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Diffuse Pulmonary Hemorrhagic Syndrome (Morphology (temporal heterogeneity…
Diffuse Pulmonary Hemorrhagic Syndrome
Autoimmune
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Goodpasture's
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antibodies attack alpha subunit BM
type IV collagen
glomerular
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Pulmonary
Immunofluorescence
linear IgG deposits in GBM
often accompanied by C3 deposition
Type II Hypersensitivity Reaction
Complications
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contraction atelectasis
cor pulmonale
Wegener Granulomatosis
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necrotizing granulomatous vasculitis
small to medium sized vessels
other organs
kidneys
respiratory tract
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C-ANCA (circulating anti-neutrophil cytoplasmic antibodies)
AP3 (anti-protease 3)
within lysosomes of neutrophils
Immunofluorescence
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binding of C-ANCA to ethanol-fixed neutrophils
T-Cell Mediated Hypersensitivity
fibrinoid necrosis
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early infiltrate by neutrophils
granuloma with giant cells
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more common in young men
Complications
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Idiopathic
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self-limiting
unknown cause
children more common than adults
Immunofluorescence
negative for hypersensitivty
Pauci-immune
Complication
fibrosis
RHF
Morphology
alveolar wall fibrosis
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multiple pulmonary nodules
temporal heterogeneity
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new hemorrhage
less than two weeks old
hemosiderin-laden macrophages
heart failure cells
at least 2 weeks old
Prussian blue stain
cavities
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pleural effusions
mucosal ulcerations
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Signs and Symptoms
kidney involvement
glomerulonephritis
type III hypersensitivty
hematuria
renal failure
URT involvement
sinusitis
nasal ulcerations
Triad
cough with hemoptysis
pulmonary infiltrates on CXR
Iron deficiency anemia
Other causes
drug reactions
methotrexate
cardiac
mitral stenosis
pulmonary infections
Hantavirus
Leah Adeli
Group 14
CM #4
4/5/2017