Nephritic syndromes Flashcards
IgA nephropathy (Maladie de Berger): pathology and causes
- abnormal IgA forms, -> IgA-antibody complex formation -> deposits in kidneys → kidney damage
Associated with gastrointestinal (GI)/respiratory tract infections
IgA nephropathy (Maladie de Berger): RF
- Most common nephropathy worldwide
- usually presents in childhood
- Highest prevalence in people of East Asian/European ancestry
- Family history of chronic nephritis, alcohol consumption, recurrent infections
IgA nephropathy (Maladie de Berger): Signs and symptoms
- Episodic hematuria
Sometimes accompanying upper respiratory tract infections - Asymptomatic microscopic hematuria, with subnephrotic proteinuria
- Classic nephrotic syndrome/kidney injury (minority)
IgA nephropathy (Maladie de Berger): Diagnosis
Perform urinalysis and a renal biopsy
Urinalysis -> hematuria
- RBCs, RBC casts
Renal biopsy:
- Light microscopy: Mesangial proliferation, immune complexes deposited in mesangium
- Electron microscopy: Immune complexes deposited in mesangium
- Immunofluorescence: Mesangial IgA deposits, +/- IgA, +/- IgM
IgA nephropathy (Maladie de Berger): treatment
- Prednisone (Oral/parenteral glucocorticoid intermediate actions)
Rapidly Progressive Glomerulonephritis: pathology and causes
- Inflammation of kidney’s glomeruli → crescent-shaped proliferation of cells in Bowman’s capsule → renal failure within weeks/months
RPG: Primary type
- Idiopathic
RPG: Secondary type
Type I: anti-GBM antibodies
- Goodpasture syndrome
Type II: immune complexes
- Poststreptococcal glomerulonephritis
- systemic lupus erythematosus
- IgA nephropathy
- Henoch-Schonlein purpura
Type III: anti-neutrophilic cytoplasmic antibodies (ANCA)
- Cytoplasmic ANCA (C-ANCA): Wegener’s granulomatosis
- Perinuclear ANCA (P-ANCA): microscopic polyangiitis, Churg-Strauss syndrome
RPG: Signs and symptoms
Nephritic syndrome:
- Hematuria,
- oliguria,
- edema,
- hypertension
RPG: Complications
-> rapid progressive to acute renal failure if untreated
RPG: Diagnosis
Perform a kidney biopsy + immunofluorescence
Urinalysis: -> hematuria
Kidney biopsy
- Light microscopy: crescent-shaped glomeruli
Immunofluorescence
- Type I: linear, antibodies bind to collagen of glomerular basement membrane
- Type II: granular, immune complex deposition in subendothelium
- Type III: negative (pauci-immune), associated with ANCAs in blood
RPG: treatment
Pulse methylprednisolone, then:
- prednisone
- cyclophosphamide
- rituximab
- plasmapheresis
In case of renal failure -> dialysis + kidney transplant
Goodpasture syndrome: pathology and cause
- anti-GBM antibody disease -> damage to the basement membrane in lungs + kidneys
Mostly composed of Type IV collagen
Goodpasture syndrome: RF
- Bimodal distribution: male between 20-30, female between 60-70
- genetic: predisposition for genes that code for HLA-DR15
- Environmental: infection, smoking, oxidative stress, hydrocarbon-based solvents
Goodpasture syndrome: Signs and symptoms
Pulmonary manifestations usually occur before renal ones. Minority (20–40%) with only renal manifestations
- Damaged lung alveoli → cough, hemoptysis, dyspnea
- Kidney filtration problems -> hematuria, proteinuria), nephritic syndrome