Restrictive Lung Disorders Flashcards
Spirometry findings in Restrictive lung disorders
TLC decreases
FVC decreases
FEV1 - Normal or slightly decreased
FEV1/FVC Increases
Fibrosis - decreased lung compliance
Diffusion capacity decreases
Causes of Restrictive lung disorders can be classified into
Extra parenchymal causes
Parenchymal cause
Extra parenchymal causes in RLD includes
Chest wall disorders - Obese/ Kyphosis/ Ankylosis spondylitis
Neuromuscular disorders - Diaphragmatic palsy, Myasthenia gravis, Gullian Barre syndrome
Muscular dystrophy
Parenchymal cause of RLD can be classified to
Acute
Chronic
Acute parenchymal causes of RLD
ARDS
Chronic parenchymal causes of RLD can be divided to
Fibrosing
Granulomatous
Eosinophilic
Smoking related
Fibrosing parenchymal causes include
Idiopathic pulmonary fibrosis (IPF)
Non specific interstitial pneumonia (NSIP)
Cryptogenic organising pneumonia (COP)
Pneumoconiosis
Granulomatous parenchymal causes includes
Hypersensitivity pneumonitis
Sarcoidosis
Eosinophilic parenchymal causes includes
Loffler syndrome
Drug allergy
Smoking related parenchymal causes includes
DIP
Resp. Bronchiolitis
Idiopathic pulmonary fibrosis also termed as
Cryptogenic fibrosing alveolitis
Pathogenesis of Idiopathic pulmonary Fibrosis (IPF)
Environmental factors (GERD/Smoking etc) - alveolar epithelial cell injury (Alveolitis) - TGF- beta secretion - Fibrous tissue deposition
Lobes involved in IPF
Bilateral Lower lobes
Subpleural location
Along interlobular septum
Commonly affected age group in IPF
Elderly patients
Clinical features of IPF
M > F
Dyspnea on exertion
Dry cough
Dry inspiratory crackles
Diagnosis of IPF
Clinical Presentation
Surgical biopsy
Radiology - HRCT Scan
Findings of surgical biopsy in case of IPF
Usual interstitial pneumonia
Patchy interstitial fibrosis
Architecture distortion
Honeycombing pattern microscopically
Treatment of IPF
Lung transplant
Pirfenidone - inhibits TGF-beta activity
Nintedanib
In case of massive fibrosis in IPF
Leads to death in 3 years
Cause of Non specific Interstitial pneumonia (NSIP)
Idiopathic
NSIP is associated with connective disorders except
Rheumatoid arthritis
Clinical features of NSIP
Dry cough
Progressively increasing dyspnea
Age - 50-60 years
NSIP commonly seen in
Non smoker females
Heterogenous fibroplastic foci is seen in
IPF
Diagnosis of NSIP
HRCT Scan
Surgical biopsy
Most common lobe involved in NSIP
Lower lobe
Surgical biopsy findings in NSIP
Uniform/homogeneous involvement - same pattern
Cellular variant of NSIP includes
Chronic Inflammatory cells +ve
Fibrous tissue -ve
Fibrosing NSIP includes
Chronic Inflammatory cells + fibrosis +ve
Clinical features of Cryptogenic Organising pneumonia (COP)
Cough
Dyspnea