Small Group 5 Kidney Diseases with Hematuria

Question 1

1. Describe and differentiate the presentation systemic lupus erythromatosis.

Answer 1

key symptoms include fatigue, arthralgias, myalgias, fevers, FH of rheumatologic disease, miscarriage may indicate complication of antiphopholipid syndrome (hyper coagulable state and thrombin glomerulopathy)

signs of fever, malar rash, arthritis, labs with high ESR and CRP, low WBC, low complement and high ANA titer

further supported by proteinuria, active urinary sediment and increased serum creatinine

tests to confirm is antibody against double stranded DNA (included in an ANA profile)

Question 2

1. Differentiate using light, immuno-florescent and electron microscopic findings of SLE.

Answer 2

LM: glomeruli show endocapillary proliferation and segmental wire loop immune complex deposits in the sub endothelial areas, increased cellularity of PMN
IMF: full-house patter of immunofluroescence (IgG, IgA, C3, C1q and IgM) in mesangium and capillary walls
EM: large sub endothelial and intracapillary electron-dense deposits corresponding to wire loop deposits, sub endothelial electron-dense deposits and **endothelial tubuloreticular inclusions

Question 3

2. Describe the pathophysiologic mechanisms for including disease severity, prognosis and decisions regarding tx of SLE.

Answer 3

principal initiator is the deposition of immune complexes with antigen dsDNA which triggers an inflammatory reaction and activates complement

severity is highly variable
I- normal LM
II immune complex in mesangiu
III- focal proliferative
IV-diffuse proliferative
V- membranous 
VI >90% vasculitis

SLE can also lead to vasculitis or vasculopathy

Tx. primary disease with corticosteroids, plaque nil can decrease autoimmune inflammation; long term kidney outcomes improve with addition of cyclophophamide (IV- infectious and infertility toxicities) or mycophenolate

Question 4

1. Describe and differentiate the presentation systemic IgA nephropathy.

Answer 4

hx. of hematuria with gross hematuria in the face of URI, lack of other signs

serum IgA elevated in 60%, renal ultrasound rule out other sources of blood, CXR rule out syndromes with respiratory component, spot urine protein to quantitize protein and creatinine, ANCA, C3, C4 and ANA can be pertinent negatives

Question 5

1. Differentiate using light, immuno-florescent and electron microscopic findings of IgA nephropathy.

Answer 5

LM: glomerulus with mesangial widening due to mesangial proliferation, accumulation of matrix, immune deposits or some combination of these (PAS and silver highlights cellularity and extra-cellular matrix

IMF: IgA is positive in the mesangium

EM: electron dense deposits in the mesangium, mesangial widening and increased matrix

Question 6

2. Describe the pathophysiologic mechanisms for including disease severity, prognosis and decisions regarding tx of IgA nephropathy

Answer 6

deposition of IgA immune complexes in the mesangium causes inflammation and damage

disease is not typically aggressive; low proteinuria and normal function means better prognosis (no tx, just monitoring), ACEI or ARB may slow the progression to ESRD (will decrease proteinuria) and perhaps protein restriction depending on the patient; fish oil capsules (eicosapentoic acid may be helpful

Question 7

Which diseases are commonly present with gross hematuria? Which occur with respiratory and kidney presentations?

Answer 7

ADPKD
IgA nephropathy
some GN (particularly post-infectious GN)

Wegner’s, Goodpasture’s syndrome and Churgg-Strauss
post-infectious GN is associated with pneumonia or sinusitis, IgA can flare in case of URI

Question 8

What are the most common causes of glomerular disease with low complement?

Answer 8

MEPPS: MPGN, endocaridits, post strep, post infectious and SLE (complement is being consumed)

Question 9

What kidney problem is often accompanied by deafness and cataracts?

Answer 9

Alports Syndrome, more severe in men