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BY: DR. NURUL NAZIFAH AZIZACUTE NEPHRITIS SYNDROME
Disease associated with acute nephritis syndromeInfective causesPost streptococcal glomerulonephritisNon streptococcal post infectious glomerulonephritisStaphylococcus, pneumococcus, legionella, syphilis, mumps, varicella, hepatitis B and C, echovirus, Epstein barr virus, toxoplasmosis, malaria, schistosomiasis, trichinosis
Non infectiveMultisystemVasculitis (eg wagener granulomatosis)Collagen vascular disease (eg SLE)Henoch schonlein purpuraPolyarteritis nodosaCryoglobulinemiaGoodpasture syndromePrimary renal diseaseMPGNIgA nephropathy (berger disease)Idiopathic RPGN
epidemiologyWorldwide, Berger disease is the most common cause of GNthe incidence of PSGN has fallen in most Western countries. PSGN remains much more common in regions such as Africa, the Caribbean, India, Pakistan, Malaysia, Papua New Guinea, and South Americathe incidence of acute GN in children aged 3-16 years was 15.5 cases per year, with a male-to-female ratio of 1.1:1
Post streptococcal glomerulonephritis
etiology
Group A beta hemolytic streptococcusPSGN usually develops 1-3 weeks after acute infection with specific nephritogenic strains of group A beta-hemolytic streptococcusOnly few strains of streptococci are nephritogenic, eg. Type 4 and 12 causing pharyngitis and type 49 causing pyoderma.The incidence of GN is approximately 5-10% in persons with pharyngitis and 25% in those with skin infections.
pathogenesis
glomerular-immune complex formation
immune complexes formed from antigen, antibodies, and complement, get trapped in kidney filters (Glomeruli).
The filters become inflamed, which leads to ineffective kidney function.
pathologyHistopathology- glomeruli appear enlarged and shows diffuse mesangial cell proliferation with invasion of acute inflammatory cellsImmunofluorescence- C3 and IgG deposition on glomerular BM and in the mesangium.Electron microscopy- electron dense deposits or hump like deposits of immunocomplexes seen on epithelial side of GBMChanges usually restricted to mesangium and endothilium with minimal epithilial proliferation
PSGN - hypercellular glomerulus with proliferating endothelial and mesangial cells, and neutrophil infiltration. PSGN- granular bumpy pattern of immune deposits on immunofluorescence
Clinical presentation
This disorder may begin to develop one to two weeks after an untreated throat infection
three to four weeks after an untreated skin infection.
mostly common in children ages five to twelve.
Clinical presentation
3 phase sequence: infection - interval - nephritic syndrome
The severity of renal involvement varies from asymptomatic microscopic hematuria with normal renal function to acute renal failure
Abrupt onset of:
Hematuria(cola coloured)
Oliguria( Reduced GFR)
Oedema (Salt and water retention) periorbital puffiness and pedal edema
Hypertension
DiagnosisUrinalysisRbcFrequently in association with rbc castsMild proteinuriaPolymorp leukocytes
A mild normochromic anemia may be present from hemodilution and low-grade hemolysis.
The serum C3 level is usually reduced in the acute phase and returns to normal 68 wk after onset
positive throat culture report may support the diagnosis
The antistreptolysin O titer is commonly elevated after a pharyngeal infection
anti-deoxyribonuclease (DNase) B level after cutaneous infection.
Indications for renal biopsyRenal biopsy should be considered only in the presence of acute renal failure, nephrotic syndrome, absence of evidence of streptococcal infection, or normal complement levels. In addition, renal biopsy is considered when hematuria and proteinuria, diminished renal function, and/or a low C3 level persist more than 2 mo after onset
treatmentFocused on treating acute effects of renal insufficiency and hypertension
Restriction of salt and fluid
Antibiotics (ex. Penicillin), for streptococcal bacteria- to limit spread of organism
Blood pressure medications calcium channel blocker-nifedipine
diuretic medications may be needed to control swelling and high blood pressure.
prognosis
Usually mild disease
recovery typically within weeks ( 95% )
1 week: onset of diuresis
4 weeks: normalization of renal function
3-6 months: resolution of hematuria
Possible complications
Congestive heart failure
pulmonary edema
Hyperkalemia
High blood pressure (hypertension)
Acute renal failure
Chronic glomerulonephritis
IgA nephropathy
IgA nephropathyPathogenesisFocal and segmental proliferative glomerulonephritis with mesangial deposit of polymeric IgAEtiologyUnknownMay be a result of an exaggerated bone marrow and tonsillar IgA immune response to viral or other antigens
Clinical and lab manifestationTends to occur in children and young males
> 80% children have experience of gross hematuria (in U.S.)
Asymptomatic microscopic hematuria or recurrent macroscopic hematuria following URTI or GI viral infection
Proteinuriaoften, but severity < nephrotic, often
Although IgA nephropathy does not lead to significant kidney damage in most children, progressive disease develops in 2030% of children at 1520 yr after disease onset.
Poor prognostic indicators include persistent hypertension, diminished renal function, and heavy or prolonged proteinuria. A worse prognosis is suggested by histologic evidence of diffuse mesangial proliferation, extensive glomerular crescents, glomerulosclerosis, and tubulointerstitial changes, including inflammation and fibrosis.
treatmentPrimary treatment is proper blood pressure control
Fish oil, which contains anti-inflammatory omega-3 polyunsaturated fatty acids, decreases the rate of renal progression
Immunosuppressive therapy with corticosteroids
Angiotensin-converting enzyme inhibitors and angiotensin II receptor antagonists are effective in reducing proteinuria and retarding the rate of renal progression when used as single agents or in combination.
Tonsillectomy may reduce the frequency of gross hematuria and the rate of renal disease progression
Henoch schonlein purpura
Henoch schonlein purpuraa small vessel vasculitischaracterized by a purpuric rash, arthritis, abdominal pain, and glomerulonephritisHSP nephritis and IgA nephropathy demonstrate identical renal pathologic findings, but systemic findings are only seen in HSP nephritis
pathogenesisThe pathogenesis of HSP nephritis remains unknownthis disease appears to be mediated by the formation of immune complexes containing polymeric IgA1 within capillaries of the skin, intestines, and glomerulus.
Clinical and lab manifestationThe symptoms and signs of HSP nephritis typically appear 13 wk after an upper respiratory tract infectiongross hematuria is seen in 2030% of casespatients may also present with isolated microscopic hematuria, hematuria and proteinuria.Renal manifestations of HSP nephritis occur up to 12 wk after the initial presentation of HSP
treatmentSymptomaticSome studies suggest that short courses of low-dose prednisone initiated at diagnosis reduce the subsequent risk of developing any clinical signs of nephritis
Rapidly progressive glomerulonephritisasyndrome of thekidneythat is characterized by a rapid loss of renal function,(usually a 50% decline in theglomerular filtration rate(GFR) within 3 months)with glomerularcrescent formation seen in at least 50%or 75%of glomeruli seen on kidney biopsies.
Crescentic glomerulonephritis (PAS stain). Note the collapsed glomerular tufts and the crescent-shaped mass of proliferating cells and leukocytes internal to Bowman capsule
ANTI-GBM ANTIBODYMEDIATED RPGN 20%Goodpasture syndromeIdiopathic anti-GBM nephritisMembranous nephropathy with crescentsRPGN ASSOCIATED WITH GRANULAR IMMUNE DEPOSITS 40%PostinfectiousPoststreptococcal glomerulonephritisBacterial endocarditisShunt nephritisVisceral abscesses, other nonstreptococcal infectionsNoninfectiousSystemic lupus erythematosusHenoch-Schnlein purpuraMixed cryoglobulinemiaSolid tumorsPrimary Renal DiseaseMembranoproliferative glomerulonephritisIgA nephropathyIdiopathic immune-complex nephritisRPGN WITHOUT GLOMERULAR IMMUNE DEPOSITS 40%VasculitisPolyarteritisHypersensitivity vasculitisWegener granulomatosisIdiopathic RPGN
Sign & symptomsThe clinical picture is consistent withnephritic syndrome, although the degree of proteinuria may occasionally exceed 3g/24 h, a range associated withnephrotic syndromeDiagnosisThe presence of anti-Glomerular basement membrane(GBM) antibodies suggests type I RPGN;antinuclear antibodies(ANA) may support a diagnosis ofsystemic lupus erythematosus and type II RPGN; and type III and idiopathic RPGN are frequently associated withanti-neutrophil cytoplasmic antibodies(ANCA)-positive serum.
TreatmentExcellent therapeutic response using a combination of corticosteroids and cytotoxic therapy with cyclophosphamide often occurs in patients with systemic lupus erythematosus, IgA nephropathy, and Henoch-Schnlein purpura nephritis
Plasmapheresis beneficial in patients with systemic vasculitides and Goodpasture syndrome
THANK YOU.
ReferencesNelson Textbook Of Paediatric 18th Ed.Kumar & Clarks Clinical Medicine 7th Ed.Ghais Essential Paediatric 7th Ed.Emedicine.medscape.com
