nephritic syndrome€¦ · nephritic syndrome done by: ahmad al-masri •it’s an inflammatory...
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Nephritic Syndrome
Done by: Ahmad Al-Masri
• It’s an Inflammatory process that involves glomeruli (damage involving the basement membrane ,capillary endothelium , and mesangium) (while in nephrOtic syndrome the main structure to be damaged is Podocyte)
• Due to the glomeruli damage:
• filtration barrier is weakened and enables passage of proteins ,erythrocytes
• ↓GFR Oliguria & ↑BUN/Cr ratio
• ↑Hydrostatic pressure Hypertension & Edema
Classic presentation: • Dark urine (RBCs)
• Proteinuria (<3.5g/day)
• Swelling/edema
• Fatigue (uremia)
• Hypertension
Causes
• Could be classified Primary ( idiopathic) v.s. Secondary
• Or classified as Immune mediated v.s. Pauci-immune
Rapid progressive glomerulonephritis (RPGN)
• is an acute GN resulting from the secondary sequelae of inflammatory (crescenteric) GN, of which there are many causes.
Crescenteric apperance
Negative IF Granular IF Linear IF
Post-infectious GN
• GN secondary to bacterial infection most commonly the nephritogenic strains of streptococcus (group A beta hemolytic streptococci).
• Immune complexes deposit in subendothelial space.
• Common in children (can also occur in adults) • Classic case: • Child presented 2-3 weeks following strep throat
infection or 6 weeks post impetigo with Nephritic syndrome findings
Post-infectious GN • If we take a Bx. we will see
• Under light microscope: Enlarged, hypercellular glomeruli
• Under immunofluorescence: Granular apperance (IgG, IgM, C3)
• Under Electron microscopy: Subepithelial “humps’’
Post-infectious GN
• Good prognosis in children (95% recover completely)
• Adults have worse prognosis (About 60% recover)
• No specific therapy (supportive with management of high blood pressure and fluid overload , spontaneous resolution is expected )
IgA Nephropathy Berger’s Disease
• Most common form glomerulonephritis in adults worldwide.
• Most common cause of repeated episodes of hematuria (nephritic).
• Typically cause hematuria 2 days post URTI or Diarrheal illness.
• Over time leads to ESRD and HD (50% patients)
• Affect mainly Males ,especially Asian or white population.
IgA Nephropathy Berger’s Disease
• IgA Immune complexes deposit in mesangium. • Bx. under immunofluorescence: Granular apperance (IgA)
• Classic case: • Recurrent episodes hematuria since childhood, follow URI or
diarrheal illness, Slowly worsening renal function (BUN/Cr) over time, Possible progression to ESRD and HD (20yrs+)
• Don’t confuse with other glomerular disorders 1) Post-strep GN: weeks after infection 2) IgA GN: days after infection 3) Minimal change: nephrotic syndrome after URI
IgA Nephropathy Berger’s Disease
• Patients who have IgA nephropathy with a low risk of progressive disease should be treated with ACE-I to maintain a goal 24 hour urine protein excretion of less than 1 g /24 hours , and a blood pressure of less than 130 / 80 .
• Immunosuppressive therapy must be considered for: 1- patients with persistent proteinuria ( more than 1g/24 h) , 2-progressive kidney dysfunction, 3-or histological findings indicative of RPGN ( like crescents ) This includes pulse corticosteroids and cyclophosphamide followed by azathioprine with ACE-I . • Few studies shows that Fish Oil have role in delay some of the
late complications of IgA nephropathy
Henoch-Schonlein Purpura
• IgA nephropathy + extra-renal involvement
• Skin: palpable purpura on buttocks/legs
• GI: abdominal pain, melena
• Joint pains
• Most common childhood systemic vasculitis
• Diffuse IgA deposition
• Tissue biopsy: demonstrates IgA
Lupus Nephritis
• They are subdivided into 6 types, here in nephrtic syndrome we are concern with
type lll (Focal Proliferative lupus nephritis) & type IV (Diffuse Proliferative lupus nephritis). • Focal: less than 50% glomeruli affected
• Diffuse: More than 50% glomeruli affected
• Anti-dsDNA Subendothelial deposits
Lupus Nephritis • If we take a Bx. we will see
• Under light microscope: capillary loops thickened
• “Wire looping”
• Under immunofluorescence: Granular apperance
• “Full house” immunofluorescence (IgG, IgA, IgM, C3, C1q)
Lupus Nephritis
• Usually we take Bx. To know the severity to decide the prepare treatment.
• Steroids alone or with Cyclophosphamide?
Membranoproliferative glomerulonephritis
• Also called mesangiocapillary GN.
• Immune complex formation secondary to chronic antigen stimulation.
• Common causes are : 1. Chronic indolent infections including hepatitis C, syphilis ,
mastoiditis …etc. .
2. Autoimmune such as Sjogren , lupus …etc.
3. Essential cryoglobulinemia ( types I and II )
4. Malignancies such as carcinomas , sarcomas , lymphomas and leukemias .
immune-complex and/or complement protein deposition in the mesangium and sub endothelium of the capillaries this results in proliferation of the mesangial and remodeling of the capillary wall
• We usually treat them with:
1. Corticosteroids
2. Immunosuppressive agents
3. Antiplatelets
• More than 50 % progress to advanced CKD .
Anti-GBM antibodies disease Goodpasture’s Syndrome
• IgG antibodies develop against the GBM (alpha-3 chain to type IV collagen ) , deposited in a linear patter.
• Classic case: • Young adult, Male present with Hemoptysis &
Hematuria • Treatment usually is Plasmapheresis using albumin
replacement for 1-2 weeks ,followed by corticosteroids and cyclophosphamide for 3-6 months , Maintenance with azathioprine for 1-2 years
Vasculities syndrome
• Most patients ANCA positive (c-ANCA or p-ANCA)
• All vasculities are associated with Fever + weight loss.
• Churg-Strauss syndrome (p-ANCA): they present with Eosinophilia, history of resistant asthma.
• Wegener's Granulomatosis (c-ANCA): they present with upper respiratory findings (sinusitis/otitis)
• All can lead to pauci-immune nephritis
Alport Syndrome Hereditary Nephritis
• Genetic Mutations in alpha-3, alpha-4, or alpha-5 chains of type IV collagen.
• Chains found in basement membranes kidney, eye, ear
• Inherited: X-linked
• Classic triad:
• Hematuria, Hearing loss, eye abnormalities
• Look for child with triad and family history
Thank You