SFP: restrictive lung disease Flashcards
Generally, describe restrictive airway diseases
The expansion of the lung is hindered either from something external to the lung or within the lung (often the latter)
What do restrictive lung diseases do to FVC, FEV1, and the FEV1/FVC ratio
Decrease FVC and FEV1 about equally, so the ratio is preserved
What is diffuse alveolar damage (DAD)
The pathological pattern caused by ARDS that is characterized by rapid onset of life-threatening respiratory failure
What do we see in the exudative (early) stage of DAD
Edema; Heavy and wet lungs with hyaline membranes
What tends to happen to alveoli in the exudative stage of DAD
They collapse
What do we see in the proliferative stage of DAD
Type II pneumocyte proliferation, interstitial fibroblasts, macrophages, edema; grayish lungs
What do we see in the fibrotic stage of DAD
Fibrosis and honeycomb appearance; dense interstitial collagen
In what stage of DAD do macrophages accumulate
Proliferative
What is nonspecific interstitial pneumonia
A pneumonia usually found in younger patients that is associated with autoimmune diseases
What is the onset of non-specific interstitial pneumonia (NSIP)
Acute
What is the histology of NSIP
Lymphocytes, plasma cells, macrophages can be seen as well as foci of cryptogenic organizing pneumonia
What may cause NSIP
Drugs or autoimmune diseases
What is the prognosis of NSIP
It varies, but better than UIP
What is usual interstitial pneumonia
An idiopathic pneumonia in older patients that is difficult to treat; it’s the worst of the fibrosing interstitial pneumonias. It progresses over months to years.
What is seen on x-ray in UIP
Bilateral interstitial disease
Describe the histology of UIP
Temporal heterogeneity (fibrosis of different ages), honeycombing, minimal inflammation, fibroblast
How do we treat UIP
We kinda don’t…many get transplants or die
What is cryptogenic organizing pneumonia
Polyps of young connective tissue in airspaces or airways
Why is cryptogenic organizing pneumonia (COP) considered interstitial?
It leads to interstitial inflammation
What is seen microscopically in COP
Fibroblasts and collagen
What is the pattern of desquamative interstitial pneumonia
Diffuse, bilateral, and in the lower lobes
What are the smoking related interstitial lung diseases
Desquamative interstitial pneumonia and respiratory bronchiolitis
Describe the histology of desquamative interstitial pneumonia (DIP)
Accumulation of macrophages and some lymphocytes and eosinophils. There is fine lamellar fibrosis
How can we treat DIP
Steroids
What is respiratory bronchiolitis interstitial lung disease
Often develops before DIP, has the same underlying cause, but is just less widespread.
What are the two granulomatous interstitial lung diseases
Sarcoidosis and hypersensitivity pneumonia
Describe hypersensitivity pneumonia
Looks like NSIP but with granulomas. It may lead to interstitial fibrosis and may have features of COP
What are common clues for hypersensitivity pneumonia
Farmers, hot tubs, working with or having birds, humidifiers
What types of hypersensitivity reactions are seen in hypersensitivity pneumonia
3 (serum specific antibodies) and 4 (granuloma)
What is the key treatment for HP
Limiting exposure to antigen
Describe sarcoidosis
Usually in younger patients and involved granulomas in the lungs that follow lymphatic distribution. May also be big lymph nodes in the mediastinum
What is distinctive about sarcoid
It tends to be systemic!
What are associated findings of sarcoid
Hypercalcemia from granulomas, elevated ACE, T cells that are reduced in blood and elevated in lungs
