haematuria/nephritic syndrome Flashcards
(53 cards)
mnemonic to remember causes of haematuria
Stone
Haematological / hereditary
Infectious/iatrogenic/idiopathic/immune
Renal
Tumour/trauma
factitious haematuria - some causes
urate crystals in infants, foods/dyes, haemoglobinuria from haemolytic anaemia, myoglobinuria from rhabdo, drug metabolites
extraglom vs glomerular haematuria
extraglom: red/pink, can have clots, usually no protein, rbc morph normal, no casts
glomerular: cola, no clots, can have protein, rbc dysmorphic, can have casts
types of RPGN
type I = anti-GBM (goodpastures); linear on IF
type II = immune complexes e.g. IgA, SLE, PSGN, HSP; granular on IF
type III = ANCA, pauci-immune; nothing on IF
cANCA vs pANCA vasculitides
CAW: cANCA = Wegner’s
PAMC: pANCA = microscopic polyangitis, Churg-Strauss
RPGN also known as what?
crescentic GN - crescent shape proliferation of cells into Bowman’s capsule
causes of haematuria with low C3 vs low C3 AND C4
Low C3 = typically due to activation of the alternate C’ pathway
- APSGN
C3 glomerulopathies including DDD (subtype of MGPN)
Low C3 + C4 = indicates activation of classic pathway due to complex formation
- Lupus nephritis
- MPGN I
- Shunt nephritis + associated with bacterial endocarditis
What would the follows IF stains suggest?
i. IgG and C3 on external side of GBM
ii. IgG and C3 found along GBM + mesangium
iii. IgG1, IgG3, IgA, IgM, C3, C4, C1q
i. IgG and C3 on external side of GBM = APSGN (‘starry sky’)
ii. IgG and C3 found along GBM + mesangium = MPGN type I + II
iii. IgG1, IgG3, IgA, IgM, C3, C4, C1q = ‘full house’ ie. SLE
what are these pathognomonic features of?
i. Crescentic
ii. Tram tracking
iii. Wire loops
iv. Starry sky
v. Subepithelial humps
i. Crescentic = RPGN – ANCA associated, anti-GBM, post strep
ii. Tram tracking = MPGN
iii. Wire loops = lupus class III/ IV
iv. Starry sky = APSGN (granular pattern) on immunofluorescence
v. Subepithelial humps = APSGN on EM
name some synpharyngitic causes of haematuria besides PSGN
• Alports
• Thin basement membrane
• IgA
• HSP nephritis
pathogenesis in goodpasture’s
anti-GBM Abs against alpha3 chain of collagen IV of BM
esp HLA DR15
environmental stress will expose the alpha 3 further
lung Sx first, then kidneys
PSGN vs IgA nephropathy - timeline after infection
IgA = 1-2 DAYS (by definition <5/7 days)
VS. postinfectious GN = 10-14 days post pharyngitis, or 3-6 weeks post skin infection
5 key presentation types of IgA nephropathy
- haematuria
- nephritic
- RPGN
- nephrotic (rare <10%)
- mixed nephrotic/nephritic
HTN treatment in IgA nephropathy vs PSGN
IgA = ACE/ARB
PSGN = frusemide
IgA biopsy findings similar to what disease?
HSP, but IgA isolated to renal disease
ESKD likelihood with IgA nephropathy?
20-30% of children will develop ESKD in 15-20 years after disease onset
genetics of alport’s syndrome
mutation in COL4A3/4/5 causing collagen IV abnormality
- COL4A3 and 4 autosomal (AR - early onset/AD - late onset)
- most COL4A5 which is X-linked
clinical manifestations of alport’s
ALPORT:
anterior lenticonus
persistent haematuria
ototoxicity and SNHL
renal - FSGS, basket weave
Treatment - ACEI, renal transplant
pathognomonic features of Alport’s
anterior lenticonus
basket weave BM pattern on EM
*IF will be non-diagnostic…nothing will light up
thin basement membrane disease associated with what condition and why?
alport’s
also a/w COL4A3 and COL4A4 mutations
in fact, homozygous mutations will result in AR Alport’s
also a/w haematuria and BM thinning
TBMD - gross haematuria present when?
associated with resp illness
when is TMBD termed benign familial haematuria?
isolated haematuria in multiple family members, not associated with other signs of renal disease (proteinuria, renal impairment)
age range of PSGN
2-13, uncommon <3yo
when does ASOT and anti-DNase B rise post strep infection?
ASOT - 3-4 weeks post
anti-DNase B - 6-8 weeks post
