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CR - Pul Path (v) Vasc Lung Diseases continued - Vasculitis (Eosinophilic…
CR - Pul Path (v) Vasc Lung Diseases continued - Vasculitis
Diffuse alveolar haemorrhage
primary = immune-mediated disease
secondary
necrotising bacterial pneumonia
passive venous congestion
bleeding diathesis (tendency)
triad
haemoptysis
anaemia
diffuse pul infiltrates
early form = goodpasture syndrome
aka anti-GBM disease
rapidly progressive glomerulonephritis + haemorrhagic interstitial pneumonitis
renal + pul lesions cause by Igs against antigens common to glomerularl + pul basement membranes
type 2 cytotoxic Ig-mediated hypersensitivity
anti-GBM Igs detected in serum of over 90% of patients
focal necrosis of alveolar walls, intraalv haemorrhages, fibrous thickening of septae
if acute: visible haemosiderin macrophages
Tx
plasma exchange to remove Igs
immunosupressants to inhibit Ig production
Granulomatosis with Polyangiitis (GPA)
aka Wegener granulomatosis or ANCA-associated vasculitis (AVA)
multisystem autoimmune disorder
unknown aetiology
hallmarks
pauci-immune vasculitis of small-medium vessels (min evidence of hypersensitivity upon immunofluorescent staining for IgG)
necrotising granulomatous inflamm
wide spectrum of presentations
recurrent RTIs
fever
night sweats
faitgue
lethargy
weightloss
anorexia
conjunctivitis
episcleritis
chronic sinusitis
epistaxis
pul infiltrates
cough
haemoptysis
dyspnoea
alveolar capillaritis + diffuse haemorrhaging
myalgia
arthralgia
cresenteric necrotising glomerulonephritis
vasculitic ulcers
pericardial rub
Dx
bloods
urinalysis
ANCA detection
CXR
CT-thorax/sinus
only do Bx when presentation atypical
blood vessel Bx (fibrinoid necrosis of wall, inflamm)
lung Bx (look for necrotising vasculitis + granulomatous inflamm)
Tx
cyclophosphamide
glucocorticosteroids
rituximab
plasma exchange
maintenance tx when in remission
Eosinophilic GPA (Churg-Strauss syndrome)
rare systemic necrotising granulomatous vasculitis
affects small/medium vessels
associated with severe asthma + tissue eosinophilia (extravasc eosinophils on Bx)
pul infiltrates
mono/poly-neuropathy
paranasal sunus abnormality - allergic rhinitis
investigations
bloods
ANCA
rheumatoid factor
BAL (raised eosinophils)
CXR
CT-thorax
GI investigations (look for bleeding)
EMG (look for N damage)
Bx of involved sites
Tx
mostly steroids
less often cyclophosphamide or rituximab
symptoms
malaise
fever
myalgia
weightloss
cough
haemoptysis
arthralgia
nasal polyposis (polyps)
skin purpura (haemorrhaging of small vessels into skin)
digital ischaemia
myocarditis
pericarditis
MI
eosinophilic gastroenteritis
mononeuritis multiplex
Collagen vasc disorders
SLE, RA, dermatomyositis-polymyositis
interstitial pneumonia
diffuse fibrosis
pul hypertension
vasculitis
diffuse alv haemorrhaing
pleuritis