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Clin. Med.
Chronic Diffuse Infiltrative Diseases
52
Medical
Graduate
09/13/2010

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Term
What are the most common chronic diffuse infiltrative lung diseases?
Definition
Postinflammatory pulmonary fibrosis
Idiopathic pulmonary fibrosis
Term
What are the steps of chronic diffuse infiltrative lung diseases?
Definition
-Initiation of tissue injury
-Maintenance of inflammation
-Resolution or development of fibrosis
Term
Name the common symptoms of chronic diffuse infiltrative lung diseases.
Definition
-dyspnea of gradual onset
-nonproductive cough
Term
What diagnostic imaging study would you use to dx chronic diffuse infiltrative lung diseases
Definition
CT
Term
What drugs can cause chronic diffuse infiltrative lung diseases?
Definition
-Bleomycin
-cyclophosphamide
-Methotrexate
-Nitrosoureas
Term
What noncytotoxic drugs can induce chronic diffuse infiltrative lung diseases?
Definition
-Nitrofurantoin
-Amiodorone
-Gold
-Penicillamine
-Illicit drugs
Term
Findings on CXR for chronic diffuse infiltrative lung diseases?
Definition
B/L symmetrical interstitial infiltrates
Term
Nitrofurantoin
Definition
-One of the most common causes of drug induced lung disease
-cough, dyspnea, and fever
-Pleural effusion
-Eosiophilia
-Pleurisy
Term
Amiodorone
Definition
-cough, dyspnea, fever
-pleural effusion
-Pulmonary toxicity 5-7% of cases
Term
Gold and Penicilamine
Definition
-Difficult to differentiate between drug or underlying cause
Term
Illicit Drugs
Definition
-Talc-induced granulatosis
-pulmonary fibrosis
-Dx by biopsy
Term
What are the types of pneumoconioses?
Definition
-Asbestosis
-Silicosis
-Coal worker's pneumoconiosis
-Berylliosis
Term
What are the main features of asbestosis?
Definition
-pleural plaques
-Incr. malignancy
-Diffuse interstitial fibrosis
Term
What are the sxs of asbestosis?
Definition
-Sxs
-Dyspnea onset after 15-20 yrs.
-non-productive cough
-rales
-clubbing
-cor pulmonale
Term
Name the occupations that are at risk of developing silicosis.
Definition
-mining
-cutting, polishing, and carving of stone
-foundry work
-sandblasting
Term
With what disease does TB occur more frequently?
Definition
Silicosis
Term
What are the two forms of silicosis?
Definition
-Simple nodular silicosis
-progressive massive fibrosis (PMF)
Term
Name the findings that are seen on a CXR for silicosis.
Definition
-opacities in the upper lobes
-hilar node enlargement w/ egg-shell calcifications
Term
What pneumocomiosis disease is similar to sarcoidosis?
Definition
Berylliosis
Term
Name the differentiating sxs of berylliosis.
Definition
-chest pain
-fatigue
-weight loss
-arthralgia
Term
How would a person's labs turn out if they had berylliosis?
Definition
-hyperuricemia
-hypercalcemia
-hypercalcinuria
Term
What kind of diffuse lung disease is Farmer's lung?
Definition
Hypersensitivity pneumonitis
Term
What are some of the features of Farmer's lung?
Definition
-2/3 have granulomas
-development of fibrosis if chronic
-50% have bronchiolitis obliterans
Term
List the sxs of acute hypersensitivity pneumonitis.
Definition
fever, chills, cough, dyspnea, and malaise 4-8 hrs after exposure.
Term
How would you dx hypersensitivity pneumonitis?
Definition
Bronchoscopy with BAL and transbronchial biopsy.
Term
Malignant diffuse infiltrative lung disease occurs with what diseases?
Definition
-lymphangitic carcinomatosis
-alveolar cell carcinoma
-lymphoma
-leukemia
Term
What are the findings of CXR in malignant diffuse infiltrative lung disease?
Definition
B/L reticular/reticulonodular infiltrates, Kerley B lines, pleural effusion, and hilar lymphadenopathy.
Term
Name the 11 chronic diffuse infiltrative lung diseases of unknown etiology.
Definition
Sarcoidosis, idiopathic pulmonary fibrosis, desquamative interstitial pneumonia, acute interstitial pneumonia, lymphocytic interstitial pneumonia, bronchiolitis obliterans organizing pneumonia (BOOP), collagen vascular diseases, eosinophilic granuloma of the lung, alveolar, poteinosis, eosinophilic pneumonia, and diffuse alveolar hemorrhage.
Term
Sarcoidosis
Definition
-Multisystem
-Noncaseating granulomas
-blacks 10x more than whites
-5% have normal CXR
Term
List the sxs of sarcoidosis.
Definition
-dyspnea
-cough
-fever
-B/L hilar lymphadenopathy
-arthralgia
-erythema nodosum
Term
What is the more common form of sarcoidosis? Rare?
Definition
-Intrathoracic
-Extrathoracic
Term
What are some of the body systems affected by extrathoracic sarcoidosis?
Definition
-skin (erythema nodosum and lupus pernio)
-eyes (uveitis)
-nervous system
-Cardiac
-endocrine
Term
Lymphocytic interstitial pneumonia
Definition
-occurs with autoimmune diseases
-women
-rales and lymphadenopathy
-BAL = b cells
Term
What does BOOP stand for?
Definition
bronchiolitis obliterans organizing pneumonia
Term
What is BOOP?
Definition
-secondary disease
-fever, malaise, fatigue, cough and dyspnea
-rales
-diagnose with CT/biopsy
-diff. dx=farmer's lung (no granulomas)
Term
Name the collagen vascular diseases with associated pulmonary involvement.
Definition
-SLE
-RA
-Polymyositis
-Dermatomyositis
-Sjogren syndrome
Term
What are some of the results of a PFT in collagen vascular diseases?
Definition
-small airway obstruction
-decr. dlco
-hypoxemia at rest
Term
Eosinophilic granuloma of the lung.
Definition
-Strong association with smoking
-Granulomas of the bone
-Pos. CT for cysts
-tx: smoking cessation
Term
Idiopathic pulmonary fibrosis
Definition
-rales
-cyanosis
-cor pulmonale
-clubbing
-Dx: Exclusion
-lung transplant
-incr. risk of lung carcinoma
Term
Desquamative interstitial pnuemonia
Definition
-clubbing
-men
-smokers
-CXR: vague bibasilar opacities
Term
Acute interstitial pnuemonia
Definition
-progression in 30 days: dyspnea, cough with mucoid sputum to respiratory failure
-mortality 33-78% in 6 months
Term
what are the diseases that are assoc. with diffuse alveolar hemorrhage?
Definition
-Goodpasture Syndrome
-glomerulonephritis
-vasculitits-Wegener's
-antiphospholipid Ab syn.
-Anticoagulants
-idiopathic pulmonary hemosiderosis (hemoptysis)
Term
What tests would you perform to dx diffuse alveolar hemorrhage?
Definition
-Labs: anti-GBM Ab, antinuclear and anti-DNA, ANCA, complement levels, and immune complexes
-BAL: w/ iron stain, finding of hemosiderin-laden macrophages
Term
Anti-GBM Ab disease
Definition
-young smokers
-hemoptysis
-Fe anemia
-Renal failure
-fluffy perihilar acinar shadows
-PFT: restrictive, incr. Dclo
-Dx by presence of anti-GBM Ab
Term
Alveolar proteinosis
Definition
-Intra-alveolar accumulation of lipoproteinaceous material
-90% acquired caused by autoantibodies to GM-CSF
-3 presentations: Asymptomatic w/ abn. CXR, abrupt onset of dyspnea, cough, fever and chest pain, and gradual onset of dyspnea and cough.
-Dx: BAL findings of PAS positive material
Term
What are the 7 forms of eosinophilic pneumonia?
Definition
-Simple pulmonary eosinophilia (loffler syn.)
-Prolonged pulmonay eosinophilia w/o asthma
-Pulmonary eosinophilia with asthma
-Tropical pulmonary eosinophilia
-Pulmonary vasulitis
-Hypereosinophilic syn.
-acute eosinophilic pneumonia
Term
simple pulmonary eosinophilia
Definition
-Transient, migratory infiltrates
-accumulation of eosinophils, macrophages and fluid
-tx: stop drugs causing, treat parasitic infection
Term
Prolonged pulmonary eosinophilia w/o asthma
Definition
-middle-aged women
-productive cough, dyspnea, malaise, weight loss, night sweats, and fever
-Dx: CXR, BAL, peripheral eosinophilia and bronchoscopy
Term
Tropical pulmonary eosinophilia
Definition
-asthma, fever, marked blood eosinophilia, and CXR with reticulonodular infiltrates
-recently traveled to Far East
Term
Pulmonary vasculitis
Definition
-granulomatous inflammation of the lungs
-eosinophilia with elevated IgE levels
Term
Acute eosinophilic pneumonia
Definition
-cough, dyspnea, fever, tachypnea, and rales
Term
Lymphangioeiomyomatosis
Definition
-young women
-CT: cysts throughout lung
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