therapy and antihypertensive treatment with the blockade of the renin-angiotensin system (RAS). Given the relatively benign natural history of MN, an immunosuppressive-free therapeutic regimen should be considered as the primary treatment option. In a single-center, retrospective analysis we compared the outcome of 54 patients with biopsy-proven idiopathic MN 12, 24 and 60 months after initiation of therapy. All patients had RAS-blocking agents and 36 patients received additionally an immunosuppressive regimen. In both groups the patients initially had a nephrotic proteinuria (median 8.7 vs. 6.0 g/day, n.s.). Median blood pressure reduction was comparable after 12, 24 and 60 months in both groups. The median evolution of proteinuria during therapy after 12, 24 and 60 months was 3.4, 1.7 and 1.1 g/day in the group with immunosuppression compared to 3.0, 1.1 and 0.32 g/day in the non-immunosuppressive group. After 60 months no patient developed endstage renal failure. The number of severe side effects was significantly higher in patients with immunosuppression. Regarding renal function and reduction of proteinuria, patients with idiopathic MN treated without immunosuppressive therapy but with measures to ensure optimal blood pressure control and the full blockade of RAS had a similar outcome after 60 months as compared to patients who received additional immunosuppressive therapy.Correspondence to:
Dr. R. Dikow
Nierenzentrum Heidelberg
Im Neuenheimer Feld 162
69120 Heidelberg, Germany
Email: ralfdikow@yahoo.de

Abstract

J.Y Xie, N. Chen, H. Ren, X.M. Huang and P. Zhu

Department of Nephrology, Shanghai Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, P.R. China
Aim: To compare the difference of clinical curative effects of continuous ambulant peritoneal dialysis (CAPD) patients in China by using a two-cuff Swan neck catheter and a Tenckhoff catheter. Methods: 110 patients with end-stage renal disease (ESRD) were enrolled. They were divided into Group A (Swan neck catheter group, n = 55) and Group B (Tenckhoff catheter group, n = 55). One-year follow-up visits were made and information was recorded. Survival analysis was made by adopting the Kaplan-Meier method. Results: After 12 month follow-up visits, 17 patients had died, 3 had been transferred to renal transplantation, 8 had been transferred to hemodialysis, 3 were transferred to other hospitals, and the remaining 79 patients (71.8%) continued their peritoneal dialysis therapy in our hospital. 26 patients in both groups had peritonitis, with a total of 35 occurrences taking place. The total incidence of peritonitis was 0.32 times/patient year, with the detailed figure of 0.35 times/patient year for Group A and 0.29 times/patient year for Group B respectively (p > 0.05). Regarding mechanical complications of the 2 groups concerned, including catheter tip migration, Omental enwrapment, peritoneal dialysate leakage, skid of outer cuff, incidence of inguinal hernia and bellyache, etc, no significant difference existed between two groups (p > 0.05). The two groups had the same 12-month technical survival rate of 92.73%. The 12-month survival rate for Group A was 86.34% while the corresponding figure for Group B was 80.68% (p > 0.05). Conclusions: Infections, mechanical complications, technical survival rate and patients’ survival rate were quite similar, when a Swan neck catheter and a Tenckhoff catheter were used in Chinese CAPD patients.Correspondence to:
N. Chen, MD
200032, Shanghai, P.R. China
Email: cnrj100@126.com

Abstract

M. Haag-Weber¹, A. Vetter² and U. Thyroff-Friesinger² for the INJ-⁹ Study Group

¹Kuratorium für Dialyse und Nierentransplantation e.V., Straubing, and ²Sandoz AG/Hexal AG, Holzkirchen, Germany
Background: The recombinant human epoetin-a HX575 (Sandoz Pharmaceuticals GmbH/Hexal AG, Holzkirchen, Germany) is the first biosimilar erythropoiesis-stimulating agent (ESA) with marketing authorization in Europe. The primary objective of the study was the evaluation of therapeutic equivalence in terms of hemoglobin (Hb) response of HX575 compared with the comparator product (EPREX^®/ ERYPO^®, Janssen-Cilag/Ortho Biotech, Neuss, Germany) in the long-term intravenous (i.v.) treatment of anemia in chronic renal failure patients on hemodialysis following a 1 : 1 dose conversion from the comparator product to HX575. Methods: Hemodialysis patients with Hb levels of 10.0 – 13.0 g/dl were randomized to either continue their current i.v. epoetin-a treatment or switch to HX575. During treatment, epoetin dosages were titrated to maintain Hb values. The primary endpoint was the difference between treatment groups in the mean absolute change of Hb levels between baseline and evaluation period (Weeks 25 – 28). Results: Therapeutic equivalence of HX575 and the comparator epoetin-alpha, assessed during the evaluation period, was statistically confirmed: mean changes in Hb levels were 0.15 ± 0.09 g/dl in the HX575 and 0.06 ± 0.12 g/dl in the comparator epoetin-a group, with a difference between groups of 0.08 g/dl (95% confidence interval: –0.17; 0.34). Hb levels and epoetin dosages remained stable throughout the entire study period of 56 weeks. The long-term safety profile of HX575 was similar to that of the comparator epoetin-alpha. No antibody formation was detected. Conclusions: The study demonstrated therapeutic equivalence of biosimilar HX575 to the comparator epoetin-a, together with a comparable safety profile.Correspondence to:
Prof. Dr. M. Haag-Weber
Kuratorium für Dialyse und Nierentransplantation e.V.
Elisabethstraße 23
94315 Straubing, Germany
Email: marianne.haag-weber@kfh-dialyse.de

Abstract

T. Horino¹, M. Okazaki¹, H. Nishikawa², T. Takao³, Y. Taniguchi¹, T. Morita¹ and Y. Terada¹

¹Department of Endocrinology, Metabolism and Nephrology, ²Department of Urology, and ³Division of Community Medicine, Department of Community Nursing, Kochi Medical School, Kohasu, Okoh-cho, Nankoku, Japan
A 77-year-old female with abdominal pain and ascites was admitted to our hospital. She had a past history of the postoperative pelvic irradiation for uterine cancer and subsequently suffered from neurogenic bladder. On admission, serum creatinine (s-Cr) and blood urea nitrogen (BUN) were elevated to 9.9 mg/dl and 131 mg/dl, respectively. However, both the ratio of BUN/s-Cr and creatinine in ascites/s-Cr were significantly elevated. The clinical manifestations of the present case were not typical for acute kidney injury. Furthermore, 2 days after urethral catheterization, both s-Cr and BUN were normalized (0.69 mg/dl and 10 mg/dl, respectively) and her symptoms had improved immediately. Therefore, we diagnosed her disease as spontaneous bladder rupture. We report a case with spontaneous bladder rupture mimicking acute kidney injury forty years after postoperative pelvic irradiation for uterine cancer.Correspondence to:
T. Horino MD, PhD
Department of Endocrinology, Metabolism and Nephrology
Kochi Medical School
Kohasu, Okoh-cho
Nankoku, Kochi 783-8505, Japan
Email: horinott@yahoo.co.jp

Abstract

S.-C. Chen¹,², J.-M. Chang¹,²,⁴, C.-S. Wang¹,³, C.-H. Kuo¹,⁴ and H.-C. Chen²,⁴

¹Department of Internal Medicine, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung Medical University, ²Division of Nephrology, ³Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kaohsiung Medical University Hospital, and ⁴Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
Intraperitoneal bladder rupture is a rare cause of acute abdomen with bloody ascites. We report herein the case of a patient who had alcoholic liver cirrhosis and multiple liver nodules, and experienced acute bloody ascites and oliguric acute renal failure in association with intraperitoneal bladder rupture. A 33-year-old male suffered from acute abdominal pain and oliguria following consumption of a large amount of alcohol and after blunt abdominal trauma. He was also found to have acute renal failure and newly onset bloody ascites that rapidly subsided following transurethral catheter drainage. Computed tomography cystography revealed intraperitoneal extravasation of contrast from the dome of the bladder, suggestive of intraperitoneal bladder rupture. The patient received surgical repair and was discharged with full recovery. This case shows that it is important for physicians to be aware of the possibility of intraperitoneal bladder rupture after alcohol consumption accompanied with abdominal blunt trauma. In particular, it has diagnostic complications for underlying liver tumors.Correspondence to:
C.-H. Kuo, MD
Department of Internal Medicine
Kaohsiung Municipal Hsiao-Kang Hospital
Kaohsiung Medical University
482, Shan-Ming Rd., Hsiao-Kang Dist.
812 Kaohsiung, Taiwan, R.O.C.
Email: 0840049@kmhk.kmu.edu.tw

Abstract

J. Gerth¹, M. Busch¹, U. Ott¹, H.-J. Groene² and G. Wolf¹

¹Department of Internal Medicine III of the Friedrich-Schiller-University Jena,
Jena and ²Department of Cellular and Molecular Pathology, German Cancer Research Center, Heidelberg, Germany
A 44-year-old female was diagnosed with proteinuria due to nodular glomerulosclerosis secondary to light chain deposition disease (LCDD). After 6 years, deterioration of kidney function occurred and autologous stem cell transplantation was considered, but the patient refused specific therapies. The disease progressed slowly, over a period of 8 years reaching now chronic renal insufficiency stage 4 with a creatinine clearance of 20 ml/min, in spite of no specific therapy. This case, documented by repeated biopsies, demonstrates the very slow loss of kidney function, suggesting the possibility of conservative treatment strategies without taking the risks of chemotherapy or autologous stem cell transplantation, since no long term follow up data of these therapies are available for LCDD.Correspondence to:
Prof. Dr. G. Wolf
Department of Internal Medicine III
University of Jena
Erlanger Allee 101
07347 Jena, Germany
Email: Gunter.Wolf@med.uni-jena.de

Abstract

S. Lovric, J.U. Becker, D. Kayser, A. Wagner, M. Haubitz and J.T. Kielstein

Department of Nephrology, Hannover Medical School, Hannover, Germany
While newly developed potent immunosuppressive agents have dramatically reduced the incidence of rejection of transplanted organs, they have increased the patients’ susceptibility to opportunistic infections and cancer. Here we report a rare skin infection caused by atypical mycobacterium marinum in a 50-year-old female renal transplant recipient. The patient presented with localized skin lesion on the dorsum of her hand, which was misdiagnosed as gout. Only after the lesions spread in a sporotrichoid pattern, a cutaneous infection with atypical mycobacteria was suspected. The diagnosis was based on histopathological analysis as well as mycobacterial culture, both showing infection with atypical mycobacterium. Three months of antimycobacterial treatment led to a marked regression of the lesions. Sporotrichoid lesions in renal transplant patients are rare and a diagnostic challenge for the physician. A thorough history and a low threshold for skin biopsies could prevent painful and unnecessary surgical interventions.Correspondence to:
PD Dr. J.T. Kielstein
Department of Internal Medicine
Medical School Hannover
Carl-Neuberg-Straße 1
30625 Hannover, Germany
Email: Kielstein@yahoo.com

Abstract

T. Malek-Marín¹, M.D. Arenas¹, M. Perdiguero², M. Salavert-Lleti³, A. Moledous¹, E. Cotilla² and M.T. Gil¹

¹Departments of Nephrology of Hospital Perpetuo Socorro, ²Hospital General of Alicante, and ³Department of Infectious Diseases of Hospital La Fe of Valencia, Spain
We report a case of A. xylosoxidans endocarditis of larvate clinical presentation in a central venous catheter-dependent hemodialysis patient of difficult diagnosis and poor evolution despite a high index of suspicion and consequent assessment. A 50-year-old man on hemodialysis presented with inflammatory-malnutrition parameters during the months prior to diagnosis of endocarditis, whilst he was otherwise asymptomatic. No vegetations were detectable on his cardiac valves at repeated echocardiography until third transesophageal echocardiography was performed, and confirmed intraoperatively. On the occasion of positive peripheral blood culture for Alcaligenes (Achromobacter) xylosoxidans, the etiological diagnosis was retrospectively explained given his history of animal exposure – hunting rabbits using his pet ferrets. This bacterium is an emergent and resistant organism, mostly related to nosocomial infections and environmental water sources. Reservoirs include the microflora of the nasal cavity of rabbits and the dentogingival sulcus of ferrets. We presume that A. xylosoxidans endocarditis was transmitted from the patient’s pets. It highlights the importance of early recognition of an inflammatory status and investigation of the underlying cause. Additionally, the causative bacterium emphasizes the importance of registering exposure to animals in CVC-dependent patients as well as the need for their awareness of hygienic precautions and the infectious risk associated to catheters.Correspondence to:
T. Malek-Marín
Nephrology Department
Hospital Perpetuo Socorro
Plaza Dr. Gómez Ulla 15
Apartado de Correos No. 240
03013 Alicante, Spain
Email: tamaramalek@gmail.com

Abstract

D.J. Hobbs¹, G.-M. Barletta¹, J.Y. Chung¹,² and T.E. Bunchman¹

¹Pediatric Nephrology, Dialysis and Transplantation, Helen DeVos Children’s Hospital and Michigan State University College of Human Medicine, Grand Rapids, MI, and ²MSU/GRMERC Pediatric Nephrology Fellowship Program, Grand Rapids Medical Education and Research Center (GRMERC), Grand Rapids, MI, USA
Sarcoidosis is a multisystem disease of unknown etiology primarily affecting the lungs, skin, and lymph nodes. The disease usually manifests in young adults and is uncommon in childhood. Renal involvement, including granulomatous interstitial nephritis (GIN), is rare, and few cases of isolated sarcoid GIN have been reported in pediatrics. We report a case and review the literature.Correspondence to:
D.J. Hobbs, MBSc
Pediatric Nephrology, Dialysis & Transplantation
Helen DeVos Children’s Hospital
221 Michigan St NE, Suite 406
Grand Rapids, MI 49503, USA
Email: hobbsdav@msu.edu

Abstract

S. Chacko¹, H. Taskapan², J. Roscoe³, J. Stein⁴, E. Woods⁵, T. Denton⁶, R. Ting²,³, P. Tam²,³, D.G. Oreopoulos⁷, M. Rodriguez-Justo⁸ and T. Sikaneta²,³

¹Department of Family Medicine, University of Toronto, Toronto, ²Nephrology Associates, Scarborough, Ontario, ³Departments of Nephrology and Medicine, Scarborough General Hospital, ⁴Departments of Rheumatology and Medicine, ⁵Department of Urology, ⁶Diagnostic Imaging, Humber River Regional Hospital, ⁷Departments of Nephrology and Medicine, University Health Network, Toronto, Ontario, Canada, and ⁸Department of Histopathology, Royal Free and University College Medical School, University College Hospital, London, UK
We report a patient with multifocal fibrosclerosis presenting as sialadenitis, hepatic fibrosis, and retroperitoneal fibrosis with renal failure. His medical management consisted of prednisone (4 months at 40 mg daily, then tapered down to 5 mg daily for another 14 months) and 18 months of tamoxifen. He responded clinically and radiographically to this regimen, and remains in clinical remission 10 months after discontinuing medical therapy. Subsequent histologic examination of submandibular gland tissue revealed strong staining for IgG₄-positive plasma cells. To our knowledge, this is the first case of confirmed multifocal hyper-IgG₄ disease to be successfully treated with sequential corticosteroids and tamoxifen.Correspondence to:
Dr. T. Sikaneta
Nephrology Associates
Scarborough, Ontario, M1H 3G4, Canada
Email: tabosikaneta@yahoo.ca

Abstract

T.H. Lee, W. Kim, S. Lee, K.P. Kang, Y.K. Kim, M.K. Kim, Y.B. Jeong, H.S. Park, N.H. Lee and S.K. Park

Abstract

E.M. Atasoyu, O. Bilgi, C. Kinalp, B. Karagoz, A. Haholu, S. Unver and T.R. Evrenkaya

Abstract

M. Huijssoon, K. Ipema and C.F.M. Franssen

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Volume 72, No. 5/2009(November)