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CR - Interstitial Lung Disease (ii) (Other rare causes of ILD (progressive…
CR - Interstitial Lung Disease (ii)
Signs
hands
digital clubbing
common in (70%) IPF + asbestosis
rare in sarcoidosis
sclerodactaly in scleroderma
localised thickening/tightness of skin of fingers + toes (can lead to ulceration)
vasculitic phenomena -> splinter haemorrhages in RA + SLE
Raynaud's in SLE + scleroderma
symmetrical small joint arthropathy + onycholysis (nail separates from skin underneath it) in RA
gottron's papules (erythema of knuckles) in dermatomyositis
hyperkeratonic (mechanic's, rough, cracking) hands in progressive aggressive ILD
head + neck
butterfly/malar rash
red-purple on cheeks + bridge of nose
SLE but not pathognomonic
signs in sarcoidosis
lupus pernio (purple lesion on face)
parotid swelling
LMN VII palsy
iritis
keratoconjunctivitis sicca (dry) in sacroidosis + RA
uveitis (dry eye + irregular pupil) in sarcoidosis
signs in scleroderma
telangiectasia (widened venues -> spider appearance)
microstomia
beaking of nose
chest
rapid shallow breathing
central cyanosis
tracheal deviation
decreased chest expansion + diameter
tactile fremitis normal/increased
percussion normal/dull
bilat fine end inspiratory creps (VELCRO CRACKLES)
cor pulmonale
rare
due to pul hypertension
parasternal heave
increased JVP
erythema nodosum
painful red rash on ant shins
sarcoidosis
Investigations
CXR
bilat intersitial infiltrates
may be patchy/nodular
start peripherally
occasionally may be asymmetrical
look for signs of CHF
prominent hilar vessels
upper lobe diversion (elevation of left atrial pressure - stag-antler's sign)
small pleural effusions (blunted costo-phrenic angles)
cardiomegaly
seldom pathognomonic - simply suggests ILD
10% normal (usually vary)
compare with previous CXRs if possible
HRCT
if there's 3 indicators of ILD but no cause = IPF (Dx of exclusion)
gold-standard
course reticular opacities, patchy, GROUND GLASS
honeycomb cysts (scar tissue)
traction bronchiectasis (seoncdary, bronchi pulled due parenchymal fibrosis)
central bronchovasc thickening in sarcoid
peripheral fibrosis in COP (cryptogenic organising pneumonia), asbestosis, connective tissue diseases
blood tests
often exclude other causes
FBC
CRP/ESR (inflamm in autoimmunity)
U+E
liver + bone profile (incl Ca2+)
autoIgs
rheumatoid factor
anti-centromere
anti-GBM (goodpasture's)
ANA (anti-nuclear Igs)
dsDNA
anti-SCL70 (anti-topoisomerase)
ANCA
Myositis-specific Igs (MSAs) detected with a myositis panel
ACE level (used to Dx sarcoidosis - not used much anymore)
ABG
type 1 resp failure or normal
6 min walk test
also done in COPD
assesses exertional desaturation
bronchoscopy
Bx rarely
must be done for sarcoid/suspected malignancy
EBUS-TNA of mediastinal nodes
not always indicated
ECG (detects pul hypertension)
PFTS
spirometry
restrictive
both FEV1 + FVC reduced (esp FVC), so fraction normal/increased/preserved (>80%)
if obstructive in ILD its sarcoidosis or coexisting COPD
diffusion lung capacity
reduced in alveolar disease, anaemia, emphysema
reduced total lung capacity
Pneumoconiosis
alveolitis + fibrosis in response to inorganic dusts
upper zone predominant
occupational
CWP
can progress to massive fibrosis
silicosis
Caplan's syndrome (coexisting RA)
Pul manifestations of RA
fibrosis (similar pattern to IPF but not idiopathic)
pul nodules
pleuritis
exudative pleural effusions
Radiation pneumonitis
dose + frequency related
onset = 6 months post Tx
look for STRAIGHT LINES on CXR
may be steroid responsive
Other rare causes of ILD
progressive systemic sclerosis
Wegener's GPA
Churg-Strauss syndrome (eosinophilic GPA)
polymyositis
dermatomyositis
lymphangioleiomyomatosis
chronic eosinophilic pneumonia
eosinophilic granuloma
pul alveolar proteinosis
lypmocytosis interstirial pneumonia