Restrictive Diseases Flashcards
Restrictive disease
TLC decreases
decrease in FVC and FEV1 but more so in FVC so the FEV1:FVC ratio stays the same or is increased (greater than 80%)
what is the most common cuases of restrictive disease
interstitial disease - interstitial fibrosis
may also arise with chest wall abnormality (obesity)
ideopathic pulmonary fibrosis
fibrosis of lung interstitum
- etiology is releated to cyclical lung injury (and healing)
- TGF-b from injured pneumocytes induce the fibrosis
- secondary cuases such as drugs (bleomycin and amiodarone) and radiation therapy must be excluded before making the diagnosis
clinical featuers of ideopathic pulm fibrosis
progressive dyspnea and cough
fibrosis on lung CT - usually just starts in the sub pleura region but can progress to include the entire lung
treatment is lung transplantation
cobble stone pleural surface
intralobular - sub pleural
pneumoconioses
- interstitial fibrosis due to ocupational exposure (cuasing the macrophages to lay down fibrosis)
- requires chronic exposure to small particals that are fibrogenic
- must be small enough to get to the bottom of the lung
- small particals get taken up by macrophages to induce the fibrosis
things that can cuase pneumoconioses
- coal workers pneumoconiosis - carbond dust
- leads to diffuse fibrosis (black lung) lung shrinks becuase of fibrosis, associated with rheumatoid arthritis (caplan syndrome)
- mild exposure = anthracosis (dark macrophages in lung and LNs = not clinically significant
- Silicosis - seen in sand blasters and silica miners
- fibrotic nodules in upper lobes of the lung
- increased risk for TB (only one that does this), silica impares phagolysosome formation by macrophages
- berylliosis - seen in beryllium miners and workers in aerospace industry
- noncaseating granulomas in the lung, hilar LNs and systemic organs (sounds like sarcoidosis but it is driven by beryllium)
- it also increases risk of lung cancer
- asbestosis - construction workers, plumbers and shipyard workers
- can cuase fibrosis of lung or pleura(plaques) and cancer of the lung or pleura (mesothelioma)
- these patients are much more likely to get lung cancer than mesothelioma
- can see asbestos bodies histologically
- greeks and people from turky are more likely
- can cuase fibrosis of lung or pleura(plaques) and cancer of the lung or pleura (mesothelioma)
sarcoidosis
- systemic disease characterized by noncaseating granulomas in multiple organs (usually lung and hilar LNs)
- leads to restrictive lung disease
- other commonly involved tissues
- uvea (uveitis) - eye
- skin (cutaneous nodules or erythema nodosum)
- salivary and lacriminal glands
- almost any tissue can be involved
- classically seen in AA females
- etiology is unknown but probably due to CD4 helper T cell response to unknown antigen
- can see epitheiliodi histeocytes with giant cells, astroid bodies can also be seen sometimes also showmans bodies
- high ACE and high calcium
clincal features of sarcoidosis
- dyspnea or cough
- elevated serum ACE
- hypercalcemia - occurs becuase the granulomas can activate Vit D - this will happen with any disease that has noncaseating granulomas
- treatment is steriods; often resolves spontaneously without treatment
hypersensitivity pneumonitis
- granulomatous reaction to inhaled organic antigens (pideon breeders lung) - but there are eosinophils associated with the granuloma (because this is a hypersensitive reaction)
- farmers lung - moldy hay
- bagassosis - moldy sugar cain
- humidifie lung - thermophilic bacteria in heated water resevours
- presents with fever, cough, and dyspnea hours after exposure; resolves with removal of the exposure
- chronic exposure leads to interstitial fibrosis
- if you take away stimuli before this then it resolves
- type 3/ type 4 immune response
Cryptogenic organizing pneumonia
unknown etiology
polyploid plugs of loose organizing CT within small airways
treat with steriods - may also resolve on its own
Langerhans cell histiocytosis
birbeck granules (tenssis racket shaped) cells on electron microscopy
seen in adults who smoke
pneumothorax is a classic complication
pulmonary alveolar proteinosis
autoimmune (acquired) APA - most common type
- anti GM-CSF antibodies - blocks macrophages ability to digest surfactant - you get accumulation of acellular surfactant in intra alveolar and bronchiolar spaces
- when you do a CXR you will have bilateral opacities (in both lung)
Respiratory bronchiolysis
- presents in 30s and 40s males and females who has excessive cigarette smoking
- macrophage infiltrates whith rare eosinophils, mucostasis and atelectasis
- CXR = bilateral reticulnodular infiltrates; ground galss alveolar inflitrates focally
- positive response to steriods; stop smoking - very good survival rate 100%
usual interstitial pneumonia
same as ideopathic pulm fibrosis