Restrictive Pulmonary Disorders Flashcards
Lung restriction due to causes outwith the lungs eg.s
Skeletal - kyphoscoliosis, ankylosing spondylitis, rib #
Muscle weakness - myaesthenia gravis, Guillain-Barre
Obesity/ascites
Rectrictive lung diseases affect lung ____
interstitium/parenchyma - tissue and space around alveoli
effect of lung restriction arising outwith the lungs
alveolar under ventilation
low arterial Sa+PaO2 and raised PaCO2
reduced lung vol
ILD overall effect
impaired alveolar gas exchange
low Pa+SaO2 and normal PaCO2
alveolar ventilation is normal
6 broad causes of ILD
fluid consolidation inflammatory infiltrate Dust carcinomatosis eosinophilic
Causes of fluid in lung interstitium
LHF
altitude sickness
sepsis
ARDS
Causes of consolidation of alveolar air spaces => ILD
pneumonia
infarction - PE/vasculitis
BOOP/COP - crytogenic organising pneumonia
Causes of inflammatory infiltrate in alveolar walls (alveolitis -> ILD)
Granulomatous alveolitis - EAA, Sarcoidosis
Drug induced - amiodarone, bleomycin, methotrexate, gold
toxic fumes - Cl2
fibrosing alveolitis - rheumatoid, IPF
AI - SLE, Wegener’s, Churg-Strauss, Behcets
Eg.s of pneumoconiosis - dust ILD
fibrogenic - asbestosis,silicosis
non-fibrinogenic - baritosis
Spirometry pattern of restrictives
FEV1 reduced FVC reduced FEV1% and peak flow normal DLCO reduced Pa+SaO2 reduced
___ and ____ raised in sarcoidosis
serum ACE and Ca2+
Important Hx points in ILD
pets, drugs, occupation, arthritis
Tests for ILD
Spirometry
Bloods - antibodies, serum ACE, Ca2+
CXR - LV hypertrophy, bilateral infiltrates
ECG - LHF
CT - inflam = groundglass ; fibrotic nodules
BAL/sputum
biopsy
Treatments for ILD
1- remove trigger Immunosuppressants: 2 - systemic corticosteroids 3 - PO azathioprine, anti-fibrotics (pirfenidone, nintendanib) + anti-ox (acetylcysteine) for IPF O2 if needed 4- transplant
EAA/_____ is a ___ hypersensitivity reaction
eg.s
hypersensitivity pneumonitis
Type 3 - immune complex
Farmer’s/pigeon fancier’s lung
Sarcoidosis is more/less common in smokers
less
sarcoidosis commonly affects
lungs, lymph nodes, joints, liver, skin, eyes
acute sarcoidosis signs and symptoms
treatment
erythema nodosum, bilateral hilar lymphadenopathy, arthritis, uveitis, parotitis, fever
NO TREATMENT
chronic sarcoidosis signs and symptoms
treatment
alveolitis, skin infiltrations, peripheral lymphadenopathy, hypercalcaemia
Oral steroids, immunosuppression (anti-TNF, azathioprine, methotrexate)
Diagnosis of Sarcoidosis tests
CXR = bilateral hilar lymphadenopathy
CT = peripheral nodular infiltrate
Biopsy = non-casseating granuloma
Bloods - increased serum ACE (activity marker) and Ca2+, inflammatory markers
2ndry causes of pulmonary fibrosis -
rheumatoid, SLE, systemic sclerosis, asbestos, drugs
IPF presentation:
progressive SOB, dry cough, clubbing, bilateral fine inspiratory crackles, PFT restrictive pattern
IPF imaging:
CXR bilateral infiltrates
CT reticulonodular fibrotic change worse at bases
Drugs to slow progression of IPF
N-acetyl cisteine has anti-fibrotic effects
