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Nephritic Syndrome Causes (Small Vessels Vasculitis (Churg-Strauss…
Nephritic Syndrome Causes
PSGN
Associations
Weeks after GAS infxn
Pharyngitis - 1-2 weeks
Skin - 3-4 weeks
Children 3-12yo
Can lead to RPGN
Findings
positive strepto AB (ASO, ADB)
↓ serum C3
"cola-colored" urine
LM: hypercellular
IM: "lumpy-bumpy"
EM: "humps"
TX
Self-limiting
Supportive
IgA Nephropathy
Associations
Most common worldwide
episodic gross hematuria after infxn (URT or GI)
♂ >♀
aprox. 25-30% progress to ESRD
Findings
assymp. micro-hematuria/intermitent gross hematuria
↑ serum IgA
Normal C3 levels
LM: mesangial proliferation
IM: mesangial IgA deposits
EM: mesangial immune-complex deposits
Tx
Supportive
Corticoids in severe cases
Small Vessels Vasculitis
Wegener's
Pulmonary and nasopharyngeal involvement is common
c-ANCA
Bx: segmental necrotizing GN
Microscopic polyangiitis
p-ANCA
Churg-Strauss
Presentation
Ashtma, allergic rhinitis, purpura...
p-ANCA (50%)
peripheral eosinophilia
Focal segmental necrotizing GN
Tx
Supportive
Immunosupresive tx
Goodpasture Sd
ABs agains collagen IV of BM
can cross react with alveoli
Pulmonary hemorrhage
Can lead to RPGN
Findings
Pulmonary infiltrates on CXR
IM: linear deposition of IgG along the glomerular BM
Tx
Immunosupressive
Plasmapheresis
Alport Sd
Associations
X-linked (usually males)
Mutation in gene for type IV collagen
Often leads to ESRD
Findings
Micro//macro-hematuria
Hearing loss and abdnormalities of the eyes
EM: splitting and alternating thickening and thining of GBM (basket-wave)
Tx
Supportive
Renal transplant can lead to Goodpasture Sd
Lupus Nephritis
Can lead to RPGN in some cases
Findings
ANA, anti-dsDNA
Histologic changes are very variable
Tx
Supportive
Immunosupressive
RPGN
Progress to ESRD in weeks-months
Findings
Can be caused by a variety of diseases (Goodpasture, PSGN, SLE, Microscopic poliangiitis...)
LM, IM, EM:
crescent formation
, monocytes, macrophages