SYSTEMIC LUPUS
The “classic” autoimmune disease
Statistics
Females are more at risk then males
Ratio of female to male risk is 10:1
Anyone can be affected
Many Organ Systems InvolvedSkinJointsNervous systemLungBloodKidney*Heart
The Kidney
Filters bloodExcretes wasteManages body chemicals - Sodium (salt), Potassium, Calcium
Manages acid state of bodyBlood PressureKeeps protein from leaking out
The Kidney
The kidney has high blood flow rates, prone to immune damage
This will cause inflammation and possibly scarring
Kidney Involvement When:
Body swellingHigh blood pressureBlood in urineAbnormal body chemistry--too much acid, too much water, abnormal potassium, etc.
Foamy urineDecreased kidney function
Both Kidneys Always InvolvedFillers (Glomeruli) and support structures are involved
Therefore called Glomerulonephritis
Recurrent urinary tract infections can be misdiagnosed as urinary infection when really inflammation
Will manifest with blood in urine (hematuria), and/or protein in urine (proteinuria)
If more then 3.5g of protein in 24hrs, we call this Nephrotic Syndrome
Blood proteins can drop, cholesterol and blood pressure can rise, and the kidney can scar
Best Way To Access:
Urine examKidney function: CreatinineCholesterolProtein levelsBlood pressureMarkers of inflammation
Markers of inflammation or immune function:
ANA double strand DNAC3C4ESRCRP
Kidney biopsy is the best way to diagnosisand determine treatment of Lupus Nephritis.
It tells the degree of inflammation and scarring present.
WHO Classifications - biopsy
I
Normal
II
Mesangial
III
Focal Proliferative
IV
Diffuse Proliferative
V
Memberanous
VI
Scarred
If no scarring, then treat all:InflammationHigh Blood PressureSalt RetentionHigh Cholesterol
These treatments have been worked out by enrolling patients into well designed studies, where they have been treated ethically and with the utmost care.
Control blood pressure to normal level 120/80
Use ACE inhibitor and/or ARBThese also decrease protein leakage
PROTEIN SPILLAGE CAUSES KIDNEY SCARRING!!!
Lower cholesterol with statins
Get rid of salt and lower blood pressure with diuretics
We usually use all of these methods in concert
Decreasing inflammation will decrease scarring
Cortisone-Prednisone is base of regimen
Cyclophosphamide very critical - worked out through large studies at NIH
Treatments
Azathioprine-weakerMycophenalate- “Cellcept”CyclosporinePrografRituxiumab
Treat Before Scarring
Once scarring occurs, cannot reverse injury
One should not prophylactically treat patients without kidney disease who have lupus
By lowering blood pressure, cholesterol, etc., scarring can be decreased and non renal complications lessened.
How to Determine Which Medication to Administer
Use studies that compare and contrast
Determine if one treatment is better for a certain group of patients
Get involved with trials
YOU ARE NOT A GUINEA PIG!!!
Lupus has become a very treatable disease. Only good studies can make it more treatable.
THANK YOU
Remember, being involved in good studies will help lupus therapy for you and
everyone.