Glomerular Disease 1 Flashcards
(32 cards)
- What is a diffuse process?
- What is a focal process
- What is a global disease?
- What is a segmental disease?
- All of the glomeruli
- Some of the glomeruli
- The entirety of a glomeruli
- Only part of a glomeruli
How does glomerular disease present? (3)
Loss of GFR - Temporal change
Hematuria - Quality
Proteinuria - Quantity
What is a nephrotic syndrome?
Proteinuria > 3.5 g/day
Hypoalbuminemia
Edema
Hyperlipidemia
Lipiduria
Hypercoaguability (Loss proteins C and S)
What is nephritis?
Mild proteinuria
Hematuria
Hypertension
Edema
What are causes of acute glomerulonephritis?
- IgA nephropathy
- Post-infectious GN
- Anti-GBM dz/Goodpasture’s
- Small vessel vasculitis
- Lupus nephritis
- Membranoproliferative GN
What are mainly nephrotic: dz?
\What are mainly nephritic dz?
Which ones have crossover?
Nephrotic: Minimal change, focal segmental glomerulosclerosis, membranous nephropathy, IgA nephropathy
Nephritic: Crescentic (ANCA), Acute post-infectious,, Membranoproliferative GNs
Crossover: Membranoproliferative GN, IgA nephropathy
What is IgA nephropathy?
What is the common age range for patients?
What is the most prominent feature?
Is proteinuria present?
Most common GN worldwide
Most patients between age of 10-50
Hematuria is most prominent feature: 50-60% episodic gross hematuria, 30% persistent microhematuria, 10% acute GN or nephrotic syndrome
Proteinuria, if present, is generally mild
Many cases are subclinical
What are other complications that accompany IgA nephropathy?
Dysuria and loin pain with hematuria
Hematuria frequently occurs in conjunction with a URI (synpharyngitic hematuria)
HTN may be present in patients with more advanced dz
How does IgA nephropathy appear on LM, IF, and EM?
LM: Variable mesangial hypercellularity
IF: Mesangial IgA deposition
EM: Mesangial ectrone dense deposits
What is IgA nephropathy’s prognosis?
What are treatment’ options?
Prognosis: Based on serum creatinine, BP, proteinuria
40% will slowly develop CKD
Tx: Fish oil can slow progression
ACEi to control BP
Corticosteroids/immunosuppressants used in progressive dz
What is Henoch-Schonlein Purprua?
How can it manifest?
Systemic disorder haracterized by IgA deposition in multiple organs
Manifestations:
Skin - Characteristic non-blanching purpura or legs and buttocks
Joints - Transient arthralgias
GI - Abdominal pain, vomiting, melena, hematochezia
Kidney - Hematuria, proteinuria, rarely with progressive renal dz
What is post-infectious GN and its standard course?
Often post-streptooccal GN
What are the symptoms of post-strep GN?
What are the laboratory studies for post-strep GN?
Sudden onset HTN, azotemia, oliguria, edema and cola- or tea-colored urine
Low C3 level, ASO elevated, urinalysis reveals RBC casts, mild proteinuria
Morphologic features of post-infectious GN (LM, IF, EM)
LM: Enlarged, hypercellular, glomeruli, diffuse mesangial and endocapillary proliferation with neutrophils. May see crescents
IF: Granular capillary wall and mesangial IgG and C3
EM: Mesangial and large subepithelial “hump-like” deposits
What is the prognosis of post-strep GN
95% of children will recover with conservative management (1% progress to renal failure)
60% of adults will recover promptly
What is rapidly progressive GN?
What are the causes?
What are the characteristics on LM?
“Crescentic CN”
Classic nephritic syndrome with rapid progression (days to weeks) to renal failure
Causes: Anti-GBM/Goodpasture’s, immune complex GN (lupus, post-infectious, cryoglobulinemia), ANCA associated (Pauci immune)
Segmental necrosis and cellular crescent
What is Anti-GBM/Goodpasture’s syndrome gender predisposition?
How does it present
What is the cause?
More often in males than females
Presents as a pulmonary-renal failure syndrome: hemoptysis, pulmonary infiltrates, glomerulonephritis
Due to circulating anti-GBM Ab
Antigen is alpha-3-chain of type IV collagen
What is the diagnostic criteria for Anti-GBM/Goodpasture’s Syndrome?
What is treatment?
- Anti-GBM antibody in blood, linear IgG and C3 on kidney biopsy IF
- Treatment with plasmapheresis, prednisone, cytoxan
What is pauci-immune GN?
What causes it?
Crescenteric GN with little deposition of immune reactants
Idiopathic or ANCA
What small vessel vasculitis are associated with nephritis?
Microscopic polyangiitis - No granulomatous inflammation and no asthma
Wegner’s granulomatosis - Necrotizing granulomatous inflammation; no asthma
- c-ANCA in 80%
- URT symptoms, mononeuritis multiplex, purpura, nephritis
- Renal biopsy: cresenteric GN without immune deposits
Curg-Strauss syndrome: Necrotizing granulomatous, inflammation, asthma, eosinophilia
What are secondary causes of nephrotic syndrome?
What is used for diagnosis?
Treatment?
Systemic diseases, Diabetes mellitus, SLE, amyloidosis, infections (HIV, HBV, HCV, syphilis), drugs (NSAIDs, gold penicillamine)
2ndary causes: ANA, anti-dsDNA, complement, serum and urine protein electrophoreses, HBV/HCV serologies, cryoglobulins, syphilis serology
Also renal biopsy generally indicated
ACEi/ARB (reduce intraglomerular pressure and proteinuria), statins (lipids), diuretics, salt restriction (edema)
What is minimal change disease?
What is the course with children?
What are the causes with adults?
Most common cause of nephrotic syndrome in children
Peak incidence ages 2-6, 5% progress to ESRD, spontaneous remissions can occur, treatment with steroids often induces remission although relapses occur in about 75%, fewer relapses after puberty
In adults can be idiopathic or associated with drugs (NSAIDs), neoplasms (Hodgkin’s lymphoma, other cancers), infections (syphilis, HIV)
What is observed for Minimal Change Disease on LM, IF, EM?
What is treatment?
LM: Glomeruli, interstitium and tubules appear normal
IF: Negative or mesangial IgM
EM: Podocyte foot process effacement
Children respond well to corticosteroids, majority of adults also respond to steroid (longer than in children, partial remissions)
What is membranous nephropathy?
What are the secondary causes?
Most common cause of nephrotic syndrome in Caucasian adults
Secondary causes account for 15-20% of cases
Infections - HBV
Connective tissue diseases - SLE
Neoplasms - Carcinoma of lung, colon, stomach, breast, non-Hodgkin’s lymphoma
Drugs - Gold, penicillamine, Hg, NSAIDs, captopril
