Restrictive lung disease Flashcards
What is the main restriction of RLD?
Reduced lung volume
What happens to the FEV/FVC in RLD?
It goes up as airflow (FEV) is preserved and FVC goes down
Lung function tests in RLD =
Reduced TLC and FVC. Preserved FEV (airflow). Gas transfer factor can be reduced in the case of parenchymal disease or normal in RLD
What causes hypoxia in intrinsic RLD?
VQ mismatch
What causes hypoxia in extrinsic RLD?
VQ mismatch in areas of atelectasis
What is atelectasis?
The complete or partial collapse of the entire lung an area of the lung
What happens to compliance in extrinsic RLD?
Compliance decreases
Main feature of interstitial lung disease?
Matrix deposition in the interstitium
ILD leads to which range of conditions?
Pneumonia
Which bloods might be useful in RLD?
FBC, Antinuclear Abs and RhF (collagen vascular disorders), creatine kinase (polymyositis), antineutrophilic cytoplasmic antibodies (vasculitis), and antiglomerular basement membrane antibody (Goodpastures syndrome)
Which investigations?
Bloods, CXR/HRCT, Lung function tests, ABG (O2 sats), biopsy, bronchoalveolar lavage
What might a CT of a patient with non-specific interstitial pneumonia show?
Ground glass opacities and fibrosis
Treatment options for idiopathic pulmonary fibrosis?
Pirfenidone (oral, antifibrotic) Nintedanib (triple tyrosine kinase inhibitor) Pulmonary rehabilitation Palliative care Lung transplant
Treatment for pneumonias: NSIP, DIP, AIP, COP?
Steroids and immunosuppressants
How might Sarcoidosis present?
Non-productive cough, SOB, sometimes wheeze, pain on the shin (Loefgren’s syndrome), erythema nodosum, arthralgia
Inflammatory RLD?
Sarcoidosis
Which cell drives Sarcoidosis
T-cell
Which enzyme is increased in Sarcoidosis?
ACE
Investigations for Sarcoidosis?
CXR, serum ACE, Ca, LFTs, U+Es, FBC (bone marrow suppression), biopsy (EBUS, skin lymph node), lung function test, CT/PET/cardiac MRI
What histological finding would suggest Sarcoidosis?
Granulomas
What is a poor prognostic factor in Sarcoidosis?
Pulmonary hypertension
Differentials for the granulomas in Sarcoidosis?
TB and lymphoma
What might a CXR in Sarcoidosis reveal?
Bilateral hilar lymphadenopathy
Drugs categories for Sarcoidosis?
Steroids, immunosuppressants and biologics
Drugs used to treat Sarcoidosis:
Prednisolone
Methotrexate, hydroxychloroquine (anti-malarial), mycophenolate, azathioprine
Anti-TNF, nfliximab, adalimumab
Nothing
