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Lupus Nephritis

Alternative Names: Nephritis – lupus; Lupus glomerular disease

Definition:
Lupus nephritis is an inflammation of the kidney caused by systemic lupus erythematosus (SLE), a disease of the immune system. Apart from the kidneys, SLE can also damage the skin, joints, nervous system and virtually any organ or system in the body.

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Symptoms:
General symptoms of lupus include malar rash, discoid rash, photosensitivity, oral ulcers, nonerosive arthritis, pleuropericarditis, renal disease, neurological manifestations, and haematological disorders.

Clinically, SLE usually presents with fever, weight loss (100%), arthralgias, synovitis, arthritis (95%), pleuritis, pericarditis (80%), malar facial rash, photodermatosis, alopecia (75%), anaemia, leukopaenia, thrombocytopaenia, and thromboses (50%).

About half of cases of SLE demonstrate signs of lupus nephritis at one time or another. Renal-specific signs include proteinuria (100%), nephrotic syndrome (55%), granular casts (30%), red cell casts (10%), microhematuria (80%), macrohematuria (2%), reduced renal function (60%), RPGN (30%), ARF (2%), hypertension (35%), hyperkalemia (15%) and tubular abnormalities (70%).

The World Health Organization (WHO) developed a system to classify the six different stages of lupus nephritis:

Stage 1: no evidence of lupus nephritis:
In histology, Stage I (minimal mesangial) disease has a normal appearance under light microscopy, but mesangial deposits are visible under electron microscopy. At this stage urinalysis is typically normal.
Stage 2: mildest form, easily treated with corticosteroids:
Stage 2 disease (mesangial proliferative) is noted by mesangial hypercellularity and matrix expansion. Microscopic haematuria with or without proteinuria may be seen. Hypertension, nephrotic syndrome, and acute renal insufficiency are rare at this stage.
Stage 3: earliest stage of advanced lupus. Treatment requires high amounts of corticosteroids. The outlook remains favorable.

Stage 3 disease (focal lupus nephritis) is indicated by sclerotic lesions involving less than 50% of the glomeruli, which can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions. Under electron microscopy, subendothelial deposits are noted, and some mesangial changes may be present. Immunofluorescence reveals the so-called “Full House” stain, staining positively for IgG, IgA, IgM, C3, and C1q. Clinically, haematuria and proteinuria is present, with or without nephrotic syndrome, hypertension, and elevated serum creatinine.
Diffuse proliferative lupus nephritis; photo shows the classic “flea-bitten” appearance of the cortical surface in the diffuse proliferative glomerulonephritides

Stage 4: advanced stage of lupus.

Stage 4 lupus nephritis (diffuse proliferative) is both the most severe, and the most common subtype. More than 50% of glomeruli are involved. Lesions can be segmental or global, and active or chronic, with endocapillary or extracapillary proliferative lesions. Under electron microscopy, subendothelial deposits are noted, and some mesangial changes may be present. Immunofluorescence reveals the so-called “Full House” stain, staining positively for IgG, IgA, IgM, C3, and C1q. Clinically, haematuria and proteinuria are present, frequently with nephrotic syndrome, hypertension, hypocomplementemia, elevated anti-dsDNA titres and elevated serum creatinine. There is the risk of kidney failure. Patients require high amounts of corticosteroids and immune suppression medications.
A wire-loop lesion may be present in stage III and IV. This is a glomerular capillary loop with subendothelial immune complex deposition that is circumferential around the loop. Stage V is denoted by a uniformly thickened, eosinophilic basement membrane. Stage III and IV are differentiated only by the number of glomeruli involved (which is subject to inherent sample bias), but clinically the presentation and prognosis are both expected to be more severe in stage 4 versus stage 3.
Stage 5:
Stage 5 (membranous lupus nephritis) is characterized by diffuse thickening of the glomerular capillary wall (segmentally or globally), with diffuse membrane thickening, and subepithelial deposits seen under electron microscopy. Clinically, stage V presents with signs of nephrotic syndrome. Microscopic haematuria and hypertension may also been seen. Plasma creatinine is usually normal or slightly elevated, and stage V may not present with any other clinical/serological manifestations of SLE (complement levels may be normal; anti-DNA Ab may not be detectable). Stage 5 also predisposes the affected individual to thrombotic complications such as renal vein thromboses or pulmonary emboli.Excessive protein loss and swelling. Doctors will treat with high amounts of corticosteroids. Doctors may or may not give immune-suppressing drugs.
A final stage is usually included by most practitioners, stage VI, or advanced sclerosing lupus nephritis. It is represented by Global sclerosis involving more than 90% of glomeruli, and represents healing of prior inflammatory injury. Active glomerulonephritis is usually not present. This stage is characterised by slowly progressive renal dysfunction, with relatively bland urine sediment. Response to immunotherapy is usually poor.

A tubuloreticular inclusion is also characteristic of lupus nephritis, and can be seen under electron microscopy in all stages. It is not diagnostic however, as it exists in other conditions. It is thought to be due to chronic interferon exposure.
Causes:
Systemic lupus erythematosus (SLE, or lupus) is an autoimmune disease. This means there is a problem with the body’s immune system.

Normally, the immune system helps protect the body from infection or harmful substances. But in patients with an autoimmune disease, the immune system cannot tell the difference between harmful substances and healthy ones. As a result, the immune system attacks otherwise healthy cells and tissue.

SLE may damage different parts of the kidney, leading to interstitial nephritis, nephrotic syndrome, and membranous GN. It may rapidly worsen to kidney failure.

Lupus nephritis affects approximately 3 out of every 10,000 people. In children with SLE, about half will have some form or degree of kidney involvement.

More than half of patients have not had other symptoms of SLE when they are diagnosed with lupus nephritis.

SLE is most common in women ages 20 – 40.
Diagnosis:
The diagnosis of lupus nephritis depends on blood tests, urinalysis, X-rays, ultrasound scans of the kidneys, and a kidney biopsy. On urinalysis, a nephritic picture is found and RBC casts, RBCs and proteinuria is found.

The World Health Organization has divided lupus nephritis into five stages based on the biopsy. This classification was defined in 1982 and revised in 1995.

* Class I is minimal mesangial glomerulonephritis which is histologically normal on light microscopy but with mesangial deposits on electron microscopy. It constitutes about 5% of cases of lupus nephritis. Renal failure is very rare in this form.[2]

*Class II is based on a finding of mesangial proliferative lupus nephritis. This form typically responds completely to treatment with corticosteroids. It constitutes about 20% of cases.[2] Renal failure is rare in this form.[2]

* Class III is focal proliferative nephritis and often successfully responds to treatment with high doses of corticosteroids. It constitutes about 25% of cases. Renal failure is uncommon in this form.

*Class IV is diffuse proliferative nephritis. This form is mainly treated with corticosteroids and immunosuppressant drugs. It constitutes about 40% of cases. Renal failure is common in this form.

* Class V is membranous nephritis and is characterized by extreme edema and protein loss. It constitutes about 10% of cases.[2] Renal failure is uncommon in this form.

Medicines are prescribed that decrease swelling, lower blood pressure, and decrease inflammation by suppressing the immune system: Patients may need to monitor intake of protein, sodium, and potassium. Patients with severe disease should restrict their sodium intake to 2 grams per day and limit fluid as well. Depending on the histology, renal function and degree of proteinuria, patients may require steroid therapy or chemotherapy regimens such as cyclophosphamide, azathioprine, mycophenolate mofetil, or cyclosporine.
Possible Complications:

*Acute renal failure
*Chronic renal failure
*End-stage renal disease
*Nephrotic syndrome
Treatment:

There is no cure for lupus nephritis. The goal of treatment is to keep the problem from getting worse. Stopping kidney damage early can prevent the need for a kidney transplant.

Treatment can also provide relief from lupus symptoms.

Common treatments include:

*minimizing intake of protein and salt
*taking blood pressure medication
*using steroids to reduce swelling and inflammation
*taking immune-suppression medicines like prednisone to reduce immune system damage to the kidneys

Extensive kidney damage may require additional treatment.

Drug regimens prescribed for lupus nephritis include mycophenolate mofetil (MMF), intravenous cyclophosphamide with corticosteroids, and the immune suppressant azathioprine with corticosteroids. MMF and cyclophosphamide with corticosteroids are equally effective in achieving remission of the disease. MMF is safer than cyclophosphamide with corticosteroids, with less chance of causing ovarian failure, immune problems or hair loss. It also works better than azathioprine with corticosteroids for maintenance therapy.

Prognosis:  How well you do depends on the specific form of lupus nephritis. You may have flare-ups, and then times when you do not have any symptoms.

Some people with this condition develop chronic kidney failure.

Although lupus nephritis may return in a transplanted kidney, it rarely leads to end-stage kidney disease.
Prevention: There is no known prevention for lupus nephritis.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.
Resources:
http://en.wikipedia.org/wiki/Lupus_nephritis
http://www.nlm.nih.gov/medlineplus/ency/article/000481.htm
http://www.healthline.com/health/lupus-nephritis#Diagnosis3

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Categories
Ailmemts & Remedies

Nephritis

Definition:
Nephritis is inflammationof the nephrons of one or both of the kidneys – the organs that filter the blood and get rid of excess fluid and unwanted chemicals.  The inflammation can affect the kidneys’ function, including their ability to filter waste and this can be caused by many different conditions.

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Symptoms may develop as the disease gets worse, but as nephritis resolves completely in about 60 per cent of adults and as many as 90 per cent of children, for many it comes and goes with little disruption to their life.

The downside is that for those in whom the disease doesn’t get better and instead progresses into a more severe condition, advanced kidney (renal) failure may have developed before they have had any reason to seek medical help.

Types:
*Glomerulonephritis is inflammation of the glomeruli. (When the term “nephritis” is used without qualification, this is often the condition meant.)...CLICK & SEE

*Interstitial nephritis or tubulo-interstitial nephritis is inflammation of the spaces between renal tubules……CLICK & SEE

*Pyelonephritis is inflammation that results from a urinary tract infection that reaches the pyelum (pelvis) of the kidney…….CLICK & SEE

*Lupus nephritis is an inflammation of the kidney caused by systemic lupus erythematosus (SLE), a disease of the immune system....CLICK & SEE

Symptoms:
Symptoms of nephritis include:

•Swelling of the tissues (initially the face and around the eyes, later more prominent in the legs)
•Reduction in urine volume
•Dark urine (contains blood which may not be visible)
•Increase in blood pressure
•Headaches
•Drowsiness
•Visual disturbances
•Tiredness and general malaise (feeling ill)
•Nausea
•In rapidly progressive disease, loss of appetite, vomiting, abdominal pain and joint pain may occur
•Chronic nephritis may go unnoticed for years until symptoms of kidney failure appear: tiredness, itchy skin, nausea and vomiting, shortness of breath

About half of those who develop acute nephritis actually have no symptoms. If symptoms do develop, they point clearly to the problem. The inflammation causes blood and protein to leak into the urine. As protein levels in the blood fall, excess fluid accumulates in the body.

Tests show protein, blood cells, and kidney cells in the urine, while a high concentration of the body’s waste products of metabolism (such as urea and creatinine) may be found in the blood.

Swabs of the throat may show there’s been a streptococcal infection, while blood tests may be used to check for antibodies to streptococci or other infections, or signs of an abnormal immune response.

Sometimes a small biopsy or sample of tissue is taken from the kidney to examine in the laboratory.

Causes:
The causes of nephritis (or acute nephritic syndrome as the collection of symptoms is sometimes called) tend to be different in adults and children.

One of the commonest, especially in children, is after infection with the streptococcus bacteria, which leads to an immune reaction that damages the filtering units of the kidney known as the glomeruli. This condition is called post-streptococcal glomerulonephritis.

Other causes seen more frequently in children than adults include Henoch-Schönlein purpura (an inflammation of the blood vessels caused by an abnormal immune response) and haemolytic-uraemic syndrome (an abnormal immune reaction with triggers including gastrointestinal infection).

Risk Factors:
In adults, diseases that frequently underlie nephritis include vasculitis (inflammation of the blood vessels), pneumonia, abscesses, infections such as measles, mumps or glandular fever, hepatitis, and a range of different immune disorders that cause types of glomerulonephritis.

In more serious, rapidly progressive glomerulonephritis, about half of people remember having had a flu-like illness in the month before symptoms start.

Diagnosis:
Your doctor may suspect lupus nephritis if your urine is bloody or has a foamy appearance, if you have high blood pressure, or if you show signs of swelling in your hands or feet. Diagnostic tests for lupus nephritis  may include:

*Renal function testing.  Nephrologists may use a variety of tests, including blood tests and 24-hour urine collection, to accurately measure your kidney function. Iothalamate clearance testing, which uses a special contrast agent to track how well your kidneys are filtering, may be done if traditional tests don’t provide clear measurement of your kidney function.

*Kidney biopsy. Biopsy is the gold-standard test to confirm the diagnosis of many kidney diseases, including lupus nephritis. It can also help determine the severity of the disease. Because of the large number of people treated for kidney diseases.

Treatment :
The treatment of nephritis depends on the type and cause of the condition. The aim is to reduce inflammation, limit the damage to the kidneys and support the body until kidney function is back to normal.

Restriction of sodium (salt), potassium, protein and fluids in the diet may be necessary. Sometimes bed rest is advised. Steroids, or more powerful immunosuppressant drugs, may be given to reduce the inflammation.

Antibiotics may be needed too, although in many cases the infection that initially triggered the nephritis has long since gone. Medication may also be needed to control blood pressure.

In severe cases, renal dialysis may be necessary, although this may only be a temporary measure.

Adults are slower to recover than children and more likely to develop complications or progress into chronic nephritis. Acute nephritic syndrome is unlikely to recur, but if it does there’s at least a one in three chance that an adult will develop what is known as ‘end-stage kidney disease’, leaving them in need of permanent dialysis or a kidney transplant.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose

Resources:
http://en.wikipedia.org/wiki/Nephritis
http://www.bbc.co.uk/health/physical_health/conditions/nephritis1.shtml
http://www.mayoclinic.org/lupus-nephritis/diagnosis.html
http://commons.wikimedia.org/wiki/File:Diffuse_proliferative_lupus_nephritis.jpg

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