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Dr. srikanth, M.D.

Previous lecture??

Nephritic syndromes
Discussed as three entities Acute nephritis- days to weeks Rapidly progressive glomerlonephritis- weeks

to months Chronic glomerulonephritis- months to years


Acute nephritis/RPGN
Immune complex GN

GN Group A beta-hemolytic streptococci (pharyngitis or impetigo), primarily in children Lupus nephritis Cryoglobulinemia -hepatitis C infection Bacterial endocarditis IgA nephropathy & Henoch-Schnlein purpura (HSP). Anti GBM/Goodpastures syndrome Circulating antibodies against type 4 collagen that damages the GBM and causes RPGN


Pauci-immune GN
Idiopathic Microscopic polyangiitis Wegeners granulomatosis Renal limited crescentic glomerulonephritis

Chronic nephritis
Can be a consequence of virtually all of the major glomerulopathies

Signs and symptoms

Acute nephritis
Oliguria (< 400 mL/day of urine) over days to

weeks Extracellular fluid volume expansion, edema, and hypertension RPGN Renal failure develops over weeks to months in association with variable oliguria, hypervolemia, edema, and hypertension.

Oliguri a

Chronic nephritis
Often asymptomatic. Diagnosis typically is made in one of the following

circumstances: Evaluation for secondary causes of hypertension Routine urinalysis Evaluation of unexplained anemia, blood urea and creatinine Following discovery of bilateral small kidneys on abdominal imaging During a clinical exacerbation of GN triggered by pharyngitis or other infections

What are the important ones??

Post streptococcal Lupus Bacterial endocarditis Cryogloblinemia Wegeners Anti GBM disease

Can you classify them based on immune complexes??

Post streptococcal
Poststreptococcal GN
10 days after pharyngitis or 2 weeks after a

skin infection (impetigo) with a nephritogenic strain of group A -hemolytic streptococcus. Classic clinical presentation is full-blown nephritic syndrome-oliguric acute renal failure. Most patients have milder disease. Overt disease presents as gross hematuria (red or "smoky" urine), headache, and generalized symptoms such as anorexia, nausea, vomiting, and malaise.

Swelling of the renal capsule can cause flank or

back pain. Hypervolemia, edema, and hypertension. The antecedent streptococcal infection may still be evident or may have resolved, either spontaneously or in response to antibiotic therapy Most of the times- a clinical diagnosis. Investigations can help. (Now, what are they?? and how do I treat this???)

Lupus nephritis
40-85% of SLE patients Varying picture- asymptomatic proteinuria to full

blown nephritic Pathogenesis is contributed by thrombotic microangiopathy(??), in addition to Immune complex mediated nephritis(remember this??) 5 classes based on biopsy (I to V)

Cryoglobulinemia and bacterial endocarditis

Circullating immune complexes- deposited in the

glomerulus. Cryoglobulins- also have skin ulcers, arthralgias, fatigue, raynauds. HCV has been implicated. 50% have renal involvement Bacterial endocarditis

80% of the patients show renal involvement. Varies from indolent smoldering inflammation to

rapidly progressive renal failure. Upper & lower respirarory tract involvement

Anti GBM disease


Anti GBM disease

Antibodies to Col4A5 Anti-GBM nephritis with lung hemorrhage (50

70% of patients) is referred to as Goodpastures syndrome. Presents with hematuria and rapidly progressive renal failure over weeks, with or without pulmonary hemorrhage. When pulmonary hemorrhage occurs, it usually predates nephritis by weeks or months. Mild dyspnea on exertion to life-threatening pulmonary hemorrhage. hypertension is unusual


Urea, creatinine C3, C4 levels ASO, Anti- DNAse Urine- RBCs, sediment, protein. ANA, ANCA, HCV Anti- GBM antibodies.

Granular, Linear

Renal biopsy
Gold standard Delineates the problem to glomerulus,

tubulointerstitum. Identifies specific immunoglobulins- eg IgA Immunoflorescence

Not a childs play

Post streptococcal- simple
Eliminate streptococcal infecton Supportive therapy with diuretics and anti

hypertensives Rarely need renal replacement therapy Excellent prognosis

Lupus nephritis- not so easy,needs biopsy.

No Rx- normal biopsy results or only mesangial

deposits, these histologies portend an excellent prognosis. Glucocorticoids and cyclophosphamide are the mainstays of therapy for patients with proliferative nephritis. Cyclophosphamide IV pulsed cyclophosphamide has good efficacy and is less toxic. Initially monthly intravenous boluses of cyclophosphamide for 6 months. Subsequent therapy is tailored to disease activity and typically involves dosing every 36 months for a total treatment period of 1224 months.


dose is 0.5 g/m2, with the dose increased gradually to a maximum of 1 g/m2 unless patients develop leukopenia or other side effects. May be replaced with either azathioprine or mycophenolate.


at 1 mg/kg per day and taper over the first 6 months to a maintenance dose of 5 10 mg/d for the duration of cyclophosphamide therapy. High dose IV steroids to tide over the acute

Cryoglobulinemia- Glucocorticoids with/without

cyclophosphamide Wegeners- Cyclophosphamide Anti- GBM disease-Emergency plasmapheresis daily or on alternate days until anti-GBM antibodies are no longer detected in the circulation (usually 12 weeks). Prednisone (1 mg/kg per day) is started simultaneously, in combination with either cyclophosphamide or azathioprine to suppress new synthesis of anti-GBM antibodies. Early initiation of therapy is critical Relapses are not unusual.