Restrictive Lung Diseases

Terms in this set (97)

What is the typical healing outcome in uncomplicated bacterial pneumonia?
Complete resolution is the usual outcome, because the alveolar wall is not damaged.
What is the typical healing outcome in viral pneumonia?
Severe viral pneumonia is more likely to result in fibrosis.
What is the typical healing outcome with significant or continuous injury to the alveolar wall?
It may lead to interstitial fibrosis and remodeling of the lung.
What is the root of the pathogenesis of restrictive lung disease?
-Alveolitis is the primary problem regardless of the type or specific cause of interstitial disease.
What process of alveolitis leads to the development of restrictive lung disease?
In severe or ongoing low-grade alveolitis healing by fibrosis leads to loss of functioning lung parenchyma and results in respiratory insufficiency.
What is the key event in the development of pulmonary fibrosis?
Activation of pulmonary macrophages.
Describe the process of pulmonary fibrosis development (3).
-Chemoattractants from activated macrophages activate and recruit PMNs (e.g. IL-8 and leukotriene B4 )
-Oxidants and proteases from macrophages and PMNs injure type I pneumocytes and degrade lung scleroproteins
-Type II pneumocytes proliferate and repair the epithelium but also secrete chemotactic and growth factors that attract more macrophages to alveoli and stimulate fibroblast growth (platelet-derived growth factor, TGF-β and others)
What are the major categories of chronic interstitial lung disease (8)?
-Fibrosing diseases
-Granulomatous inflammation
-Occupational and environmental exposure
-Drug- or treatment related
-Post DAD
-Immunologic lung disease
-Idiopathic pulmonary fibroses
-Eosinophilic
-Smoking-related
-Other
List the fibrosing diseases (7).
-Pneumoconioses
-Collagen vascular diseases associated lung diseases
-Drug reactions
-Radiation pneumonitis
-Idiopathic pulmonary fibrosis (IFP) aka cryptogenic fibrosing alveolitis -Cryptogenic organizing pneumonia (COP), aka bronchiolitis obliterans organizing pneumonia (BOOP)
-Non-specific interstitial pneumonia
What diagnostic criteria are required for the diagnosis of idiopathic pulmonary fibrosis?
Histologic pattern of usual interstitial pneumonia (UIP) and exclusion of all other causes.
List the diseases of granulomatous inflammation (2).
-Sarcoidosis
-Hypersensitivity pneumonitis
List the interstitial lung disease that can result from occupational or environmental exposure (4).
-Asbestosis
-Silicosis
-Coal worker's pneumoconiosis
-Hypersensitivity pneumonitis
List the typical causative agents of drug or treatment related interstitial lung disease (3).
-Chemotherapeutic agents
-Radiation
-Oxygen
List the immunological interstitial lung diseases (5).
-Sarcoidosis
-Collagen vascular diseases
-Wegener granulomatosis
-Goodpasture syndrome
-Allograft rejection
List the eosinophilic interstital lung diseases (7).
-Eosinophilic pneumonia
-Loffler syndrome
-Chronic eosinophilic pneumonia
-Tropical eosinophilia
-Secondary eosinophilia due to parasitic, fungal, and bacterial infections
-Asthma
-Drug allergies
List the smoking related interstitial lung diseases (2).
-DIP
-Respiratory bronchiolitis
Which interstitial lung disease falls under the category of other?
Pulmonary alveolar proteinosis.
Image of severe lung fibrosis secondary to rheumatoid arthritis.
What are pneumoconioses?
Diseases induced by inorganic and organic particulates and chemical fumes and vapors.
What is the development of pneumoconiosis dependent on (3)?
-The amount of dust retained in the airways and lung parenchyma
-The size and shape of particles
-Additional effects of other irritants, such as tobacco smoke
What are the most dangerous particles in terms of the development of pneumoconiosis?
Particles that measure 1-5μm, because they can reach the terminal airways and alveoli.
List the air pollutants associated with lung disease (5).
-Mineral dusts
-Organic dusts that induce hypersensitivity pneumonitis
-Organic dusts that induce asthma
-Chemical fumes and vapors
-Urban air
List the mineral dusts associated with lung disease (7).
-Coal dust
-Silica
-Asbestos
-Beryllium
-Iron oxide
-In oxide
-Barium sulfate
List the organic dusts that induce hypersenitivity pneumonitis that are associated with lung disease (3).
-Moldy hay
-Bagasse
-Bird droppings
List the organic dusts that induce asthma that are associated with lung disease (4).
-Cotton
-Flax
-Hemp
-Red cedar dust
List the chemical fumes and vapors associated with lung disease (5).
-Nitrous oxide
-Sulfur dioxide
-Ammonia
-Benzene
-Insecticides
Image of anthracosis (coal workers' pneumoconiosis). There is pigment in the macrophages within the bronchial wall and in the peribronchovascular lymphatics due to inhalation of carbon particles (smoker). Note the lack of fibrosis.
Image of silicosis. The specimen is from the lung of a construction worker. Note the silicotic nodules and thickened fibrotic pleura.
Image of silicotic nodules.
Image of silicotic nodules. Note the compressed pulmonary artery lumen and the fibrinoid necrosis in the center of the nodule.
Image of silicosis with a silicotic nodule composed of fibroblasts and macrophages contains bi-refringent crystalline material and carbon.
What is anthracosilicosis?
Anthracosis combined with silicosis. It results from an accumulation of carbon and silica in the lungs from inhaled coal dust.
Image of anthracosilicosis and bullous emphysema from mixed dust pneumoconiosis.
Image of anthracosilicosis with a silicotic granuloma.
Image of anthracosilicosis. Note the carbon accumulation and severe fibrosis due to silica.
Image of a perivascular silica nodule that resulted from recreational silicosis in an IV drug user.
Image of perivascular silica nodules in an IV drug user. (Polarized light)
Image of a ferruginous body. Club-shaped asbestos body with a central asbestos fiber, which is coated with iron. Macrophages are trying to phagocytose the asbestos fiber/asbestos body and die trying releasing growth factors that stimulate fibroblast proliferaton.
Is the finding of asbestos bodies enough criteria for a diagnosis of asbestosis?
No.
Image of chest x-ray of asbestosis with pleural plaques.
Image of chest x-ray of asbestosis with pleural plaques. Lateral view.
Image of asbestosis with pleural plaques. These are often more prominent in the diaphragmatic pleura.
Image of dense fibrotic pleural plaque.
Image of asbestosis. Note the thickened pleura interstial fibrosis and black asbestos bodies.
Image of close up of asbestos bodies.
Image of hypersensitivity pneumonitis (extrinsic allergic alveolitis). Note the increased number of mast cells (dark pink) and lymphocytes. The lighter pink cells are eosinophils.
In which restrictive lung conditions does the mast cell population increase (2)?
-Hypersensitivity pneumonitis
-Fibrosing lung diseases
Image of lymphocytosis in hypersensitivity pneumonitis. There are more CD8+ T cells than CD4+ T cells. CD4+ (helper) T-cells = 29%. CD8+ (suppressor) T-cells = 83%.
Image of poorly formed granulomas with multinucleated giant cells in hypersensitivity pneumonitis. There is chronic interstitial inflammation and fibrosis.
Image of hypersensitivity pneumonitis (extrinsic allergic alveolitis). Note the interstitial lymphoplasmacellular infiltrates and intra-alveolar granulomas.
Image of pulmonary fibrosis in bird breeder's disease. The lung parenchyma is compact and stiff. The pleural surface is nodular. This is an example of how hypersensitivity pneumonitis can lead to severe pulmonary fibrosis.
Image of a welder's lung. Focal interstitial accumulations of siderophages and silicoanthracotic pigment, moderate fibrosis. Note the pigment deposits of carbon and iron from welding smoke and fumes.
Image of siderophages seen in a welder's lung.
What are some other names for idiopathic pulmonary fibrosis (2)?
-Usual interstitial pneumonia (UIP)
-Cryptogenic fibrosing alveolitis
What is the etiology of idiopathic pulmonary fibrosis?
Unknown. An immune mechanism is suspected.
What are the clinical features of idiopathic pulmonary fibrosis (2)?
-Insidious onset
-Patchy recurring alveolitis, which heals by fibrosis is the basis of the disease
What characterizes the morphology of idiopathic pulmonary fibrosis (2)?
-Diffuse interstitial fibrosis
-Lesions of different ages are typical
Which patients are most commonly affected by idiopathic pulmonary fibrosis (2)?
-Males are affected more often than females
-60% of patients are older than 60 at the time of presentation
Image of chest x-ray of usual interstitial pneumonia with reduced lung volume and reticulo-nodular infiltrates, which are more prominent in the lower lobes.
Image of CT scan of usual interstitial pneumonia with reduced lung volume and reticulo-nodular infiltrates, which are more prominent in the lower lobes.
What is clubbing?
It is a deformity of the fingers and fingernails associated with a number of diseases, mostly of the heart and lungs.
Which types of restrictive lung disease is clubbing commonly seen with (2)?
-Idiopathic pulmonary fibrosis
-Asbestosis
Image of remodeling of the lung in usual interstitial pneumonia. Severe progression of remodeling of the lung results in end stage "honeycomb" lung.
Image of usual interstitial pneumonia. Prominent interstitial fibrosis accompanied by sparse mononuclear inflammatory infiltrates. Irregular air spaces are lined by reactive bronchiolar and type II cells with nuclear atypia, which may be incorrectly interpreted as malignancy.
Image of an area of active fibrosis in a lung with usual interstitial pneumonia.
Image of the fibroelastotic thickening of the interstitium. The elastic stain helps in the recognition of the original alveolar walls and blood vessels.
Image of a honeycomb lung in interstitial pulmonary fibrosis. This fibrotic lung is smaller than normal and has a nodular pleural surface. The cut surface of the fibrotic lung shows honey-comb-like thick-walled air spaces and no normal alveoli.
What is another name for cryptogenic organizing pneumonia?
Bronchiolitis obliterans organizing pneumonia.
What are the clinical features of cryptogenic organizing pneumonia (2)?
-Cough
-Dyspnea
What are the histological features of cryptogenic organizing pneumonia (3)?
-Polypoid plugs of connective tissue in alveoli and bronchioli
-Connective tissue is all of the same age in contrast to UIP
-There are usually no honeycomb changes
Image of cryptogenic organizing pneumonia. Note the intra-alveolar Masson bodies (plugs of young fibroblasts) and mononuclear inflammatory cells and how the fibrosis appears to be the same age.
Image of bronchiolitis obliterans (bronchioli not shown on this field) with organizing pneumonia (BOOP). Note the intraalveolar fibromyxoid connective tissue proliferation, lymphoplasmacellular perivascular infiltrates, interstitial edema and bronchiolitis with a smattering of eosinophilic granulocytes.
The diagnosis of idiopathic organizing pneumonia can only be made after careful consideration of both clinical and radiologic findings. Which possibilities should be excluded first (9)?
-Infection
-Organizing DAD
-Bronchial obstruction
-Aspiration pneumonia
-Hypersensitivity pneumonia
-Drug reaction
-Toxic gas
-Bone marrow
-Lung or heart-lung transplantation
Image of cryptogenic organizing pneumonia (COP) aka bronchiolitis obliterans with organizing pneumonia (BOOP). Note the damaged bronchiolus with a plug of proliferating connective tissue.
What are Masson bodies?
Rounded balls of myxomatous (bluish) connective tissue that form intraluminal polypoid plugs within bronchioles and air spaces.
List the conditions that cause granulomatous inflammation of the lung (5).
-Tuberculosis
-Fungal infections
-Sarcoidosis
-Wegener granulomatosis
-Churg-Strauss allergic angiitis and granulomatosis
Describe the clinical features of sarcoidosis (4).
-Uncommon idiopathic systemic granulomatous disease
-Often affects the lung and causes respiratory insufficiency
-Lesions are non-caseating granulomas without identifiable organisms of any kind
-Sarcoidosis may "burn out" but leaves a scarred, fibrotic lung
Which parts of the respiratory system are affected by sarcoidosis (2)?
-Granulomas are commonly distributed along the bronchovascular bundle or adjacent to the pleura
-Hilar nodes are usually affected
Describe the epidemiology of sarcoidosis (3).
-Patients are often younger than 40 years of age
-Danes, Swedes and African Americans have a high incidence
-Higher prevalence in non-smokers
What is the etiology of sarcoidosis?
Disordered immune regulation in genetically predisposed individuals exposed to environmental agents. A cell-mediated response to an unidentified antigen is proposed.
Describe the pathogenesis of sarcoidosis (4).
-Oligo-clonal expansion of T-cell subsets
-Intra-alveolar and interstitial accumulation of CD4+ T-cells
-BAL: CD4/CD8 > 2.5; CD3/CD4 <0.31
-TNF is released from activated alveolar macrophages
What is the significance of TNF in sarcoidosis?
TNF-level in BAL is a marker of disease activity.
What is the significance of Bcl-2 in sarcoidosis?
Bcl-2 protein overexpression as determined by immunohistochemistry (IHC) is associated with poor response to chemotherapy and poor survival.
Image of CD4+ T cells in sarcoidosis. Note that the helper/suppressor ratio is reversed compared to hypersensitivity pneumonitis.
What are the systemic immunological abnormalities associated with sarcoidosis (2)?
-Anergy to common skin test antigens (Candida or PPD) due to pulmonary recruitment of CD4+ T-cells
-Polyclonal hypergammaglobulinemia
List the organs affected by sarcoidosis (8).
-Lungs
-Lymph nodes
-Spleen
-Liver
-Bone marrow
-Skin
-Eye: iritis or iridocyclitis
-Salivary glands
What is Mikulicz syndrome?
Sarcoidosis combined with uveoparotid Involvement.
Image of chest x-ray of sarcoidosis with bilateral hilar lymphadenopathy.
Image of sarcoidosis with enlarged axillary lymph nodes with confluent granulomas.
Image of chest x-ray of sarcoidosis with reticulo-nodular interstitial infiltrates.
Image of the pleural surface of lung tissue with sarcoidosis.
Image of epithelioid cell granulomas surrounding bronchovascular bundles in sarcoidosis.
Where are sarcoid granulomas are typically found?
In a peribronchovascular location or subpleurally.
Image of non-caseating granulomas in sarcoidosis.
Any fibrosing lung disease can end up like this "honeycomb" lung. What do the cystic areas represent?
Traction emphysema.
Image of Cor pulmonale, a clinical finding of a patient who had sarcoidosis with lung fibrosis.
What is Cor pulmonale?
Massive hypertrophy of the right ventricle.
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