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Renal Tubular Acidosis. Bethesda, MD: National Kidney and Urologic Diseases Information Clearinghouse. 2008. 6 p.
Renal tubular acidosis (RTA) is a disease that occurs when the kidneys fail to excrete acids into the urine, which causes a person’s blood to remain too acidic. Without proper treatment, chronic acidity of the blood leads to growth retardation, kidney stones, bone disease, and progressive renal failure. This fact sheet reviews the diagnosis, the subtypes of RTA, therapy, and current research activities in RTA. To diagnose RTA, the doctor will check the acid-base balance in samples of the patient’s blood and urine. Physicians use a three-category classification system to describe the different types of RTA. Type 1, also called classic distal RTA, is an inherited disorder associated with diseases that affect many organ systems such as the autoimmune disorders Sjögren’s syndrome and lupus erythematosus. Type 2 is called proximal RTA and occurs most frequently in children as part of a disorder called Fanconi’s syndrome; it can also occur as a side effect of treatment with ifosfamide, a drug used in chemotherapy. Type 4 is caused by another defect in the kidney tubule but is different from classic or proximal RTA because it results in high levels of potassium in the blood instead of low levels. If treated early, most people with RTA will not develop permanent kidney failure. Therefore, the goal is early recognition and adequate therapy, which will need to be maintained and monitored throughout the patient’s lifetime. The fact sheet concludes with a summary of research programs in Renal tubular acidosis (RTA) area and a brief description of the activities of the National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC), a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) that provides information about diseases of the kidneys and urologic system to patients and their families, the general public, and health care professionals. Readers are referred to the National Kidney Foundation at www.kidney.org or 1–800–622–9010, the American Association of Kidney Patients at www.aakp.org or 1–800–749–2257, and the American Kidney Fund at www.kidneyfund.org or 1–800–638–8299 for more information.
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Lupus Nephritis. Bethesda, MD: National Kidney and Urologic Diseases Information Clearinghouse. 2007. 2 p.
This fact sheet describes lupus nephritis, an inflammation of the kidney caused by systemic lupus erythematosus (SLE), a disease of the immune system. Written in a question-and-answer format, the fact sheet describes the symptoms of lupus nephritis, how the condition is diagnosed, treatment options, and where patients can get more information. Lupus nephritis may cause weight gain, high blood pressure, dark urine, or swelling around the eyes, legs, ankles, or fingers. Diagnosis may require urine and blood tests as well as a kidney biopsy. Treatment depends on the symptoms and test results. Corticosteroids may be used to decrease swelling and inflammation by suppressing the immune system. Patients may need medications to help control their blood pressure and a diet limited in sodium, protein, and potassium. The fact sheet includes the contact information for the National Institute of Arthritis and Musculoskeletal and Skin Diseases, and the Lupus Foundation of America, and a brief description of the goals and activities of the National Kidney and Urologic Diseases Information Clearinghouse.
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Glomerular Diseases. Bethesda, MD: National Kidney and Urologic Diseases Information Clearinghouse. 2006. 12 p.
This fact sheet reviews glomerular diseases, which involve problems with the glomeruli, the tiny units within the kidney where blood is cleaned. The fact sheet focuses on glomerulonephritis, defined as inflammation of the membrane tissue in the kidney; and glomerulosclerosis, the scarring or hardening of the tiny blood vessels within the kidney. Written in a question-and-answer format, the fact sheet covers the anatomy and function of the kidneys, how glomerular diseases interfere with kidney function, the symptoms of glomerular disease, diagnostic tests used to confirm glomerular disease, the causes of glomerular disease, renal failure, and end-stage renal disease (ESRD). Specific diseases covered include systemic lupus erythematosus (SLE), Goodpasture's syndrome, IgA nephropathy, Alport syndrome, infection-related glomerular disease, bacterial endocarditis, diabetic nephropathy, focal segmental glomerulosclerosis, and minimal change disease (MCD). The booklet summarizes the points covered, provides a brief glossary of terms, lists resource organizations for readers seeking additional information, and a briefly describes the goals and activities of the National Kidney and Urologic Diseases Information Clearinghouse. 2 figures.
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Spectrum of Renal Diseases Associated with Extreme Forms of Insulin Resistance. Clinical Journal of the American Society of Nephrology. 1(4): 616-622. July 2006.
This review article considers the spectrum of renal diseases associated with extreme forms of insulin resistance. In patients with syndromes of extreme insulin resistance, the proteinuric forms of renal disease are common, but the authors note that the renal pathology usually is not diabetic nephropathy. For example, in the lipodystrophy syndromes, membranoproliferative glomerulonephritis type 1 and type 2, focal segmental glomerulosclerosis, and also diabetic nephropathy are seen. In the syndromes of autoantibodies to the insulin receptor, the various forms of lupus glomerulonephritis are seen. Even in patients with type 2 diabetes, the renal pathology may not be diabetic nephropathy. The authors conclude that this means that renal biopsy has an important role in defining the pathology that leads to proteinuric nephropathy; this leads to a more successful therapeutic approach. 4 figures. 4 tables. 40 references.
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Evaluation and Preparation of Renal Transplant Candidates. IN: Danovitch, G.M. Handbook of Kidney Transplantation. Philadelphia, PA: Lippincott Williams and Wilkins. 2005. pp. 169-192.
The preparation of patients with end-stage renal disease for kidney transplantation should start from the time of recognition of progressive chronic kidney disease (CKD). This chapter on the evaluation and preparation of renal transplant candidates is from a handbook that offers a practical guide for health care providers who manage kidney transplant patients. The authors note that the management of these patients consists of an initial evaluation followed, if they are appropriate transplant candidates, by their supervision while awaiting transplantation. Initial evaluation is designed not only to assess the chances of recovery from surgery, but also to increase short- and long-term patient survival. The limited number of organs available has changed the focus of the transplant evaluation toward better long-term outcome over short-term benefits. The authors first focus on the initial recipient evaluation and then consider the management of the waiting list for deceased donor transplantation. Specific topics covered include the benefits of early referral, patient education, the routine evaluation, the evaluation of specific transplant risk factors related to organ system disease (cardiovascular disease, cerebrovascular and peripheral vascular disease, malignancy, infections, gastrointestinal disease, pulmonary disease, urologic evaluation, renal osteodystrophy and metabolic bone disease, and hypercoagulable states), and risk factors related to specific patient characteristics (aged, obesity, highly sensitized patients, previously transplanted patients, and candidates for double-organ transplants). An additional section considers the relevance of the etiology of renal disease to the transplant evaluation, including diabetes mellitus, focal and segmental glomerulosclerosis, recurrent glomerulonephritis, thrombotic thrombocytopenic purpura, systemic lupus erythematosus and vasculitis, oxalosis and oxaluria, Fabry disease, Alport syndrome, sickle cell disease, amyloidosis and plasma cell dyscrasias, and polycystic kidney disease. 1 figure. 5 tables. 16 references.
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Medical and Surgical Aspects of Kidney Donation. IN: Danovitch, G.M. Handbook of Kidney Transplantation. Philadelphia, PA: Lippincott Williams and Wilkins. 2005. pp. 135-168.
The appropriate identification and preparation of kidney donors (both living and deceased) contribute critically to the success of the transplant endeavor on both the individual and the national levels. This chapter on the medical and surgical aspects of kidney donation is from a handbook that offers a practical guide for health care providers who manage kidney transplant patients. The authors divide the chapter into two sections: Part I addresses the selection and evaluation of living donors and the surgical techniques of living donor nephrectomy; Part II discusses these same issues for deceased kidney donors. Specific topics include the role of informed consent, the evaluation process, the psychosocial evaluation, risks of donation, donor age, assessment of surgical risks, the risk of disease transmission to the recipient, evaluation of future risk to the donor, assessment of renal function (glomerular filtration rate, proteinuria, hematuria, hypertension, diabetes, obesity, nephrolithiasis, inherited renal disease, autosomal dominant polycystic kidney disease, Alport syndrome, familial primary glomerulonephritis, and systemic lupus erythematosus), surgical evaluation of the living kidney donor, surgical techniques for living donor nephrectomy, long-term postnephrectomy issues (renal function, pregnancy, employment and insurance, and long-term medical care), and controversies and innovations in living kidney donor practice, including biologically unrelated donors, incompatible donor and recipient, paid donation, and the living donor registry. The second section discusses contraindications to deceased donor donation, the role of donor age, expanded criteria donors, donor biopsy, nephron dose, donation after cardiac death, diagnosis of brain death, techniques of deceased donor organ retrieval, and deceased donor kidney preservation. 2 figures. 10 tables. 32 references.
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Oculorenal Manifestations in Systematic Autoimmune Diseases. American Journal of Kidney Diseases. 43(2): 209-222. February 2004.
Vasculitides form a heterogeneous group of diseases characterized by blood-vessel inflammation and necrosis (tissue death). Systemic necrotizing vasculitis is characterized by inflammation of blood vessels, which often affects the eyes and kidneys. Vasculitides have a wide spectrum of manifestations because of the involvement of arteries and other vessels of various sizes and locations. Early diagnosis and prompt treatment may decrease the morbidity and mortality associated with systemic autoimmune diseases. In addition, the eyes and kidneys can provide clues to the diagnosis of many systemic diseases and many important complications of these diseases occur in the eye. Therefore, examination of the eyes and kidneys should be a routine and important part of a general examination in systemic diseases. This article reviews the major types of oculorenal manifestations in systemic autoimmune diseases. Diseases discussed include giant cell (temporal) arteritis, polyarteritis nodosa, Kawasaki disease, Wegener's granulomatosis and microscopic polyarteritis, Goodpasture's syndrome, IgA nephropathy and Henoch-Schonlein purpura nephritis, Churg-Strauss syndrome, Behcet's disease, systemic lupus erythematosus, primary antiphospholipid syndrome (APS), sarcoidosis, Sjogren's syndrome, cryoglobulinemia, and tubulointerstitial nephritis and uveitis syndrome. 6 figures. 124 references.
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Update in Nephrology. Annals of Internal Medicine. 140(2): 106-111. January 2004.
This article summarizes important, recent clinical advances in nephrology; the author focuses on three key topics of interest: hypertension (high blood pressure), proteinuria (protein in the urine), and renal replacement therapy (RRT, which include dialysis and transplantation). Two single studies, one in the area of nephrolithiasis (kidney stones) and the other dealing with glomerulonephritis in the form of lupus-related nephropathy, are also reviewed. The editor chose studies that provide pathophysiologic insight into a given problem or that are likely to alter clinical practice. Each study is briefly summarized, with clinical strategies highlighted. 3 references.
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Recurrent Lupus Nephritis in Renal Transplant Recipients Revisited: It Is Not Rare. Transplantation. 75(5): 651-656. March 2003.
Although recurrent lupus nephritis (RLN) after kidney transplantation is reported to be rare (1 to 4 percent), recent studies suggest a higher incidence. This article reports on a study undertaken to determine the incidence of RLN in a large cohort of renal transplant recipients with systemic lupus erythematosus (SLE). The authors reviewed the records of 54 renal (kidney) transplant recipients with SLE. Thirty-one patients underwent biopsy because of worsening renal function and proteinuria (protein in the urine). Among the 50 patients with at least 3 months of followup, RLN was present in 15 (52 percent of patients who underwent biopsy, 30 percent of total patients). One patient had graft loss because of RLN at 10.5 years. The duration of dialysis before transplantation was not different between patients with RLN compared to patients without RLN. Overall patient survival was 96 percent at 1 year and 82 percent at 5 years. The authors conclude that RLN is more common than previously reported, but in this series, graft loss because of RLN was rare. Aggressive use of allograft biopsies and morphologic evaluation are important factors in the diagnosis of RLN. 2 figures. 2 tables. 29 references.
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Recurrent Lupus Nephritis in Renal Transplant Recipients Revisited: It Is Not Rare. Transplantation. 75(5): 651-656. March 2003.
Although recurrent lupus nephritis (RLN) after kidney transplantation is reported to be rare (1 to 4 percent), recent studies suggest a higher incidence. This article reports on a study undertaken to determine the incidence of RLN in a large cohort of renal transplant recipients with systemic lupus erythematosus (SLE). The records of 54 renal transplant recipients with SLE were reviewed; 31 patients underwent biopsy because of worsening renal function and proteinuria. Among the 50 patients with at least 3 months of follow up, RLN was present in 15 (52 percent of patients who underwent biopsy, 30 percent of total patients): mesangial lupus nephritis (LN) in eight patients, focal proliferative LN in four, and membranous LN in three patients. One patient had graft loss because of RLN at 10.5 years. The duration of dialysis before transplantation was not different between patients with RLN compared to patients without RLN. Overall patient survival (n = 50) was 96 percent at 1 year and 82 percent at 5 years. Graft survival was worse in patients who underwent biopsy compared with patients who never underwent biopsy. The authors conclude that RLN is more common than previously reported, but in this series, graft loss because of RLN was rare. Aggressive use of allograft biopsies and morphological evaluation are important factors in the diagnosis of RLN. 2 figures. 2 tables. 29 references.
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Race and Kidney Disease: Role of Social and Environmental Factors. Journal of the National Medical Association. 94(8 Supplement): 28S-38S. August 2002.
Numerous studies have documented the presence of racial disparities among Americans in health outcomes with respect to cardiovascular disease, infant mortality, cancer, and kidney disease. With regard to kidney diseases, these disparities are more dramatic. African, Hispanic, and Native Americans have the highest risks of end stage renal disease (ESRD). The incidence of ESRD is four times higher in African Americans than in whites. This article considers the role of social and environmental factors in this disparity. Diseases causing chronic kidney failure, such as diabetes mellitus, hypertension (high blood pressure), systemic lupus erythematosus (SLE), and HIV associated kidney disease, are particularly prevalent among African-American patients. In addition to the higher prevalence, the morbidity (complications and related illness) associated with kidney complications of these diseases appears worse in African American patients. African Americans also have worse outcomes and a relatively reduced access to kidney transplantation (the best therapy for ESRD). The authors note that it is highly likely that social and environmental factors play a very significant role in the persistence of these disparities. A detailed understanding of these socioeconomic and environmental factors will be critical in formulating rational public health strategies to redress these disparities.
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Advances in the Treatment of Lupus Nephritis. In: Coggins, C.H.; Hancock, E.W., Eds. Annual Review of Medicine: Selected Topics in the Clinical Sciences, Volume 45. Palo Alto, CA: Annual Reviews Inc. 2001. p. 63-78.
Systemic lupus erythematosus (SLE) is an autoimmune disease that leads to the formation and deposition of immune complexes throughout the body, which are pathogenic (causing disease) for SLE. Different forms of glomerulonephritis (inflammation of the filtering units of the kidney) can occur in patients with SLE and can contribute significantly to the associated morbidity (illness and complications) and, ultimately, mortality (death) from the disease. Over the past two decades, there have been significant strides in the understanding of the disease and in treatments that attempt to control the formation and deposition of anti-DNA auto-antibodies and immune complexes, as well as the subsequent inflammatory cascade mediated through various cellular and humoral pathways leading to progressive renal (kidney) damage and end stage renal disease (ESRD). This article reviews the current understanding of the pathogenesis and treatment of lupus nephritis in its various stages and discusses the experimental and human data regarding some of the potential newer forms of therapy. The authors discuss data regarding the use of steroids, azathioprine, cyclophosphamide, cyclosporine A, mycophenolate mofetil, gammaglobulin, plasmapheresis, LJP 394, flaxseed oil, bindarit, anti-CD-40 ligand, and CRLA41g. The authors conclude that the long term morbidity and mortality for patients with lupus nephritis (LN) has improved markedly over the past two decades. This is due in part to the addition of newer adjunctive therapies to control blood pressure and intraglomerular pressure, reduce proteinuria (protein in the urine), and manage hyperlipidemia (high levels of fats in the blood). 89 references.
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Glomerulonephritis Recurrence in the Renal Graft. JASN. Journal of the American Society of Nephrology. 12(2): 394-402. February 2001.
Although kidney transplantation may return renal (kidney) function to the recipient, it does not necessarily remove the cause of the recipient's original kidney disease. Glomerulonephritis is the cause of renal failure for 20 to 40 percent of those who receive a transplant; for these recipients, the threat of recurrent disease is very real. This article discusses recurrent glomerulonephritis. The author first reviews the epidemiology of recurrence in a general sense, then addresses recurrence of specific forms of the disease. The incidence of recurrence and recurrence leading to graft failure is examined, and risk factors for disease recurrence are assessed. Where available, data on the pathogenesis and management of recurrent glomerulonephritis is also presented. The typical features of recurrent glomerulonephritis are those of nephritis involving the native kidney, including proteinuria (protein in the urine), hematuria (blood in the urine), and deterioration in renal function. When the diagnosis of recurrence is suspected, renal biopsy is essential. The author discusses IgA nephropathy and Henoch Schonlein purpura; antineutrophil cytoplasmic antibody associated vasculitis, Wegener's granulomatosis, microscopic polyangiitis, and idiopathic necrotizing crescentic glomerulonephritis; anti GBM disease; hemolytic uremic syndrome (HUS); focal and segmental glomerulosclerosis; membranous glomerulonephritis; mesangiocapillary glomerulonephritis; lupus nephritis; systemic sclerosis and scleroderma; and fibrillary glomerulonephritis and immunotactoid glomerulonephritis. Strong data have emerged on patterns of recurrence, risk factors for recurrence, and the implications for patient and graft outcomes after recurrence of the most common glomerulopathies. However, data available on recurrence of the less common nephropathies are inadequate and make treatment and prevention more difficult. 1 figure. 1 table. 60 references.
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Goodpasture Syndrome: Pathophysiology, Diagnosis, and Management. Nephrology Nursing Journal. 28(3): 305-310. June 2001.
Goodpasture syndrome is an autoimmune disease characterized by glomerulonephritis (inflammation of the capillary loops in the glomeruli of the kidney), pulmonary hemorrhage (bleeding in the lungs), and autoantibodies to the glomerular and alveolar basement membranes. This article reviews the pathophysiology, diagnosis, and management of patients with Goodpasture syndrome. The cause of the condition is unknown and, if left untreated, Goodpasture syndrome usually is fatal. Early diagnosis and prompt initiation of therapy can decrease disease progression and increase the patient's chance of survival. Differential diagnosis includes Wegener's granulomatosis, Henoch Schonlein purpura, lupus nephritis, and polyarteritis nodosa. Diagnostic tests include chest x rays, urinalysis, renal (kidney) biopsy, and lung biopsy. Treatment modalities remain controversial and vary among practitioners. Currently, treatment is focused on plasmapheresis and immunosuppression. The goals for treatment are to remove circulating antibodies, stop further production of antibodies, and remove any antigens that stimulate antibody formation. Plasmapheresis, or therapeutic plasma exchange (TPE), is an extracorporeal (outside the body) process that decreases large molecular weight substances in the blood; as this removal is taking place, a replacement fluid is used to maintain hemodynamic stability. The replacement fluid used for patients with Goodpasture syndrome is albumin (protein). Complications of TPE can include hypotension (low blood pressure), citrate metabolic alkalosis, respiratory distress, and allergic reaction. Renal transplantation provides a good option for these patients, particularly if other therapies first reduce or eliminate autoantibody production before transplantation is undertaken. 1 table. 27 references.
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Renal Disease in the Inner City. Seminars in Nephrology. 21(4): 334-345. July 2001.
For various ethnic and socioeconomic reasons, the pattern of renal (kidney) disease in the inner city displays distinctive features. This article covers the evolution and present status of hypertension and nephrology (study and medicine of the kidney) in the inner city as seen by the authors over several decades. Hypertension (high blood pressure) is frequent, often intractable, and generally conditioned by salt sensitivity and a high sodium intake. Chronic hypertensive nephrosclerosis, found predominantly in African Americans, comprises marked cardiomegaly (increased heart size), renal shrinkage, and hypertensive retinopathy (eye disease). While hypertension has been overdiagnosed in the past, it actually accounts for less than 20 percent of end stage renal disease (ESRD) in African Amerians. Malignant hypertension, less frequent nowadays, may cause renal shutdown, which is reversible in a few cases; the heart and kidneys are often of normal size. Idiopathic focal segmental glomerulosclerosis (scarring in the filtering parts of the kidney) is the most common cause of the primary nephrotic syndrome in blacks, but its incidence has also been rising in whites and Hispanics; it does not respond well to treatment, and almost one half of the patients develop ESRD within 10 years. Systemic lupus erythematosus is also more common in African Americans, in whom the severe proliferative forms of lupus nephritis (kidney infection associated with lupus) pursue a more deadly course: one half of such patients develop ESRD in 5 years. Cocaine, the use of which has assumed epidemic proportions, may cause accelerated hypertension, acute renal failure from rhabdomyolysis (a disease of skeletal muscle), and progression of preexisting renal disease. Heroin nephropathy has all but disappeared and has been replaced by HIV nephropathy. The prognosis of HIV infected patients maintained by dialysis has greatly improved. Sickle glomerulopathy (kidney disease associated with sickle cell anemia) may cause the nephrotic syndrome in 4 percent of patients with severe sickle cell anemia, heralding death within 2 years in one half of patients and ESRD in two thirds; survival has not improved with dialysis. Diabetes is now the most common cause of ESRD. 92 references.
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Efficacy of Mycophenolate Mofetil in Patients with Diffuse Proliferative Lupus Nephritis. New England Journal of Medicine. 343(16): 1156-1162. October 19, 2000.
The combination of cyclophosphamide and prednisolone is effective for the treatment of severe lupus nephritis (kidney inflammation associated with systemic lupus erythematosus or SLE) but has serious adverse effects. This article reports on a study that investigated the efficacy of mycophenolate mofetil in patients (n = 42) with proliferative lupus nephritis. The authors compared the efficacy and side effects of a regimen of prednisolone and mycophenolate mofetil given for 12 months (group 1) with those of a regimen of prednisolone and cyclophosphamide given for 6 months, followed by prednisolone and azathioprine for 6 months (group 2). Of the patients in Group 1 (n = 21), 81 percent had a complete remission, and 14 percent had a partial remission, as compared with 76 percent and 14 percent, respectively, of the 21 patients in Group 2. The improvements in the degree of proteinuria (protein in the urine) and the serum albumin (protein levels in the blood) and creatinine concentrations were similar in the two groups. One patient in each group discontinued treatment because of side effects. Infections were noted in 19 percent of the patients in Group 1 and in 33 percent of those in Group 2. Other adverse effects occurred only in group 2; they included amenorrhea (23 percent), hair loss (19 percent), leukopenia (10 percent), and death (10 percent). The rates of relapse were 15 percent in Group 1, and 11 percent in Group 2. The authors conclude that for the treatment of diffuse proliferative lupus nephritis, the combination of mycophenolate mofetil and prednisolone is as effective as a regimen of cyclophosphamide and prednisolone followed by azathioprine and prednisolone, with similar levels of toxicity. 2 figures. 4 tables. 15 references.
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Evaluation of the Transplant Recipient. In: Danovitch, G.M., ed. Handbook of Kidney Transplantation. 3rd ed. Philadelphia, PA: Lippincott Williams and Wilkins. 2000. p. 130-145.
This chapter on evaluation of the transplant recipient is from a handbook of kidney transplantation that provides practical information on therapy, patient monitoring, and patient care management. In this chapter, the authors stress that the potential kidney transplant recipient must be evaluated by the transplantation team to determine whether he or she is a suitable candidate. The patient must also make a personal evaluation of the transplantation option, and the transplant team must see to it that the patient's evaluation is an educated one. The excellent statistics achieved by most transplantation centers for graft survival and morbidity have changed the attitude of both physicians and patients regarding the appropriateness of transplantation. Now, nearly all patients with end stage renal disease (ESRD) can be regarded as potentially acceptable candidates for transplantation. Instead of denying the option to broad groups of patients, such as the elderly or those with diabetes mellitus and coronary artery disease, each person's candidacy should be evaluated individually. The author of this article outlines and briefly explains contraindications to kidney transplantation, including malignancy, chronic infection, severe extrarenal disease, noncompliance, and psychiatric illness. Other topics include the general medical evaluation of the recipient, urologic evaluation, patient education concerns, special features related to the primary kidney disease (including diabetes mellitus, systemic lupus erythematosus, focal glomerulosclerosis, Goodpasture's syndrome, Alport's syndrome, amyloidosis, paraproteinemia, polycystic kidney disease, Fabry's disease, scleroderma, hyperoxaluria, thrombotic thrombocytopenic purpura, systemic vasculitis and Wegener's granulomatosis, and sickle cell disease), risk factors related to organ system diseases, and risk factors related to individual patient characteristics (age, obesity, malnutrition, peritoneal dialysis, previous transplantations, double organ candidates). 4 tables. 17 references.
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Lower Urinary Tract Symptoms in Patients with Sjogren's Syndrome and Systemic Lupus Erythematosus. International Urogynecology Journal. 11(2): 84-86. 2000.
Sjogren's syndrome (SS) and systemic lupus erythematosus (SLE) are autoimmune diseases which have many similarities with interstitial cystitis (IC), a urinary bladder disease with unknown etiology. This article reports on a survey studying the occurrence, severity, and nature of lower urinary tract symptoms among patients with SS or SLE. The results showed that these patients have significantly more urinary complaints, especially irritative bladder symptoms, than age and sex matched controls. The authors studied 36 patients with SS, 85 patients with SLE, and 121 controls. In these groups, 25 percent, 29 percent, and 66 percent, respectively, were free of urinary symptoms. The prevalences of mild symptoms were 61 percent (SS), 62 percent (SLE), and 27 percent (control group); and severe symptoms 14 percent (SS), 9 percent (SLE), and 7 percent (control group). SS and SLE patients with urinary complaints reported mostly urinary frequency (27 percent of SS and 62 percent of SLE patients) and suprapubic pain (36 percent of SS and 34 percent of SLE patients). The most common symptom in the control group was stress urinary incontinence. The frequency of lower urinary tract problems in patients with SS and SLE supports the concept that autoimmune disorders also have bladder manifestations. 2 tables. 21 references.
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Progress in the Treatment of Proliferative Lupus Nephritis. Current Opinion in Nephrology and Hypertension. 9(2): 107-115. 2000.
Lupus nephritis (kidney inflammation associated with systemic lupus erythematosus, or SLE) is often well developed at the time of diagnosis. This article reviews progress in the treatment of proliferative lupus nephritis. High dose corticosteroids are universally accepted as the initial approach to the control of severe inflammation in the kidney. Long term disease control and the minimization of iatrogenic (physician caused) risk usually require adjunctive therapies that target the more fundamental immunoregulatory disturbances of lymphoid cells. Of the available cytotoxic drugs, cyclophosphamide is currently among the most effective, although it cannot be considered ideal in terms of efficacy or toxicity. New prospects for the treatment of proliferative lupus nephritis include novel immunosuppressive agents (e.g., mycophenolate, cyclosporine, fludarabine), combination chemotherapy (e.g., cyclophosphamide plus fludarabine), and sequential chemotherapy (e.g., cyclophosphamide followed by azathioprine), immunological reconstitution using intensive cytoreductive chemotherapy (with or without stem cell rescue), and co stimulatory molecule inhibition. Gene therapy remains an attractive prospect, but its feasibility clearly depends on the further definition of lupus promoting genes and the availability of methods to establish stable expression of disease corrective genes in the appropriate lymphoid cells. 3 figures. 83 references.
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Seeing the Forest Through the Trees: For Many Patients IC Is Not Just a Bladder Disease. ICA Update. 15(1): 4-6. 2000.
This article, from a newsletter from the Interstitial Cystitis Association (ICA), reminds readers of the variations in interstitial cystitis (IC) that may occur. IC patients, when comparing their symptoms with other IC patients, may have found that not all experience the same urinary and pelvic symptoms (urinary frequency, urgency, and pain). And while some IC patients' symptoms are limited to the pelvic region or urinary tract, others have found that they experience symptoms beyond the bladder as well. The author reassures readers that if they notice that there seems to be more going on with their IC than just a bladder disease, they may be accurately perceiving certain related disorders that may be occurring. The author then outlines the top three related disorders: allergies, irritable bowel syndrome (IBS), and sensitive skin. Other conditions that are often related to IC are also discussed: vulvodynia, fibromyalgia, migraine, endometriosis, urge incontinence, asthma, inflammatory bowel disease (Crohn's disease and ulcerative colitis), and lupus erythematosis. The author notes that it may be that several different contributing factors or processes are occurring simultaneously in some IC patients. Research continues to study the involvement of neural, immune, and endocrine systems. For some patients, just knowing that these associated disorders and their connection to IC are being studied is a source of validation for the myriad of symptoms experienced beyond the bladder. The author encourages readers to seek out urologists who are developing multimodal approaches to treating IC and other related diseases. 1 figure. 14 references.
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Systemic Lupus Erythematosus: When Your Immune System Attacks You. Healthline. p.16-20. November-December 2000.
Systemic lupus erythematosus is one of a group of autoimmune diseases in which the immune system attacks the patient's own body tissues and organs. Lupus is often manifested through joint pains and skin rash, but it can also involve other organs, such as the kidneys. This article describes lupus, including the causes, the symptoms, how it is diagnosed, and the different means of treatment. Genes influence the appearance and manifestation of lupus. The differences in the manifestation of lupus in the two sexes (a higher prevalence in females) can be explained by the hormonal influences of estrogen and of the androgens. The criteria for a diagnosis of lupus include skin rash, facial butterfly shaped rash, hair loss, over sensitivity to sunlight, ulcers of the mouth, joint pain, inflammation of the lining of the heart and lungs, kidney damage, damage to the central nervous system, defects in blood cells, evidence of damage to the immune system (low level of the complement proteins), and presence of antinuclear antibodies in the blood (autoantibodies). Treatment options include nonsteroidal antiinflammatory drugs (NSAIDs), steroids, immunosuppressive drugs, and antimalaria drugs. The author describes some of the challenges to living with a chronic disease, including coping with the side effects of some of the drugs prescribed to control the disease. Readers are encouraged to educate themselves about the disease and to take an active part in their own health care team.
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Treatment of Lupus Nephritis. Seminars in Nephrology. 20(3): 265-276. May 2000.
Patients with lupus nephritis pose a therapeutic challenge and stimulate investigation of innovative treatment strategies. This article reviews those current and potential strategies that may optimize management of lupus nephritis. The clinical presentations of lupus nephritis can vary from asymptomatic hematuria (blood in the urine) or proteinuria (protein in the urine) to acute nephritic or nephrotic syndromes and from rapidly progressive glomerulonephritis to insidious chronic renal insufficiency. Although patient survival and renal function outcomes have improved over the last 4 decades, contemporary immunosuppressive regimens are not consistently effective and often require extended courses (resulting in negative drug effects and toxicity). Several strategies are under investigation to induce remissions more rapidly and to reduce the risk of long courses of cytotoxic drug therapy. The combination of pulse methylprednisolone and pulse cyclophosphamide may be more effective than pulse cyclophosphamide alone for patients with relatively severe proliferative lupus nephritis. A particularly vigorous strategy employs immunoablative cyclophosphamide, with or without stem cell rescue. Several studies of sequential immunosuppressive therapy are in progress. It is anticipated that long term toxicities can be lessened by substituting various maintenance agents (e.g., azathioprine or mycophenolate mofetil) after initial cyclophosphamide therapy has induced a renal responses. Innovative approaches (e.g., costimulatory blockade) offer the hope of more effective treatments without the risks of contemporary regimens. 2 figures. 2 tables. 88 references.
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Treatment of Lupus Nephritis: A Work in Progress (editorial). New England Journal of Medicine. 343(16): 1182-1183. October 19, 2000.
Until the pathogenesis (development of disease state) of nephritis (kidney infection) due to systemic lupus erythematosus (SLE) is unraveled, optimal treatment for patients with this disease remains an elusive goal. This article outlines one option for treatment of lupus nephritis, serving as an introduction to a separate article in this issue of the Journal. The author first reviews the differing presentations of SLE, noting that in some patients the kidneys are not involved but in others, there is rapidly progressive destructive kidney disease. This difference may be due in part to genetic risk factors, to environmental factors (such as exposure to ultraviolet light, infectious pathogens, and silica dust), race, or socioeconomic factors. In general, the treatment of lupus glomerulonephritis depends on the severity of the disease. Intravenous cyclophosphamide is given, in addition to oral glucocorticoids, for the aggressive forms of the disorder. However, the adverse effects of these therapies have prompted the search for alternative treatments. The author then comments on the accompanying article which presents the results of a study in which patients with diffuse proliferative lupus nephritis were successfully treated with prednisolone and mycophenolate mofetil. The editorial author notes that there are several reasons for caution before generalizing these findings to other patients with proliferative lupus glomerulonephritis, notably underrepresentation of patients with poor prognosis and certain demographic characteristics. 10 references.
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