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NEPHRITIC SYNDROME AND DISORDERS CAUSING IT - Coggle Diagram
NEPHRITIC SYNDROME AND
DISORDERS CAUSING IT
DEFINITION
Inflammation that damages the glomerular basement membrane (GBM)
MULTIPLE MECHANISM
Membranoproliferative
Glomerulonephritis
➢ Proliferation of mesangial & endothelial cells in glomerulus
endothelial cells in glomerulus
➢ Common: Type I MPGN (idiopathic or secondary to Hep B or Hep C > by type iii hypersensitivity reaction or inappropriate activation of alternative activation of alterative pathway (IgG autoantibody: low serum level of C3)
or secondary to Hep B or Hep C) > by
➢ Light microscopy: Tram-Track appearance
➢ Immunofluorescence: granular glomerular
➢ Type II MPGN: cause by nephritic factors > dense deposit disease (low circulating level of C3)
factors > dense deposit disease (low
➢ Light microscopy: Tram--Track appearance
Rapidly Progressive
Glomerulonephritis
➢ Initiated by Type II or type III hypersensitivity
➢ No immune complex
➢ Kidney function ↓ in days to weeks
➢ 3 types of idiopathic RPGN
➢ Goodpasture disease: type II hypersensitivity . anti-GBM antibodies
➢ Type III Hypersensitivity: PSGN and DPGN
➢ Type III: Pauci-Immune Vasculitis less anti-GBM antibodies
Granulomatosis with polyangiitis (GPA) affects
respiratory tract and kidneys
Presence of c-ANCA antibodies, non-caseating granuloma
Microscopic polyangiitis: no URT symptoms and treat by penicillin, presence of p-ANCA antibodies, no granuloma
➢ Light microscopy: crescent-moon shaped (fibrins)
➢ Immunofluorescence: Good Pasture disease (linear), Type II (granular)
SYMPTOMS
Peripheral and periorbital oedema, Arterial hypertension, ↓ urine output, Cola-colored urine; hematuria
LAB DIAGNOSIS
↑ Creatinine, ↑ BUN, Urinalysis: haematuria, proteinuria (subnephrotic range;<3.5g/day) and RBC casts, Kidney biopsy
TREATMENT
Supportive therapy (sodium diet and water restriction), ACE inhibitors, ARB, Diuretics, Immunosupressive therapy, Plasmapheresis, Transplantation, RAAS inhibitors, Prendisone
TYPE III HYPERSENSITIVITY
IgA Nephropathy/
Berger’s disease
➢ Abnormal IgA (foreign) + IgG
➢ Immune complex deposits in mesangium
➢ Activates alternative complement pathway > proinflammatory cytokines and macrophages release glomerular injury
➢ Associates with respiratory or GI infection
➢ Light microscopy: mesangial proliferation
➢ Immunofluorescence: immune complex
➢ Electron microscopy: immune complex
➢ Similar disorder: IgA vasculitis (nephritic or nephrotic syndrome, colicky abdominal pain, bloody stool, arthritis and palpable skin
lesion)
Poststreptococcal
Glomerulonephritis
➢ Frequent in children
➢ 2-4 weeks after GAS infection of the pharynx or skin
Virulence factor: M protein
➢ Antibodies (IgG & IgM form immune complexes with bacterial antigen)
➢ Inflammation of GBM with presence, of C3 complement, cytokines, oxidants, proteases
➢ Lab test: ↓ level C3 in blood and positive strep titers and serologies
➢ Light microscopy: glomeruli enlarges and hypercellular
➢ Immunofluorescence: IgG, IgM and C3 deposits at GBM and mesangium (STARRY SKY)
➢ Electron microscopy: humps
Diffuse proliferative
glomerulonephritis
Systemic Lupus Erythematosus
➢ Autoimmune condition: inflammatory reaction in kidney
➢ Diffuse: 50% of glomeruli in both kidneys affected
➢ Site of immune complex deposition: subendothelial space
➢ Light microscopy: wire loop appearances
➢ Immunofluorescence: granular immune complex
➢ Electron microscopy: subendothelial immune complex
GENETIC
Albort Syndrome
➢ Mutation in type IV collagen gene
➢ GBM becomes thin & splits
➢ Hearing loss, retinopathy and lens dislocation
➢ Electron microcopy: GBM basket weave (thick and thin lining)