Stevens - Restrictive Pulmonary Disease
Terms in this set (68)
End-stage lesion seen with many pulmonary injuries, usually chronic interstitial pneumonias. The lung architecture is permanently reorganized into functionally useless tissue?
End-stage lung/honeycomb lung
Particulate material covered by hemosiderin, highlighted by iron (Prussian blue) stain. A subset of ferruginous bodies are asbestos bodies that usually have a beaded surface, club-shaped ends, and a central thin translucent core?
Laminated concretions of calcium and protein?
Stellate inclusions within giant cells?
What is seen with chronic intersitial/restrictive diease?
Pulmonary connective tissue
Inflammation and fibrosis
Peripheral and delicate interstitium
Unknown cause and pathogenesis
What are the clinical features of restrictive lung disease?
No wheezing/airway obstruction
What are the physiologic features of restrictive lung disease?
Reductions in carbon monoxide diffusing capacity
Reduction in lung volume
Reduction in compliance
What is seen on CXR for RLD?
Bilateral infiltrative lesions
Small nodules, irregular lines, ground glass shadows
What are the Fibrosing Diseases?
Idiopathic pulmonary fibrosis
Nonspecific interstitial pneumonia
Cryptogenic organizing pneumonia
What is seen microscopically in Nonspecific Interstitial Pneumonia for the cellular pattern?
Mild to moderate chronic interstitial inflammation
-lymphocytes and plasma cells
-uniform or patchy distribution
What is seen microscopically in Nonspecific Interstitial Pneumonia for the fibrosing pattern?
Diffuse or patchy interstitial fibrosis
No temporal heterogeneity (characteristic of UIP)
No fibroblastic foci
What is the clinical course of Nonspecific Interstitial Pneumonia?
Dyspnea and cough
-several months' duration
Age=46-55 years old
-good response to steroids
What is another name for Cryptogenic Organizing Pneumonia?
Bronchiolitis obliterans organizing pneumonia (BOOP)
What are the clinical features of Cryptogenic Organizing Pneumonia?
=subpleural or peribronchial patchy areas of airspace consolidation
What is Cryptogenic Organizing Pneumonia a common response to?
Inflammtory injury to lungs
-toxins,drugs CT disorder, GvH disease
How is Cryptogenic Organizing Pneumonia diagnosed?
Cause cannot be determined from biopsy
Requires clinical history
What is seen microscopically for Cryptogenic Organizing Pneumonia?
Polypoid plugs of loose organizing connective tissue
No interstitial fibrosis or honeycomb lung
Non-neoplastic disease due to inhaltion of mineral dust via occupatonal exposure to specific airborne agents?
What can cause more dust accumulation in Pneumoconioses?
What is seen with Coal Worker's Pneumoconiosis?
Simple coal workers' pneumoconiosis
Progressive massive fibrosis
What is the pathogenesis of CWP?
Contaminating silica in the coal dust
Favors progressive disease
Carbon dust=major culprit
Most innocuous coal-induced lesion in coal miners that can be seen in urban dwellers and tobacco smokers via inhaled carbon pigment that is engulfed by macrophages?
What is seen in Simple coal workers' pneumoconiosis?
Near resp bronchioles
Lead to dilation of adj alveoli
What is seen in complicated CWP?
Background of simple CWP
Develops over many years
Intensely blackened scars
Dense collagen and pigment
Center of lesion—necrosis secondary to ischemia
Caused by an inhalation of crystalline silicon dioxide from a sandblaster that presents after decades of exposure?
What is seen with acute silicosis?
Due to heavy exposure
Similar to alveolar proteinosis
Generalized accumulation of lipoproteinaceous material
What is associated with Silicosis?
↑ TB suspectibility
What are the two forms of abestos?
What risk increase occurs in abestos?
-high w/ bronchogenic carcinoma
-even higher w/ smoking
What is the clinical course of abestos?
Usually begin after 10 years of exposure
Initially—dyspnea with exertion
Later—dyspnea at rest
Dyspnea is usually accompanied by a cough with sputum production
Irregular linear densities—particularly in lower lobes (bilaterally)
What is the pathogenesis of abestos?
Initial injury is at bifurcations of small airways and ducts
Macrophages ingest fibers
Release chemotactic factors and fibrogenic mediators
Causes interstitial fibrosis
Begins as fibrosis around respiratory bronchioles and alveolar ducts
Creates enlarged airspaces enclosed with thick fibrous walls
Eventual honeycombing of that region
What are the pathologic findings of abestos?
Diffuse pulmonary interstitial fibrosis
Multiple asbestos bodies
-golden-brown, fusiform or beaded rods
-coated with iron-containing proteinaceous material
-most common manifestation
-anterior and posterolateral aspects of the parietal pleura
-over domes of diaphragm
RA pts with exposure to mining dust with inflammation and scarring of the lungs?
What is seen wit Caplan Syndrome?
-Pallisading granulomatous inflammation
What is the Tx for Cryptogenic Organizing Pneumonia?
Most need oral steroids
-6 months or longer for complete recovery
What are the two Granulomatous Diseases?
Multsystemic disease of unknown origin that involves lung in 90% of cases and is characterizd by noncaseating granulmoas that is diagnosed via exclusion?
In what demos is Sarcoidosis seen?
-rare in chinese and SEAsians
What is the clinical course of Sarcoidosis?
-routine chest films w/bilateral hilar lymphadenopathy
Insidious onset of respiratory abnormalities
-shortness of breath, cough, chest pain, hemoptysis
Constitutional signs and symptoms
-fever, fatigue, weight loss, anorexia, night sweats
-periods of activity interspersed with remissions
What are the macroscopic findings in Sarcoidosis?
Bone marrow—Phalangeal bones of hands and feet
Skin lesions - discrete subcutanous nodules
Mucous membrane lesions
Iritis or iridocyclitis
Muscle-occult sarcoid myositis
What are the microscopic findings of Sarcoidosis?
Distribution along lymphatics, bronchi and blood vessels
Spectrum of immunologically mediated interstitial lung disorders that are similar to asthma but involves alveoli instead of the bronchI?
What are the causes of Hypersensitivity Pneumonitis?
Intense, prolonged exposure to inhaled organic dusts and occupational antigens
Spores of thermophilic bacteria
Bacteria from hay, grain, sugar cane, bark, cheese, cork, or animal feces
What syndromes are associated with Hypersensitivity Pneumonitis?
Air conditioner (humidifier) lung
Pigeon breeder's lung (bird fancier's disease)
Maple bark stripper's lung
Thermophilic bacteria in heated water reservoirs?
Air conditioner lung
Moldy hay containing spores of thermophilic actinomycetes?
Proteins from serum, feathers, excreta of birds?
Pigeon breeder's lung
Maple bark stripper's lung
What does exposure result in for Hypersensitivity Pneumonitis?
Early formation of type III immune complexes
Presence of noncaseating granulomas
T cell-mediated (type IV) delayed-type hypersensitivity
What are the microscopic findings for Hypersensitivity Pneumonitis?
Interstitial bronchiolocentric pneumonitis
-lymphocytes, plasma cells, foamy macrophages
--interstitium, alveolar space, terminal airways
Nodules of organizing fibroblasts
What are the categories of Pulmonary Eosinophilia?
Acute eosinophilic pneumonia with respiratory failure
Simple pulmonary eosinophilia (Loffler syndrome)
Tropical eosinophilia (infection with microfilariae)
Idiopathic chronic eosinophilic pneumonia
What is characteristic of acute eosinophilic pneumonia with respiratory failure?
Acute illness with unknown cause
Fever, dyspnea, hypoxemic respiratory failure
Chest x-ray w/ diffuse infiltrates
Bronchoalveolar lavage fluid
-contains more than 25% eosinophils
Prompt response to corticosteroids
What is characteristic of Simple pulmonary eosinophilia (Loeffler syndrome)?
Transient pulmonary lesions
Eosinophilia in blood
Benign clinical course
CT scan w/ irregular intrapulmonary densities
Thickened alveolar septa
-eosinophils and occasional giant cells
No necrosis, vasculitis, or fibrosis
What is characteristic of Tropical eosinophilia?
Infection with microfilaria of Wuchereria bancrofti
-circulate in pulmonary capillaries
-cause immediate type of hypersensitivity reaction
Restricted to tropical regions
What are the microscopic findings of Tropical eosinophilia?
Microfilaria within pulmonary capillaries
Marked eosinophilic infiltrate
What is characteristic of Secondary eosinophilia?
Parasitic, fungal, and bacterial infections
Association with asthma
Allergic bronchopulmonary aspergillosis
What are the Smoking-associated Interstitial Lung Diseases?
Desquamative interstitial pneumonia
Respiratory bronchiolitis-associated interstitial lung disease
Presents in the 4th/5th decade of life in man w/ Hx of cigarette smoking that present with insidious onset of dyspnea and dry cough alogn with clubbing and cyanosis?
Desquamative Interstitial Pneumonia
What is seen microscopically with Desquamative Interstitial Pneumonia?
Diffuse collections of intraalveolar macrophages
Originally thought to be desquamated pneumocytes
Dusky brown pigment ("smokers' macrophages")
Finely granular iron
Thickened alveolar septa
Common histologic lesion in cigarette smokers with pigmented intraluminal macrophages in the 1st and 2nd order bronchioles?
Respiratory Bronchiolitis-Associated Interstitial Lung Disease
Rare disease with bilateral patcy asymmetric pulmpnary opacifications and accumulation of acellular surfactant?
Pulmonary Alveolar Proteinosis
What is coughed up with Pulmonary Alveolar Proteinosis?
Gelatinous chunks of sputum
What is seen in Acquired Pulmonary Alveolar Proteinosis?
90% of all cases
Lacks familial predisposition
What is seen in Congenital Pulmonary Alveolar Proteinosis?
Rare cause of immediate-onset neonatal respiratory distress
Full term infant
Rapidly develops progressive respiratory distress
Shortly after birth
Mutations (up to now): Multiple genes
Tx: Lung transplantation
Without it, death ensues between 3 and 6 months of age
What is seen in Secondary Pulmonary Alveolar Proteinosis?
Lysinuric protein intolerance
Other inhalational syndromes
Tx= whole lung lavage
What are the 3 classes of Pulmonary Alveolar Proteinosis?
What is seen microscopically for Pulmonary Alveolar Proteinosis?
Homogeneous, granular precipitate
Causes focal to confluent consolidation of large areas of the lungs
Minimal inflammatory reaction
What is seen grossly for Pulmonary Alveolar Proteinosis?
Turbid fluid exudes from cut surface
Increase in size and weight of lung