you more detailed understanding of lupus nephritis

lupus nephritis

Systemic lupus erythematosus (SLE), or lupus, is an autoimmune disease that occurs when the body's immune system mistakenly attacks its own tissues. Some symptoms include rashes, joint pain, and fatigue.

Lupus nephritis is histologically evident in most patients with systemic lupus erythematosus (SLE), even those without clinical manifestations of renal disease. Evaluating renal function in SLE patients is important because early detection and treatment of renal involvement can significantly improve renal outcome.

Signs and symptoms
Patients with lupus nephritis may report the following:

Other symptoms of active SLE (eg, fatigue, fever, rash, arthritis, serositis, or central nervous system [CNS] disease); these are more common with focal proliferative and diffuse proliferative lupus nephritis
Asymptomatic lupus nephritis – During regular follow-up, laboratory abnormalities suggest active lupus nephritis; this is more typical of mesangial or membranous lupus nephritis
Active nephritis – Peripheral edema secondary to hypertension or hypoalbuminemia; extreme peripheral edema is more common with diffuse or membranous lupus nephritis
Diffuse lupus nephritis – Headache, dizziness, visual disturbances, or signs of cardiac decompensation
Physical findings may include the following:

Focal and diffuse lupus nephritis – Generalized active SLE with the presence of a rash, oral or nasal ulcers, synovitis, or serositis; signs of active nephritis
Active lupus nephritis – Hypertension, peripheral edema, and, occasionally, cardiac decompensation
Membranous lupus nephritis – Peripheral edema, ascites, and pleural and pericardial effusions without hypertension
See Presentation for more detail.

Diagnosis
Laboratory tests to evaluate renal function in SLE patients include the following:

Blood urea nitrogen (BUN) testing
Serum creatinine assessment
Urinalysis (to check for protein, red blood cells [RBCs], and cellular casts)
Spot urine test for creatinine and protein concentration
24-hour urine test for creatinine clearance and protein excretion

Lupus nephritis is staged according to the classification revised by the International Society of Nephrology (ISN) and the Renal Pathology Society (RPS) in 2003, as follows:

Class I – Minimal mesangial lupus nephritis
Class II – Mesangial proliferative lupus nephritis
Class III – Focal lupus nephritis (active and chronic; proliferative and sclerosing)
Class IV – Diffuse lupus nephritis (active and chronic; proliferative and sclerosing; segmental and global)
Class V – Membranous lupus nephritis
Class VI – Advanced sclerosis lupus nephritis
See Workup for more detail.

Management
The principal goal of therapy in lupus nephritis is to normalize renal function or, at least, to prevent the progressive loss of renal function. Therapy differs, depending on the pathologic lesion.

Key points of American College of Rheumatology guidelines for managing lupus nephritis are as follows:

Patients with clinical evidence of active, previously untreated lupus nephritis should have a renal biopsy to classify the disease according to ISN/RPS criteria
All patients with lupus nephritis should receive background therapy with hydroxychloroquine, unless contraindicated
Glucocorticoids plus either cyclophosphamide intravenously or mycophenolate mofetil orally should be administered to patients with class III/IV disease; patients with class I/II nephritis do not require immunosuppressive therapy
Angiotensin-converting enzyme inhibitors or angiotensin-receptor blockers should be administered if proteinuria reaches or exceeds 0.5 g/day
Blood pressure should be maintained at or below 130/80 mm Hg
Patients with class V lupus nephritis are generally treated with prednisone for 1-3 months, followed by tapering for 1-2 years if a response occurs. If no response occurs, the drug is discontinued. Immunosuppressive drugs are generally not used unless renal function worsens or a proliferative component is present on renal biopsy samples.

About half the people who have systemic lupus erythematosus develop some form of kidney inflammation, called lupus nephritis. This inflammation can lead to kidney failure, but the course of the lupus and the pattern of its effects on the kidneys is quite variable and hard to predict.

According to the guidelines, a kidney biopsy is a consideration in all people with lupus who show signs of active kidney involvement.

"With earlier diagnosis and treatment, we really minimize the damage caused by lupus nephritis," says Joan T. Merrill, MD. She is the medical director of the Lupus Foundation of America and one of the authors of the new set of guidelines.

This is important as early kidney involvement is often silent, says Merrill. "Doctors should be watching the urine and seeing if there is any evidence of kidney disease. Theoretically, we should be able to pick up kidney involvement before it gets symptomatic." Merrill is also a professor of medicine at the University of Oklahoma Health Sciences Center in Oklahoma City.

Biopsy is the best way to identify kidney disease early, says Sandra C. Raymond, the president and CEO of the Lupus Foundation of America in Washington D.C. "It is the quintessential diagnosis tool for lupus nephritis. They can tell whether it is lupus or not that is causing the symptoms."

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