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7 Cards in this Set

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Cryptogenic Organizing Pneumonia
Cryptogenic Organizing Pneumonia (COP):
1. An alveolar filling disease consisting of loose balls of young fibrous tissue (organization) in alveoli (organizing pneumonia) and bronchi-oles (bronchiolitis obliterans)
2. Many causes, usually idiopathic
3. Good prognosis, because there is no mature fibrosis
4. Formerly known as Bronchiolitis Obliterans with Organizing
Pneumonia (BOOP)
Nonspecific Interstitial Pneumonia
Nonspecific Interstitial Pneumonia (NSIP):
1. A chronic interstitial disease that tends to be temporally homogene-ous: cellular with lymphocytic infiltrates, or fibrosing
2. a disease of adults, both sexes
3. cellular NSIP has a better prognosis than fibrosing NSIP, which is bet-ter than UIP.
4. Is this a “wastebasket” diagnosis?; i.e., we don’t know how to classify it, so we’ll call it NSIP?
Pulmonary Infiltration with Eosinophilia (PIE) Syndrome:
1. A collection of entities characterized by eosinophilic infiltrates in blood, lung, or both
2. Many causes, including idiopathic but including microfilaria, drugs, association with asthma, and other infectious agents
3. Pulmonary infiltrates may come and go rapidly
4. Effective treatment is curative
Diffuse Pulmonary Hemorrhage Syndromes:
1. Goodpasture's Syndrome: alveolar hemorrhage of lungs with focal necrosis and proliferative glomerulonephritis due to basement mem-brane antibodies directed against lung and kidney. It typically involves young adult males.
2. Idiopathic pulmonary hemosiderosis is confined to the lung, with his-tologic evidence of repeated pulmonary hemorrhage. It is a disease of children and younger adults with no known cause and a variable clini-cal course.
3. Vasculitis associated hemorrhage is seen in such diseases as Wegener's granulomatosis and lupus erythematosus, occasionally polyarteritis.
Collagen Vascular Disease (an etiologic category):
Certain collagen vascular diseases are associated with diffuse interstitial lung disease that looks like UIP or NSIP: 1) Scleroderma; 2) Rheumatoid arthritis; 3) Polymyositis/dermatomyositis.
Note: Rheumatoid arthritis can also produce an acute or chronic pleuritis, rheumatoid nodules in the lungs, bronchiolitis, and Caplan's nodules when associated with silicosis or coal-workers pneumoconiosis.
Pulmonary Alveolar Proteinosis (PAP):
1. Characterized by accumulation of granular lipoprotein in alveolar spaces with very little alveolar response
2. Does not lead to chronic fibrosis
3. One third of patients get better, one third stay the same, and one third get worse
4. Cause unknown, probably not documented before 1945
5. Sometimes associated with immunosuppressed patients
Drug-induced lung disease (not a morphologic but an etiologic cate-gory):
Some drugs produce various forms of interstitial lung disease, particularly antineoplastic (radiomimetic) drugs.
1. The disease may look like DAD, UIP, NSIP, hypersensitivity pneu-monitis or other forms
2. The injury may be dose dependent or an idiosyncratic reaction
3. This complication is treatable if recognized in time