1. IgA Nephropathy
Southwest Nephrology Symposium
February 24th 2018

2. Nephron Functional unit in the kidney
There are about 1 million nephrons in each human kidney
Nephrons remove excess water, wastes and other substances from your blood into urine
Each nephron is composed of two main structures. The glomerulus and kidney tubule

3. Nephron

4. Renal Corpuscle

6. Glomerulus Tiny blood vessel or capillary Looks like a ball of yarn
Actual filtering of blood occurs in the glomerulus
Keeps normal protein and cells in your bloodstream
Allows wastes, excess fluid and other substances to pass

7. Nephron

8. Glomerulonephritis Acute inflammation of the kidney
Typically caused by an immune response

9. Immunoglobulins Also known as antibodies
Antibodies are substances made by body’s immune system in response to bacteria, viruses, animal dander or cancer cells
There are five major types
IgA, IgG, IgM, IgE, IgD

10. IgA Nephropathy
First described by pathologist Jean Berger and so also called Berger’s Disease

11. IgA Nephropathy
Immune complex disease in which IgA deposits in the glomerular mesangium and cause mesangial injury
Most common glomerulonephritis in the western world

12. Henoch-Schonlein purpura
Small vessel vasculitis
Defined by tissue deposition of IgA
Involves the skin, gut and kidneys
Difference from IgA nephropathy is that it has extra renal manifestations

13. IgA Immunoglobulin A is an antibody
Plays critical role in immune function in the mucus membranes

14. IgA nephropathy clinical features
Gross hematuria usually following upper respiratory infection 40-50%
Microscopic hematuria and mild proteinuria 30-40%
Nephrotic syndrome or rapidly progressive glomerulonephritis characterized by edema, hypertension and renal insufficiency as well as hematuria in less than 10%
More common in Asians and Caucasians and Native Americans
2:1 Male to female perdominance in Caucasians but not Asians
20-40% develop ESRD 20 years after diagnosis is made

15. Clinical Associations
Cirrhosis
Celiac disease ( Gluten enteropathy)
HIV infection

16. Urine Analysis Dipstick: Blood and Protein
Microscopic exam: RBC, RBC casts or dysmorphic RBC

17. RBC Cast

18. Dysmorphic RBC

19. Kidney Biopsy

20. Core Biopsy Specimen

21. Clinical predictors of progression
Elevated serum creatinine
Hypertension > 140/90 mm Hg or new difficult to control BP
Proteinuria more than 1 gram per day

22. Histological predictors of progression
Mesangial hyprecellularity
Endocapillary hyprecellularity
Segmental glomerulosclerosis
Tubular atrophy and interstitial fibrosis
Crescents
( Minimum 8 glomeruli needed for a good diagnosis)

23. Treatment Non immunosuppressive
High BP control: ACE-I or ARB
Omega-3 fatty acids
Statin therapy: Cholesterol lowering medications

24. Treatment Immunosuppressive
Steroids
Cytoxan
Imuran

25. ESRD due to IgA Nephropathy
Renal replacement therapy: Hemodialysis or Peritoneal Dialysis
Kidney Transplantation

26. Transplantation
60 % of transplant patients show recurrence of IgA nephropathy by transplant biopsy but clinical significance is not that high