Poststreptococcal Glomerulonephritis

Nirav Dhruva Morning Report July 2005

Poststreptococcal GN (Introduction)

Usually occurs 10 days after pharyngitis and 14 days after skin infection (not synpharyngitic) Fallen incidence in US, but common in some rural areas, poor hygiene places, and tropical countries Occurs more often in males and children

Poststreptoccal GN (nephritic strains)

Known nephritic strains include M types 1, 2, 4, 12, 18, 25, 49, 55, 57, 60 Many proposed mechanisms: Molecular mimickry vs. autoimmune vs. polyclonal activation of B lymphocytes Repeat infections are not common as immunity is type specific and not usually transient

Clinical Presentation

Most patients have milder disease Classically, presents with overt nephritic syndrome and oliguric ARF Symptoms can include gross hematuria (100% microscopic), HA, htn (60-80%), hypervolemia, and edema (80-90%)

Clinical Presentation

Nephritic urinary sediment dysmorphic RBCs, red cell casts, leukocytes, subnephrotic proteinuria Nephrotic-range proteinuria not common

Labs

Serum Cr can be commonly elevated at presentation, though mild C3 and CH50 decreased w/in 2 weeks C4 usually normal (complement level usually normal within 68 weeks) Most patients have directed Ab, such as ASO, anti-DNAse B, etc Serum IgG and IgM increased in 80% and returns to normal in 1-2 months Polyclonal cryoglobulinemia in 75%

Light Microscopy
On light microscopy, usually see diffuse proliferative GN

Immunofluorescence Microscopy

Deposition of IgG and C3

3 patterns

mesangial

Starry sky (mesangial and capillary wall)

Garland (capillary loops)

Electron Microscopy

large electron dense immune deposits in subendothelial, subepithelial, and mesangial areas

Course

Irreversible Renal Failure rare less than 1 % in children, slightly higher in adults Resolution usually quick, plasma Cr usually returns to previous levels by 3-4 weeks Hematuria resolves usually within 3-6 months, proteinuria falls at a slower rate Some patients experience htn, recurrent proteinuria, and renal insufficiency 10-40 yrs after > 20% of adults may have some degree of persistent proteinuria and or compromise of GFR 1 year out

Treatment

Eliminate strep infxn with abx Supportive therapy Diuretics and antihypertensives to control bp and extracellular fluid volume

Acute GN and Pulmonary Hemorrhage

ANCA associated systemic vasculitis and antiGBM - common causes Also reported to occur in SLE, HSP, mixed Cryoglobulinemia Study of 88 patients with Pulmonary Hemorrhage and Nephritis: 48 ANCA, 6 Anti GBM, 7 both, 27 neither ANCA nor anti-GBM (latter group not many causes could explain both findings)

The End

References

Rose, Burton. Acute Glomerulonephritis and Pulmonary Hemorrhage, Course of Poststreptococcal Glomerulonephritis. Up To Date 2005. Harrisons Online. Glomerular Disease. Ch 264. 2004-2005. Niles JL, et al. The Syndrome of Lung Hemorrhage and Nephritis is Usually an ANCA Associated Condition. Arch Intern Med. 1996; 156: 440-445. Gallagher H, et al. Pulmonary Renal Syndrome: A 4 Year, SingleCenter Experience. Am J of Kidney Disease 2002; 39: 42-47. Berrrios X, et al. Post-streptococcal Acute Glomerulonephritis in Chile 20 Years of Experience. Pediatr Nephrol 2004; 19: 306312. Johnson RJ, et al. Glomerular Disease Associated with Infection. Ch 30. Comprehensive Clinical Nephrology. 2000.