Restrictive Lung Diseases Flashcards
What is the physiological definition of restriction?
Forced vital capacity <80% of normal
What happens to all the volumes and capacities during restrictive lung disease?
They all get smaller
What is the marker of restriction?
Vital capacity
What is the marker of obstruction?
FEV1
What are anatomical causes of restriction?
Lung Pleura Muscle Bone Other
What are lung causes of restriction?
Interstitial lung diseases (idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis)
What are the pleural causes of restriction?
Pleural effusions (fluid in pleural space), pneumothorax (air in pleural space), pleural thickening
What are skeletal causes of restriction?
Kyphoscoliosis
Ankylosis spondylitis
Thoracoplasty
Rib fractures
What are muscular causes of restriction?
Amyotrophic lateral sclerosis
What are subdiaphragmatic causes of restriction?
Obesity and pregnancy
Where is the interstitium?
Tiny space between epithelium of alveoli and endothelium of the capillary
What are interstitial lung diseases?
Thickening of the interstitium lead to pulmonary fibrosis (lung fibrosis)
What is the shitologivsl hallmark for sarcoidosis?
Non caseating granuloma
What does erthyma nodosum mean?
Red nodules (lumpy)
What are the differential diagnoses for erythema nodosum?
Sarcoidosis
Infection
Some medications
Are granuloma found in the biopsy of erythema nodosum?
No
If a patient is suspected to have sarcoidosis what must you do to their eyes?
Have them checked by a spec list like an optician.
Where do granulomatus traditionally locate?
In areas of skin trauma such as tattoos or scars
Who normally gets sarcoidosis?
Under 40s
Women > Men
World wide
How do you investigate it?
History and examination CXR Pulmonary function tests Bloods Urine analysis Eye exam
What do you see in CXR of sarcoidosis?
Enlarged glands
What is seen in CXR of stage 2 disease in sarcoidosis?
Spotty lungs
What is seen in CXR of stage 3 disease in sarcoidosis?
Interstitial change
What is seen in CXR of stage 4 disease in sarcoidosis?
Fibrotic lung disease
What further assessment can be done for sarcoidosis?
Bronchoscopy including trans bronchial biopsies and endobronchial ultrasound
What surgical biopsies may be needed?
Medistinoscopy
Video assisted thoracoscopic lung biopsy
How is mild disease in sarcoidosis treated?
No treatment
How is erythema nodosum treated from sarcoidosis?
NSAIDS
How are skin lesions from sarcoidosis treated?
Topical steroids
How do you treat cardiac, neurological and eye disease that isn’t responding to topical rx in sarcoidosis?
Systemic steroids
What is the typical symptoms for idiopathic pulmonary fibrosis?
Chronic breathlessness and cough
What is the typical age group for Idiopathic pulmonary fibrosis?
60-70 years of age
What are the main signs of idiopathic pulmonary fibrosis?
Finger clubbing and crackles that don’t go away.
What is the median survival for idiopathic pulmonary fibrosis?
3 years
What drugs can be used to slow down the lung fibrosis in idiopathic pulmonary fibrosis?
Oral anti-fibrotic
Pirnfenidone
Nintedanib
Palliative care
What are surgical options for idiopathic pulmonary fibrosis?
Transplant