DOCSLIB.ORG

Pharmacological Therapy of Lupus Nephritis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Pharmacological Therapy of Lupus Nephritis Downloaded from www.jama.com at Johns Hopkins University, on November 23, 2005 GRAND ROUNDS CLINICIAN’S CORNER AT THE JOHNS HOPKINS BAYVIEW MEDICAL CENTER Pharmacological Therapy of Lupus Nephritis Derek M. Fine, MD Kidney involvement is common in systemic lupus erythematosus, occurring CASE PRESENTATION in up to 60% of affected adults during the course of their disease. Diffuse Mrs P, a 31-year-old woman, developed proliferative lupus nephritis (World Health Organization class IV), the most a blood pressure of 170/104 mm Hg at ominous variant, has traditionally been treated with cyclophosphamide and 38 weeks of pregnancy. Her obstetri- glucocorticoids. With cyclophosphamide, women of childbearing potential cian performed a laboratory evaluation must weigh the risks of sustained amenorrhea, infertility, increased suscep- ϩ that revealed proteinuria (2 ), throm- tibility to infection, bone marrow suppression, hemorrhagic cystitis, and ma- bocytopenia (platelet count, 121ϫ103/µL lignancy against the benefits of better disease control compared with glu- [normal range: 150-350ϫ103/µL]), a serum creatinine level of 0.8 mg/dL (70.7 cocorticoids alone. Because of the host of adverse effects associated with µmol/L) (normal range: 0.4-1.1 mg/dL cyclophosphamide, alternative approaches to the treatment of lupus nephri- [35.3-97.2 µmol/L]), uric acid level of 9.0 tis are desirable. A 31-year-old woman developed class IV lupus nephritis mg/dL (normal range: 2.4-5.7 mg/dL), in the postpartum period. Seeking to preserve fertility and avoid other known and aspartate and alanine aminotrans- toxicities of cyclophosphamide, she chose to undergo therapy with myco- ferase levels of 170 U/L and 190 U/L, phenolate mofetil. In the treatment of severe lupus nephritis, mycopheno- respectively (normal range: 0-35 U/L). late mofetil has emerged as an alternative to cyclophosphamide, offering a Mrs P was diagnosed with the HELLP major advance in the therapy of lupus nephritis. (hemolysis, elevated liver enzymes, low platelets) syndrome1 and underwent an JAMA. 2005;293:3053-3060 www.jama.com emergent cesarean delivery of a healthy male newborn. Despite this interven- quent 2 months. Her transient conges- At the time of Mrs P’s nephrology tion, Mrs P continued to have thrombo- tive heart failure was attributed to post- evaluation, her medications included cytopenia and hypertension. In addi- partum cardiomyopathy. Five months candesartan (32 mg/d) and metopro- tion, she developed temperatures up to after delivery, she presented with fe- lol succinate (150 mg/d). The family 102° F. She was treated empirically with ver, fatigue, arthralgias, malar rash, a medical history was noncontributory. antibiotics and received heparin briefly papular eruption on her forearms, and She had previously worked as a neo- for a presumptive diagnosis of pelvic alopecia. Her platelet count was still low natal nurse and did not smoke or drink. ϫ 3 thrombophlebitis. Fevers continued and (69 10 /µL) and she remained ane- Her child was now 6 months old; she she developed weakness and fatigue. mic (hematocrit, 26% [normal range: and her husband were eager to have Four months after delivery, she de- 38%-47%]). These findings prompted more children. veloped progressive dyspnea on exer- a serological workup that revealed high On physical examination the pa- tion and orthopnea. Echocardiogra- titer positive assay for antinuclear an- tient was afebrile with a blood pres- phy revealed an ejection fraction of tibodies (ANAs) and antibodies to sure of 132/80 mm Hg, a resting pulse 30%. Therapy was initiated with an an- double-stranded DNA (dsDNA). Her se- Author Affiliation: Division of Nephrology, Depart- giotensin-converting enzyme inhibi- rum complement levels were low, with ment of Medicine, Johns Hopkins University School tor and a loop diuretic, with resolu- the C3 and C4 complements measur- of Medicine, Baltimore, Md. ing 43 mg/dL (normal range: 79-152 Corresponding Author: Derek M. Fine, MD, Division tion of her symptoms and normalization of Nephrology, Department of Medicine, Johns Hop- of her cardiac function over the subse- mg/dL) and 5 mg/dL (normal range: kins University School of Medicine, 1830 E Monu- 12-42 mg/dL), respectively. Her eryth- ment St, Suite 416, Baltimore, MD 21205 (dfine1 @jhmi.edu). See also Patient Page. rocyte sedimentation rate was el- Grand Rounds at The Johns Hopkins Bayview Medi- evated at 107 mm/h (normal range: 4-25 cal Center Section Editors: John H. Stone, MD, MPH, CME available online at mm/h). The diagnosis of systemic lu- Charles Weiner, MD, Stephen D. Sisson, MD, The Johns www.jama.com Hopkins Hospital, Baltimore, Md; David S. Cooper, MD, pus erythematosus (SLE) was made. Contributing Editor, JAMA. ©2005 American Medical Association. All rights reserved. (Reprinted) JAMA, June 22/29, 2005—Vol 293, No. 24 3053 Downloaded from www.jama.com at Johns Hopkins University, on November 23, 2005 PHARMACOLOGICAL THERAPY OF LUPUS NEPHRITIS of 80/min, and a respiratory rate of plications. Most of the 22 glomeruli in mofetil in lupus nephritis, Mrs P chose 12/min. She had thinning hair and a ma- the sample showed diffuse mesangial ex- to undergo therapy with mycopheno- lar rash, but her skin was otherwise pansion and proliferative changes, with late mofetil. clear. Her cardiac examination re- mesangial and endocapillary prolifera- vealed a regular rate and rhythm, nor- tion. There were segmental necrotizing DISCUSSION mal S1 and S2, and no murmurs. Her lesions in 4 of the glomeruli and fibro- Mrs P’s presentation is instructive be- lungs were clear to auscultation. There cellular crescents in 2, indicating signifi- cause it highlights the fact that SLE can was no organomegaly, and she had no cant activity (representative glomeruli develop during or after pregnancy and clubbing, cyanosis, edema, or arthritis shown in FIGURE 1). In addition, im- that it can mimic preeclampsia and the in her extremities. The neurological ex- munofluorescence revealed a “full HELLP syndrome. Her case also dem- amination was normal. Additional labo- house” of immunoreactants, with onstrates how to approach difficult ratory results were notable for a se- granular staining for IgG, IgA, C3, C1q, therapeutic issues by involving the pa- rum creatinine level of 1.1 mg/dL (97.2 and ␬ and ␭ light chains. The biopsy tient in the decision-making process. In µmol/L) with a urine protein to creati- was consistent with diffuse prolifera- Mrs P’s case, the particular concern re- nine ratio of 2.5 mg protein/mg creati- tive (World Health Organization lated to the potential for both short- and nine (proteinuria approximately 2.5 [WHO] class IV) glomerulonephritis. long-term adverse effects of cyclophos- g/d). Microscopic examination of her A meeting including the patient, her phamide, particularly those related to urine showed 10 to 15 red blood cells/ husband, the rheumatologist, and the fertility. The choice was between a stan- high power field, but no red cell casts. nephrologists was convened to dis- dard medication associated with sub- Further serological workup revealed an- cuss treatment options. Although cy- stantial risks of toxicity and a newer tibodies to Ro and Sm. clophosphamide was considered the agent with a limited track record. Her A kidney biopsy was performed to de- standard of care in this setting, the pa- case illustrates emerging data that termine the histological nature and se- tient and her husband were con- strongly support mycophenolate mofetil verity of her renal process. However, cerned about the potential effects of that as an alternative to cyclophosphamide given that her platelet count remained medication on fertility, the risk of ma- in the treatment of lupus nephritis. low, biopsy was delayed until after a lignancy, and the possible infectious The diagnosis of SLE was suspected methylprednisolone pulse, 1 g/d for 3 consequences of severe immunosup- in this patient when she developed a days, followed by 60 mg/d of predni- pression. In light of these concerns, malar rash, alopecia, arthralgias, and sone. The methylprednisolone led to a treatment with mycophenolate mofetil worsening fatigue, accompanied by platelet count increase to greater than was considered. Presented with a bal- thrombocytopenia and hemolytic ane- 100ϫ103/µL within 5 days, and the kid- anced discussion of the data now avail- mia. The diagnosis was confirmed by ney biopsy was undertaken without com- able on the use of mycophenolate positive assays for ANAs, antibodies to Figure 1. Light Microscopic Findings of Representative Glomeruli A B Fibrocellular Crescent A, Hyperlobulated glomerulus with global involvement with endocapillary and mesangial hypercellularity with matrix expansion (white arrowheads) and wireloop le- sions (black arrowheads). B, Glomerulus with global endocapillary proliferation, leukocyte influx, mesangial expansion, and crescent formation (hematoxylin-eosin stain; original magnification x 400). 3054 JAMA, June 22/29, 2005—Vol 293, No. 24 (Reprinted) ©2005 American Medical Association. All rights reserved. Downloaded from www.jama.com at Johns Hopkins University, on November 23, 2005 PHARMACOLOGICAL THERAPY OF LUPUS NEPHRITIS double-stranded DNA (dsDNA), and Table 1. Classification and Treatment of the Different Forms of Lupus Nephritis the findings of her renal biopsy. Her WHO Immunosuppression presentation was confusing because Class* Description Recommendations† both preeclampsia and the HELLP syn- I Minimal mesangial lupus nephritis No specific therapy drome can
Load more

Recommended publications
  • The Case for Lupus Nephritis
    Journal of Clinical Medicine Review Expanding the Role of Complement Therapies: The Case for Lupus Nephritis Nicholas L. Li * , Daniel J. Birmingham and Brad H. Rovin Department of Internal Medicine, Division of Nephrology, The Ohio State University, Columbus, OH 43210, USA; [email protected] (D.J.B.); [email protected] (B.H.R.) * Correspondence: [email protected]; Tel.: +1-614-293-4997; Fax: +1-614-293-3073 Abstract: The complement system is an innate immune surveillance network that provides defense against microorganisms and clearance of immune complexes and cellular debris and bridges innate and adaptive immunity. In the context of autoimmune disease, activation and dysregulation of complement can lead to uncontrolled inflammation and organ damage, especially to the kidney. Systemic lupus erythematosus (SLE) is characterized by loss of tolerance, autoantibody production, and immune complex deposition in tissues including the kidney, with inflammatory consequences. Effective clearance of immune complexes and cellular waste by early complement components protects against the development of lupus nephritis, while uncontrolled activation of complement, especially the alternative pathway, promotes kidney damage in SLE. Therefore, complement plays a dual role in the pathogenesis of lupus nephritis. Improved understanding of the contribution of the various complement pathways to the development of kidney disease in SLE has created an opportunity to target the complement system with novel therapies to improve outcomes in lupus nephritis. In this review, we explore the interactions between complement and the kidney in SLE and their implications for the treatment of lupus nephritis. Keywords: lupus nephritis; complement; systemic lupus erythematosus; glomerulonephritis Citation: Li, N.L.; Birmingham, D.J.; Rovin, B.H.
    [Show full text]
  • What Is Lupus Nephritis
    What is lupus nephritis (LN)? Lupus nephritis (LN) is inflammation of the kidney that occurs as a common symptom of systemic lupus erythematosus (SLE), also known as lupus. Proteins in the immune system called antibodies damage important structures in the kidney. ⅱ Why are the kidneys important? To understand how lupus nephritis damages the kidney, it is important to understand how the kidneys work. The kidneys’ main function is to filter out excess waste and water from the blood through the urine. Kidneys also balance the salts and minerals circulating in the blood, help control blood pressure and make red blood cells. So, when the kidneys are damaged or fail, they can’t do their job as well. As a result, the kidneys are not able to filter out waste and water into the urine causing it to stay in the blood. What are the signs and symptoms of LN? Signs to ask the doctor about include blood in the urine or foamy urine which can mean that there is excess protein. Other signs to notice are swelling of legs, ankles, hands or tissue around the eyes as well as weight gain that can be caused by fluid the body isn’t getting rid of. ⅲ Symptoms of lupus nephritis also include high blood pressure, joint/muscle pain, high levels of waste (creatinine) in the blood, or impaired/failing kidney. ⅳ How common is LN? Lupus nephritis is the most common complication of lupus. Five out of 10 adults with lupus will have lupus nephritis, while eight out of 10 children with lupus will have kidney damage, which usually stems from lupus nephritis.
    [Show full text]
  • Management of Lupus Nephritis
    Journal of Clinical Medicine Review Management of Lupus Nephritis Farah Tamirou * and Frédéric A. Houssiau Department of Rheumatology, Cliniques Universitaires Saint-Luc and Institut de Recherche Expérimentale et Clinique, UCLouvain, 1200 Bruxelles, Belgium; [email protected] * Correspondence: [email protected] Abstract: Lupus nephritis (LN) is a frequent and severe manifestation of systemic lupus erythemato- sus. The main goal of the management of LN is to avoid chronic kidney disease (CKD). Current treatment strategies remain unsatisfactory in terms of complete renal response, prevention of relapses, CKD, and progression to end-stage kidney disease. To improve the prognosis of LN, recent data suggest that we should (i) modify our treat-to-target approach by including, in addition to a clinical target, a pathological target and (ii) switch from conventional sequential therapy to combination therapy. Here, we also review the results of recent controlled randomized trials. Keywords: lupus nephritis; treat-to-target approach; repeat kidney biopsy; combination therapy 1. Introduction Lupus nephritis (LN) occurs in 12 to 69% of patients suffering from systemic lupus erythematosus (SLE), depending on case series [1]. Based on clinical and laboratory findings, it affects around 50% of SLE patients, while the rates of biopsy-proven LN are somewhat lower [2]. LN is more prevalent in Asian than in African or Hispanic and European patients [3]. 2. Pathophysiology of Lupus Nephritis Citation: Tamirou, F.; Houssiau, F.A. Management of Lupus Immune complexes (IC), produced in lymph nodes, spleen, or other lymphoid tissues Nephritis. J. Clin. Med. 2021, 10, 670. are deposited in the glomeruli of LN patients [4].
    [Show full text]
  • Lupus Nephropathy
    Rev. Colomb. Nefrol. 2014; 1(2): 101- 114. http//www.revistanefrologia.org Rev. Colomb.Review Nefrol. article2014; 1(2): 101-114 http//doi.org/10.22265/acnef.1.2.182 Lupus nephropathy Luis Fernando Pinto Peñaranda1 1Internal Medicine - Rheumatology, Medical Specialties - Research Unit, Hospital Pablo Tobón Uribe, Medellín – Colombia Abstract Lupus nephritis (LN) occurs between 30% and 70% of patients with systemic lupus erythematosus (SLE), depending on race and sex. LN appears early in the disease with prevalence of severe forms such as classes III, IV and mixed (V + III IV or V +). 50% of adults and 70% of children with lupus born in Colombia, suffer LN sometime in their lifetime; in this population 25% of children and 38% of adults have nephrotic syndrome. The remission rate at six months is low, the proteinuria in nephrotic range, and the incraease of baseline creatinine predict failure to achieve remission at 6 months. Key words: Lupus, Nephritis, Nephrotic Syndrome, Proliferative glomerulonephritis. Nefropatía lúpica Resumen La nefritis lúpica (NL) se presenta entre el 30 y 70% de los pacientes con lupus eritematoso sistémico (LES), dependiendo de la raza y el sexo, ocurre temprano en la enfermedad y predominan las formas gra- ves, clases III, IV y mixtas (V + III o V + IV). El 50% de los adultos y 70% de los niños colombianos con lupus sufren NL en algún momento de la vida; en esta población el 25% de los niños y el 38% de los adultos presentan síndrome nefrótico, la tasa de remisión a 6 meses es baja, la proteinuria en rango nefrótico y la elevación de creatinina basal, predicen falla en el logro de remisión a 6 meses.
    [Show full text]
  • Pathogenesis of Human Systemic Lupus Erythematosus: a Cellular Perspective Vaishali R
    Feature Review Pathogenesis of Human Systemic Lupus Erythematosus: A Cellular Perspective Vaishali R. Moulton,1,* Abel Suarez-Fueyo,1 Esra Meidan,1,2 Hao Li,1 Masayuki Mizui,3 and George C. Tsokos1,* Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting Trends multiple organs. A complex interaction of genetics, environment, and hormones Recent work has identified patterns of leads to immune dysregulation and breakdown of tolerance to self-antigens, altered gene expression denoting resulting in autoantibody production, inflammation, and destruction of end- molecular pathways operating in organs. Emerging evidence on the role of these factors has increased our groups of SLE patients. knowledge of this complex disease, guiding therapeutic strategies and identi- Studies have identified local, organ- fying putative biomarkers. Recent findings include the characterization of specific factors enabling or ameliorat- ing SLE tissue damage, thereby dis- genetic/epigenetic factors linked to SLE, as well as cellular effectors. Novel sociating autoimmunity and end-organ observations have provided an improved understanding of the contribution of damage. tissue-specific factors and associated damage, T and B lymphocytes, as well Novel subsets of adaptive immune as innate immune cell subsets and their corresponding abnormalities. The effectors, and the contributions of intricate web of involved factors and pathways dictates the adoption of tailored innate immune cells including platelets therapeutic approaches to conquer this disease. and neutrophils, are being increasingly recognized in lupus pathogenesis. Studies have revealed metabolic cellu- SLE, a Devastating Disease for Young Women lar aberrations, which underwrite cell SLE afflicts mostly women [1] in which the autoimmune response is directed against practically and organ injury, as important contri- all organs, leading to protean clinical manifestations including arthritis, skin disease, blood cell butors to lupus disease.
    [Show full text]
  • Glomerulonephritis Histopathological Pattern Change
    AlYousef et al. BMC Nephrology (2020) 21:186 https://doi.org/10.1186/s12882-020-01836-3 RESEARCH ARTICLE Open Access Glomerulonephritis Histopathological Pattern Change Anas AlYousef1* , Ali AlSahow2, Bassam AlHelal3, Ahmed Alqallaf4, Emad Abdallah3, Mohammed Abdellatif1, Hani Nawar2 and Riham Elmahalawy4 Abstract Background: Glomerulonephritides (GN) are relatively rare kidney diseases with substantial morbidity and mortality. They are often difficult to treat, sometimes with no cure, and can lead to chronic kidney disease (CKD) and end stage kidney disease (ESKD). Kidney biopsy is the diagnostic procedure of choice with variable indications from center to center. It helps in identifying the exact specific diagnosis, assessing the level of disease activity and severity, and hence aids in proper therapy and helps predicting prognosis. There is a global change of pattern of glomerular disease over the last five decades. Methods: Retrospective analysis of all kidney biopsies (545 cases) that were done in patients over 12 year-old over last six years in four major hospitals in Kuwait. The indications for kidney biopsy were categorized into six clinical syndromes: nephrotic syndrome, sub-nephrotic proteinuria, nephrotic syndrome plus acute kidney injury (AKI), sub- nephrotic proteinuria plus AKI, isolated hematuria, and Unexplained renal impairment. We calculated the incidence of each type of kidney disease and indication of biopsy. Results: most common indication of kidney biopsy was sub-nephrotic proteinuria associated with AKI in 179 cases (32.8%). Primary Glomerulonephritis was the main diagnosis that was reported in 356 cases (65.3%). Immunoglobulin A Nephropathy (IgAN) was the commonest lesion in primary glomerulonephritis in 85 (23.9%) cases.
    [Show full text]
  • Iga Nephropathy in Systemic Lupus Erythematosus Patients
    r e v b r a s r e u m a t o l . 2 0 1 6;5 6(3):270–273 REVISTA BRASILEIRA DE REUMATOLOGIA w ww.reumatologia.com.br Case report IgA nephropathy in systemic lupus erythematosus patients: case report and literature review Leonardo Sales da Silva, Bruna Laiza Fontes Almeida, Ana Karla Guedes de Melo, Danielle Christine Soares Egypto de Brito, Alessandra Sousa Braz, ∗ Eutília Andrade Medeiros Freire School of Medicine, Universidade Federal da Paraíba, João Pessoa, PB, Brazil a r t i c l e i n f o a b s t r a c t Article history: Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has Received 1 August 2014 nephritis as one of the most striking manifestations. Although it can coexist with other Accepted 19 October 2014 autoimmune diseases, and determine the predisposition to various infectious complica- Available online 16 February 2015 tions, SLE is rarely described in association with non-lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent Keywords: primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1280, nuclear Systemic lupus erythematosus IgA nephropathy fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesan- Glomerulonephritis gial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, show- ing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed con- dition.
    [Show full text]
  • Acute Poststreptococcal Glomerulonephritis: Immune Deposit Disease
    Acute poststreptococcal glomerulonephritis: immune deposit disease. A F Michael Jr, … , R A Good, R L Vernier J Clin Invest. 1966;45(2):237-248. https://doi.org/10.1172/JCI105336. Research Article Find the latest version: https://jci.me/105336/pdf Journal of Clinical Investigation Vol. 45, No. 2, 1966 Acute Poststreptococcal Glomerulonephritis: Immune Deposit Disease * ALFRED F. MICHAEL, JR.,t KEITH N. DRUMMOND,t ROBERT A. GOOD,§ AND ROBERT L. VERNIER || WITH THE TECHNICAL ASSISTANCE OF AGNES M. OPSTAD AND JOYCE E. LOUNBERG (From the Pediatric Research Laboratories of the Variety Club Heart Hospital and the Department of Pediatrics, University of Minnesota, Minneapolis, Minn.) The possible role of immunologic mechanisms in the kidney in acute glomerulonephritis have also acute poststreptococcal glomerulonephritis was revealed the presence of discrete electron dense suggested in 1908 by Schick (2), who compared masses adjacent to the epithelial surface of the the delay in appearance of serum sickness after glomerular basement membrane (11-18). injection of heterologous serum to the latent pe- The purpose of this paper is to describe immuno- riod between scarlet fever and onset of acute glo- fluorescent and electron microscopic observations merulonephritis. Evidence in support of this con- of the kidney in 16 children with acute poststrepto- cept is the depression of serum complement during coccal glomerulonephritis. This study demon- the early stages of the disease (3) and glomerular strates 1) the presence of discrete deposits of yG- localization of immunoglobulin. Immunofluores- and fl3c-globulins along the glomerular basement cent studies have revealed either no glomerular membrane and its epithelial surface that are similar deposition of a-globulin (4) or a diffuse involve- in size and location to the dense masses seen by ment of the capillary wall (5-9).
    [Show full text]
  • Human Lupus Nephritis and Tubulointerstitial Inflammation in In
    In Situ B Cell-Mediated Immune Responses and Tubulointerstitial Inflammation in Human Lupus Nephritis This information is current as Anthony Chang, Scott G. Henderson, Daniel Brandt, Ni Liu, of September 26, 2021. Riteesha Guttikonda, Christine Hsieh, Natasha Kaverina, Tammy O. Utset, Shane M. Meehan, Richard J. Quigg, Eric Meffre and Marcus R. Clark J Immunol 2011; 186:1849-1860; Prepublished online 27 December 2010; Downloaded from doi: 10.4049/jimmunol.1001983 http://www.jimmunol.org/content/186/3/1849 Supplementary http://www.jimmunol.org/content/suppl/2010/12/27/jimmunol.100198 http://www.jimmunol.org/ Material 3.DC1 References This article cites 85 articles, 21 of which you can access for free at: http://www.jimmunol.org/content/186/3/1849.full#ref-list-1 Why The JI? Submit online. by guest on September 26, 2021 • Rapid Reviews! 30 days* from submission to initial decision • No Triage! Every submission reviewed by practicing scientists • Fast Publication! 4 weeks from acceptance to publication *average Subscription Information about subscribing to The Journal of Immunology is online at: http://jimmunol.org/subscription Permissions Submit copyright permission requests at: http://www.aai.org/About/Publications/JI/copyright.html Email Alerts Receive free email-alerts when new articles cite this article. Sign up at: http://jimmunol.org/alerts The Journal of Immunology is published twice each month by The American Association of Immunologists, Inc., 1451 Rockville Pike, Suite 650, Rockville, MD 20852 Copyright © 2011 by The American Association of Immunologists, Inc. All rights reserved. Print ISSN: 0022-1767 Online ISSN: 1550-6606. The Journal of Immunology In Situ B Cell-Mediated Immune Responses and Tubulointerstitial Inflammation in Human Lupus Nephritis Anthony Chang,*,1 Scott G.
    [Show full text]
  • Characterization of Iga Deposition in the Kidney of Patients with Iga Nephropathy and Minimal Change Disease
    Journal of Clinical Medicine Article Characterization of IgA Deposition in the Kidney of Patients with IgA Nephropathy and Minimal Change Disease Won-Hee Cho 1, Seon-Hwa Park 2, Seul-Ki Choi 2, Su Woong Jung 2, Kyung Hwan Jeong 3, Yang-Gyun Kim 2 , Ju-Young Moon 2, Sung-Jig Lim 4 , Ji-Youn Sung 5, Jong Hyun Jhee 6, Ho Jun Chin 7,8, Bum Soon Choi 9 and Sang-Ho Lee 2,* 1 Department of Medicine, Graduate School, Kyung Hee University, Seoul 02447, Korea; [email protected] 2 Division of Nephrology, Department of Internal Medicine, Kyung Hee University Hospital at Gangdong, Seoul 05278, Korea; [email protected] (S.-H.P.); [email protected] (S.-K.C.); [email protected] (S.W.J.); [email protected] (Y.-G.K.); [email protected] (J.-Y.M.) 3 Division of Nephrology, Department of Internal Medicine, Kyung Hee University Medical Center, Seoul 02447, Korea; [email protected] 4 Department of Pathology, Kyung Hee University Hospital at Gangdong, Kyung Hee University, Seoul 05278, Korea; [email protected] 5 Department of Pathology, Kyung Hee University Hospital, Kyung Hee University College of Medicine, Seoul 02447, Korea; [email protected] 6 Division of Nephrology, Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06273, Korea; [email protected] 7 Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam 13620, Korea; [email protected] 8 Department of Internal Medicine, College of Medicine, Seoul National University, Seoul 03080, Korea 9 Division of Nephrology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul 06591, Korea; [email protected] * Correspondence: [email protected] Received: 5 July 2020; Accepted: 9 August 2020; Published: 12 August 2020 Abstract: Approximately 5% of patients with IgA nephropathy (IgAN) exhibit mild mesangial lesions with acute onset nephrotic syndrome and diffuse foot process effacement representative of minimal change disease (MCD).
    [Show full text]
  • Therapy of Membranous Nephropathy in Systemic Lupus Erythematosus
    Therapy of Membranous Nephropathy in Systemic Lupus Erythematosus By James E. Balow and Howard A. Austin, III Historic changes in the criteria for pathologic diagnosis and classification of lupus membranous nephropathy (LMN) have precluded definitive descriptions of the natural history, prognosis, and treatment of this disorder. The interim practice, based on the 1982 World Health Organization classification system, of admixing membranous and proliferative lupus nephropathies under the rubric of LMN has confounded the medical literature. Cases with mixed histology should be treated according to recommendations for proliferative lupus nephritis. Patients with LMN should be treated early with angiotensin antagonists to minimize proteinuria, as well as lifestyle changes and appropriate drugs to reduce attendant cardiovascular risk factors. In patients with protracted nephrotic syndrome, consideration should be given to immunosuppressive therapies including corticosteroids, cyclosporine, myco- phenolate, and cyclophosphamide. Prospective controlled trials clearly are needed to establish solid clinical practice guidelines for use of these drugs and other experimental therapies currently under study in LMN. This is a US government work. There are no restrictions on its use. ORE THAN ONE HALF of patients with specific intervention to reduce the risk for renal Msystemic lupus erythematosus (SLE) de- failure. Typical recommendations were to tailor velop clinically significant nephropathy during the immunosuppressive drugs according to the re- course of their disease. Approximately 20% of quirements for control of extrarenal SLE disease those with SLE renal involvement are found on activity. renal biopsy examination to have lupus membra- The original perspective on renal prognosis was nous nephropathy (LMN). Unfortunately, LMN altered dramatically by the 1982 World Health has not been defined in a uniform fashion over the Organization classification system to subsume cer- past several decades.
    [Show full text]
  • Membranous Nephropathy with Crescents
    PATHOPHYSIOLOGY of the RENAL BIOPSY www.jasn.org Membranous Nephropathy with Crescents Amanda Walton Basford,* Julia Lewis,* Jamie P. Dwyer,* and Agnes B. Fogo*† Division of Nephrology and Hypertension, Departments of *Medicine and †Pathology, Vanderbilt University Medical Center, Nashville, Tennessee ABSTRACT Membranous nephropathy is a common cause of nephrotic syndrome in adults concentration of 3.4 g/dl, urinalysis with and can be primary or secondary to systemic lupus erythematosus, chronic 2ϩ protein, one red blood cell, and five infection, or drugs. Rapid decline in renal function in patients with membranous white blood cells (WBCs) without casts. nephropathy may be due to renal vein thrombosis, malignant hypertension, or On admission, serum sodium was 136 an additional superimposed destructive process involving the renal paren- mEq/L, potassium was 4.3 mEq/L, chlo- chyma. Crescents are rare in primary membranous nephropathy and thus sug- ride was 107 mEq/L, bicarbonate was 20 gest another underlying disease process, such as combined membranous and focal mmol/L, blood urea nitrogen was 36 mg/ or diffuse lupus nephritis. However, in some patients with membranous nephrop- dl, serum creatinine was 2.84 mg/dl, athy and crescents, the crescentic lesion may be due to a distinct, separate disease magnesium was 2.2 mg/dl, phosphorus process, such as anti-glomerular basement membrane antibodies or anti- was 4.6 mg/dl, total bilirubin was 0.7 mg/ neutrophil cytoplasmic antibodies-related pauci-immune glomerulonephritis. Here dl, amylase was 17 U/L, and lipase was 14 we describe a case with such renal biopsy findings, review previous reported cases, U/L.
    [Show full text]