Syndromes

Question 1

CREST scleroderma

Answer 1

Calcinosis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Question 2

Charcot’s neurological triad

Answer 2

multiple sclerosis

1. scanning speech
2. intention tremor
3. nystagmus

Question 3

albuminocytologic dissociation

Answer 3

CSF- nml cell count with elevated protein seen in Guillan Barre

Question 4

HSP

Answer 4

renal disease (IgA nephropathy), palpable purpura in dependent areas, arthralgias

Question 5

Alport Syndrome (x-linked mutation of type IV collagen that leads to thinning and splitting of the glomerular basement membrane)

Answer 5

glomerulonephritis, deafness, eye problems

can’t see, can’t pee, can’t hear high C

Question 6

Goodpasture syndrome (type II hypersensitivity characterized by anti- GBM abs and anti-alveolar BM)

Answer 6

kidney and lungs, linear deposits on immunofluorescence that are crescent-moon shaped (C3b, glomerular parietal cells, monocytes, macrophages, debris), 2/2antibodies to GBM and alvolar BM.

Question 7

Granulomatosis with polyangiitis (Wegener)

Answer 7

PR3-ANCA/c-ANCA granulomatosis with polyangiitis, also has linear deposits on immunofluorescence that are crescent-moon shaped (C3b, glomerular parietal cells, monocytes, macrophages, debris), 2/2antibodies to GBM and alvolar BM.

Question 8

Microscopic polyangiitis

Answer 8

MPO-ANCA/p-ANCA, linear deposits on immunofluorescence that are crescent-moon shaped (C3b, glomerular parietal cells, monocytes, macrophages, debris), 2/2antibodies to GBM and alvolar BM.

Question 9

Acute poststreptococcal glomerulonephritis

Answer 9

Imaging:
light microscopy: enlarged, hypercellular glomeruli
immunofluorescence: “starry sky” granular appearance; “lumpy bumpy” 2/2 IgG, IgM, C3 granular deposition along GBM and mesangium.
Complement activation leads to BM destruction.
electron microscopy: subepithelial immune complex (IC)- “humps”

Labs:
this happens 2/2 specfic strains of group A strep. Test for it using ASO titer which will be positive if the patient was recently infected.
ASO will not be positive if strep impetigo, so if you suspect impetigo look for anti-DNAse B.
CBC may show decreased serum C3

NOTE that abx will prevent acute rheumatic fever but will not prevent APSGN, as this nephritic syndroms depends on a type III hypersensitivity reaction occuring 1-3 weeks post infection.

Si/Sx: peripheral, periorbital edema, dark urine, hypertension