Clinical features and therapy of lupus membranous nephropathy
- Ronald J Falk, MD
Ronald J Falk, MD
- Nan and Hugh Cullman Eminent Professor of Nephrology
- Chair, Department of Medicine
- Director, UNC Kidney Center
- University of North Carolina-Chapel Hill
- Maria Dall'Era, MD
Maria Dall'Era, MD
- Professor of Medicine
- University of California, San Francisco
- Gerald B Appel, MD
Gerald B Appel, MD
- Section Editor — Glomerular Diseases
- Professor of Medicine
- Columbia University College of Physicians and Surgeons
- Section Editors
- Richard J Glassock, MD, MACP
Richard J Glassock, MD, MACP
- Editor-in-Chief — Nephrology
- Section Editor — Glomerular Diseases
- Emeritus Professor
- The David Geffen School of Medicine at UCLA
- Brad H Rovin, MD
Brad H Rovin, MD
- Section Editor — Glomerular Diseases
- Professor of Medicine and Pathology
- The Ohio State University College of Medicine
The optimal treatment of lupus nephritis (LN) varies with the classification of the morphological findings present on renal biopsy. Ten to 20 percent of patients with LN have pure membranous (class V) LN [1-3], although some reports suggest a higher percentage among African-American populations . Treatment is indicated for patients with lupus membranous nephropathy, especially those with a severe (symptomatic) nephrotic syndrome, an elevated or rising serum creatinine, and/or concomitant focal or diffuse proliferative changes on renal biopsy.
The clinical features and treatment of lupus membranous nephropathy will be reviewed here. The various types of LN, the treatment of focal and diffuse proliferative LN, and management of the patient with end-stage renal disease are presented separately. (See "Diagnosis and classification of renal disease in systemic lupus erythematosus" and "Therapy of diffuse or focal proliferative lupus nephritis" and "End-stage renal disease due to lupus nephritis".)
Lupus membranous nephropathy is present in 10 to 20 percent of patients with lupus nephritis (LN) [5-7]. Affected patients typically present with proteinuria (often but not always in the nephrotic range) and a normal or only slightly elevated serum creatinine [8-10]. In addition, patients with lupus membranous nephropathy frequently have microscopic hematuria, and some may have nephrotic syndrome (with hypoalbuminemia and edema) and hypertension.
The presence of significant hematuria and cellular casts with or without an elevated serum creatinine suggests concurrent proliferative disease, which is not uncommon and is associated with a much worse prognosis than class V alone. In a review of 79 patients with lupus membranous nephropathy, 36 had pure membranous lesions, 15 had endocapillary proliferation and/or necrosis in less than 50 percent of glomeruli, and 28 had endocapillary proliferation and/or necrosis in more than 50 percent of glomeruli . (See 'Prognosis' below and 'Pathology' below.)
Extrarenal or serologic manifestations of systemic lupus erythematosus (SLE) frequently precede but can occasionally develop after the onset of kidney disease. Lupus membranous nephropathy is the one form of LN that may present with few or no other clinical or serologic manifestations of SLE (eg, complement levels may be normal, and anti-double-stranded DNA antibodies, which are highly specific for SLE, may not be elevated) [6,8,11-13]. In a randomized trial of 42 patients with lupus membranous nephropathy described below, low C3, low C4, and anti-double-stranded DNA antibodies were present in 3, 3, and 9 patients, respectively (7, 7, and 21 percent, respectively) . Thus, patients without extrarenal or serologic manifestations of SLE who have a renal biopsy suggesting lupus membranous nephropathy (including mesangial deposits and tubuloreticular inclusions) should be regarded as having LN and are at risk of subsequently developing SLE. (See "Overview of the clinical manifestations of systemic lupus erythematosus in adults" and "Diagnosis and differential diagnosis of systemic lupus erythematosus in adults".)
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- CLINICAL PRESENTATION
- NONIMMUNOSUPPRESSIVE THERAPY
- Angiotensin inhibition
- Goal blood pressure
- Lipid lowering
- IMMUNOSUPPRESSIVE THERAPY
- Indications for immunosuppressive therapy
- - Nephrotic syndrome
- - Increased serum creatinine
- - Concurrent proliferative lupus nephritis
- - Other patients
- Choice of immunosuppressive therapy
- Monitoring and follow-up
- - Repeat renal biopsy
- Nonresponders and relapsing disease
- Combined membranous and diffuse proliferative lupus nephritis
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS