Lupus and Kidney disease..What is Lupus Nephritis?

Lupus nephritis
http://www.kidneypathology.com/English_version/Case_8.html

Lupus nephritis an ominous name for a condition that most people would not have heard of. Chances are if you know the entity to which I am referring you either have this disease or know someone who does.

Lupus nephritis is the name given to inflammation of the kidney because of lupus. There are several flavors of Lupus nephritis and they are graded from 1 to 6 based on testing of kidney tissues. The prognosis of lupus nephritis is tied to the class of the disease. With Class I disease having the best prognosis while class 4 and 5 disease and combinations thereof having the worst overall prognosis.

Estimates are that up to 50% of patients who have lupus will have some manifestation of lupus nephritis over the course of their illness. To understand Lupus Nephritis we must first understand some of the key points surrounding lupus itself.

HOW DOES LUPUS OCCUR

Systemic lupus is an autoimmune disease.

The native immune system attacks normal tissue as it would a foreign invading organism.

The reason for this is not yet fully known.

Majority of problems are related to INFLAMMATION within the tissues affected.

The kidney is very vulnerable to inflammation

The organ receives a very high blood flow

Inflammatory cells are easily transported through the blood to the organ.

The consequences of this inflammation, results in several distinct syndromes.

Organ involvement in Lupus

SYNDROMES ASSOCIATED WITH LUPUS NEPHRITIS

Nephrotic syndrome.

Nephritic syndrome.

Acute renal failure

Chronic renal failure

Nephrotic syndrome

A constellation of symptoms and signs which include swelling and passage of high level of protein in the urine associated with high levels of cholesterol.

The nephritic syndrome

The presence of either visible or microscopic amount of blood in the urine in conjunction with elevated blood pressure and fluid retention. There is also usually some retention of waste products in the blood on screening tests of kidney function

Acute renal failure

A sudden decrease in the ability of the kidney to perform its main functions

The excretion of nitrogenous waste products.

The excretion of excess acid.

The excretion of excess fluids.

[caption id="attachment_597" align="alignright" width="300" caption="Lupus antibodies NLM ADAM "][/caption]
Chronic renal failure

This is better termed chronic kidney disease.

A gradual loss of renal function taking place over 3 months or more.

Symptoms of renal failure may be present

Weakness

Lethargy

Fatigue

Vomiting

Fluid retention

Loss of appetite.

If the diagnosis is suspected by your physician it may be confirmed only by a series of tests.

• Measurement of electrolytes and urea and creatinine.


• Measurement of creatinine clearance.


• Measurement of 24 hour protein excretion.


• Analysis of the urine sediment.


• General investigations targetted at the activity of systemic lupus.


• dsDna antibody.


• Complement levels.


• CRP and ESR.

Based on the history and clinical findings plus the results of investigations so far


A clinical syndrome may be diagnosed

• Nephrotic syndrome.


• Nephritic syndrome.


• Acute renal failure.


• Chronic renal failure.

The treatment of lupus nephritis however depends on The HISTOLOGICAL CLASS of disease and the CLINICAL SYNDROME.

Histology is the study of tissues, usually carried out under the microscope.
Samples of tissue are obtained by a procedure known as biopsy. Biopsy may be

1. Open biopsy requiring general anaesthesia and major surgery.


2. Closed biopsy usually done under ultrasound guidance.

CLASS I

Essentially normal.

CLASS II (mesangial)

A localised Mild increase in the cells seen.

CLASS III (focal proliferative)

A diffuse or widespread increase in cells of immune origin and kidney origin. But large areas of normal or mild disease still apparent.
CLASS IV (diffuse proliferative).

As above but almost no areas of mild disease or normal kidney tissue on biopsy.

CLASS V (membranous)

May or may not have an increased number of cells. However there are deposits of abnormal protein apparent within the kidney.

CLASS VI (sclerosing).

Scarring of the kidney usually results when the above classes have progressed beyond the ability to respond to treatment.

TREATMENT OF LUPUS NEPHRITIS

Class I disease
Usually no treatment above what is required to maintain lupus in remission.

Class II disease
May require treatment with increased doses of steroids. Usually responds promptly to therapy and is easily treated.

Class III disease
May behave like Class II disease or Class IV disease.

Class IV disease
Aggressive disease that easily and rapidly progresses without aggressive treatment.

Class V disease.
Pure Class V disease is controversial.
Class V disease with features of class IV is treated as IV
Class V disease with features of III is treated as III or IV.

Class VI disease
Prevent further deterioration of renal function and if signs of chronic renal failure are also present then prepare for dialysis.

• The NIH has the most complete follow up data for severe lupus nephritis defined as Class IV And Class V with features of Class IV.

• Their data suggested that survival with Cyclophosphamide was 90 percent at 10 years.


• With Azathioprine alone 60 percent at 10 years.


• Prednisone alone 20 percent at 10 years.

New treatment options for therapy with lupus are being studied daily.