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Pathophys and Cause ⬇

Cause:Idiopathic type probably autoimmune, caused by IgG antibodies to PLA2R glycoprotein in glomerular podocytes (Nejm 2009;361:11, 81), hepatitis B (Nejm 1991;324:1457; Ann IM 1989;111:479), captopril use, NSAID use (Jama 1996;276:466), malignancy (Am J Kidn Dis 1993;22:5)

Pathophys:Increased BM thickness in glomerular tuft by electron and light microscopy

Epidemiology ⬆ ⬇

Primarily in adults

Signs and Symptoms ⬆ ⬇

Sx:Few, feel well, insidious onset

Si:of nephrotic syndrome (Nephrotic Syndrome)

Course ⬆ ⬇

80% 5-yr survival

Complications ⬆ ⬇

Nephrotic syndrome; renal failure

Lab and Xray ⬆ ⬇

Lab:

Path:Renal biopsy shows increased BM thickness on light microscopy, espwith PAS stain, and on electron microscopy

Urine:Hematuria (40%)

Treatment ⬆

Rx:

No rx beyond diuretics, lipid lowering, and antihypertensives justified; no renal failure in 88% after 5 yr, 73% after 8 yr, and 65% recover in 5 yr (Nejm 1993;329:85)

Steroids and cytotoxic drug roles controversial, usually reserved for pts w 24-h urine protein >10 gm, and/or elevated but not progressive creatinine elevations; methylprednisolone, or alternate with chlorambucil q 1 mo × 6 mo (Nejm 1992;327:599); or cyclophosphamide + prednisone × 1 yr (Ann IM 1991;114:725); or prednisone alone as good (Ann IM 1992;116:438)

In hepatitis B glomerulonephritis, image-interferon (very expensive) for 4 mo (Ann IM 1989;111:479), although recurs off rx and side effects substantial