Approximately 1.5 million Americans are living with Systemic Lupus erythematosus in the United States.
Lupus is an autoimmune disease that causes your immune system to produce proteins called autoantibodies to attack your own tissues and organs, including kidneys.
It is one of the most serious complications of lupus.
It is estimated by the Lupus Foundation of America that up to 60% of lupus patients will develop lupus nephritis and up to 2/3 of children with lupus will develop nephritis.
What does the kidney do?
- Aids in the removal of waste and toxins from the body
- Has hormones like renin and aldosterone to regulate blood pressure and volume
- Ensures that the right amount of fluids exist throughout the body
How Does Nephritis develop?
Nephritis occurs when lupus autoantibodies affect structures in your kidney that filter out waste and causes kidney inflammation. There are immune complexes (antibody plus foreign agent that antibody is attacking) that form and deposit in the kidneys.
One part of the kidney that is affected by kidney disease is the glomerulus which is a network of tubules where blood, waste and toxins are filtered. The glomerulus has mesangial cells that assist in the filtering mechanism. In lupus nephritis, the blood vessels in the glomerulus allow the immune complexes to enter the kidney.
The kidneys start to leak protein and have loss of function when the glomeruli are inflamed.
If not controlled lupus nephritis can lead to kidney failure or dialysis.
Who is more at risk for Lupus and Nephritis?
According to the Lupus Foundation of America, Africans and Asian American women as well as other groups of women of color (Hispanics/Latinos, Native Americans, Native Hawaiians, and Pacific Islanders are 2-3 times more likely to develop lupus and complications than Caucasian.
Blacks, Hispanics/Latinos, Asian Americans are more likely to get nephritis.
There is a suggestion of genetic cause. 90 percent of individuals living with lupus are female and it mostly affects women of childbearing age.
People who have lupus nephritis are at a high risk for cancer called B cell lymphoma, a type of cancer. They are also at a high risk for heart and blood vessel problems.
Six types/stages of kidney disease- WHO classification
Class 1- Minimal mesangial glomerulonephritis.
5% of Lupus nephritis cases.
Class 2- Mesangial proliferative glomerulonephritis
20% of lupus nephritis cases.
There is excessive mesangial cells in a small area.
Treatment: Steroids. Kidney failure is rare
Class 3 – Focal Glomerulonephritis
25% of lupus cases.
There are active lesions in less than <50% of glomeruli and usually focused in 1-2 areas.
Sometimes patients present with blood and/or excessive protein in urine.
Treatment: Steroids (High doses).
Class 4: Diffuse Proliferative Nephritis (DPGN)
40% of Lupus cases are class 4. Very severe subtype.
More than >50% of the glomeruli are affected with active lesions and immune complex deposits that can be seen under microscope.
HEMATURIA and PROTEINURIA are present. High blood pressure can be seen, kidney function may be altered and there may ne rising autoantibody titers (dsDNA ab).
TREATMENT: STEROIDS and immunosuppressant. Kidney failure is common.
Class 5: Membranous GN
10% of Lupus cases
EXTREME swelling and proteinuria.
Patients may experience clots or thrombosis in the kidney blood vessels, a pulmonary embolism or other thrombotic complications.
TREATMENT: Steroids and immunosuppressants.
Class 6: Advanced sclerotic glomerulonephritis
Global sclerosis- There is scarring in a significant portion of the glomeruli.
Progressively worse kidney function can occur.
Between 10 to 30 percent of people who have lupus nephritis develop kidney failure
SYMPTOMS AND SIGNS TO LOOK OUT FOR:
- FOAMY urine (excess protein)
- Blood in the urine, high RBC on urine dipstick and urinalysis
- Dark urine
- Changes in frequency of urination
- Water retention or weight gain throughout the body, legs and hands, ankles
- High blood pressure
- Blood work
- (Check levels of protein, hemoglobin, cholesterol, BUN/creatinine (kidney function), ANA antibodies, dsDNA ab, antiphospholipid antibodies, complements, parathyroid hormone, electrolytes, vitamin D, etc)
- Lab: High levels of waste product, creatinine may be found in your blood
- Urinalysis (24-hour protein, spot urine protein/creatinine ratio)
- Imaging- renal ultrasound or CT
- Kidney Biopsy
1) Reduce symptoms or make symptoms disappear and go into remission
2) Keep disease from getting progressing or getting worse
3) Maintain remission once achieved
4) Avoid need for dialysis from progressive kidney failure
- Induction therapy helps to get to remission
- Steroids (High dose or pulse steroids may be needed in some cases)
- Cyclophosphamide (Cytoxan)- Be CAUTIOUS of risk of infections, infertility, bladder irritation (hemorrhagic cystitis), leukemia/lymphoma and bladder cancer
- Azathioprine (Imuran)
- Mycophenolate (Cellcept)
- Plaquenil will help with controlling Lupus disease activity
- Benlysta- Human monoclonal ab that binds to soluble BLYS, inhibiting its binding to B cell receptors. The result is a reduction in B cell population and decreases the production of autoantibodies. In 2019 there have been positive results in studies allowing improvement in disease, reduction in steroid use, and maintenance.
- Cellcept and Cytoxan have similar efficacy in treating Nephritis as short term induction therapy.
- In research studies, Blacks and Hispanics seem to respond better to Cellcept.
Medications to lower blood pressure (ACE inhibitors, ARBs, diuretics, calcium channel blockers)
Diuretics help with removing fluid from the body
MMF and CYC have similar efficacy as short term induction therapy.
Blacks and Hispanics seem to respond better to MMF.
Sometimes dialysis is required or kidney transplant like Selena Gomez, the celebrity actress and singer has openly discussed. An individual with a transplant will need additional drugs to keep immune system from rejecting the transplanted kidneys.
People who have lupus nephritis are at a high risk for cancer called B cell lymphoma,
a type of cancer that begins in the cells of the immune system.
They are also at a high risk for heart and blood vessel problems.
- Drink plenty of fluids and water to stay hydrated.
- Eat low sodium and protein diet especially with a Hypertension diagnosis
- AVOID smoking and alcohol
- Exercise regularly
- Maintain healthy blood pressure
- Lower cholesterol
- Avoid NSAIDs (Motrin, Naproxen) or other medications that affect the kidneys