Diffuse Parenchymal Lung Disease (DPLD) Flashcards Preview

10 PULMONARY AND CRITICAL CARE MEDICINE > Diffuse Parenchymal Lung Disease (DPLD) > Flashcards

Flashcards in Diffuse Parenchymal Lung Disease (DPLD) Deck (26):
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Rare DPLD with Well-Defined Features:

Lymphangioleiomyomatosis

Langerhans cell histiocytosis

Anti-GBM disease (Goodpasture syndrome)

Chronic eosinophilic pneumonia

Pulmonary alveolar proteinosis

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Lymphangioleiomyomatosis

A rare cystic lung disease that occurs sporadically in women of childbearing age or in association with tuberous sclerosis.  Affects women in their 30s and 40s. Associated with emphysema, spontaneous pneumothorax, or chylothorax in a young woman with dyspnea and a chest radiograph that shows hyperinflation and/or cystic disease  should prompt consideration

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Langerhans cell histiocytosis

Affects younger men who smoke. Improves with smoking cessation.

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Anti-GBM disease (Goodpasture syndrome)

Associated with anti-glomerular basement membrane antibody. Hemoptysis and glomerular disease are hallmarks.

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Chronic eosinophilic pneumonia

Chest radiograph shows “radiographic negative” of heart failure, with peripheral alveolar infiltrates predominating. Other findings may include peripheral blood eosinophilia and eosinophilia on bronchoalveolar lavage.

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Pulmonary alveolar proteinosis

Slowly progressive disorder affecting patients in their 20s to 50s (predominantly men). Diagnosed via bronchoalveolar lavage, which shows abundant protein in the airspaces. Chest CT shows “crazy paving” pattern.

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Unknown Causes of DPLD:

Idiopathic interstitial pneumonias:

Idiopathic pulmonary fibrosis (IPF)

Acute interstitial pneumonia

Cryptogenic organizing pneumonia (COP)

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Idiopathic pulmonary fibrosis (IPF)

Chronic, insidious (>6 mo) (typically follows a prolonged course) onset of a dry, hacking cough and dyspnea, usually in a patient aged >50 y. Digital clubbing is present in 30% of patients. Risk factors include: history of smoking, organic dust exposure, and age (the prevalence of IPF increases with age).  Diagnosis of exclusion.

Dx: Computed tomographic (CT) scan shows the classic findings of IPF, including basal and peripheral disease with septal thickening, evidence of honeycomb changes, traction bronchiectasis, and no evidence of ground-glass opacities or nodules. Although the radiographic findings of IPF are varied, it has a dominant interstitial (reticular) pattern, with or without opacities.

Usual interstitial pneumonia pathology (honeycombing, bibasilar infiltrates with fibrosis).

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Acute interstitial pneumonia

Dense bilateral acute (<6 wks) lung injury similar to acute respiratory distress syndrome; 50% mortality rate.

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Cryptogenic organizing pneumonia (COP)

Patients with COP often present with signs and symptoms consistent with community-acquired pneumonia and may be treated at least once with antibiotics for this presumed diagnosis.

Hx: Most patients have symptoms for less than 3 months, and very few have symptoms for more than 6 months.

Dx: Chest radiograph shows bilateral diffuse alveolar opacities. In the presence of normal lung volume. One of the key radiographic features of COP is the tendency for opacities to “migrate,” or involve different areas of the lung on serial examinations. 

Bronchiolitis obliterans organizing pneumonia (BOOP): COP is the idiopathic form of BOOP. 

Many underlying conditions, including certain infectious diseases, collagen vascular diseases, and drug-induced reactions, are associated with the histopathologic features of BOOP and respond best to specific treatment of the primary disease process. Prognosis is typically favorable, with a good response to systemic glucocorticoids.

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DPLD Causes:

Drug-induced

Smoking-related

Radiation

Chronic aspiration

Pneumoconioses

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Drug-induced

Examples: amiodarone, methotrexate, nitrofurantoin, chemotherapeutic agents (bleomycin)(see www.pneumotox.com for a complete listing)

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Smoking-related

“Smoker's” respiratory bronchiolitis characterized by gradual onset of persistent cough and dyspnea. Radiograph shows ground-glass opacities and thickened interstitium. Smoking cessation improves prognosis. Desquamative interstitial pneumonitis and Langerhans cell histiocytosis are other histopathological patterns associated with smoking and DPLD.

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Radiation

May occur 6 weeks to months following radiation therapy

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Pneumoconioses

Asbestosis (shipyards, construction) - barbell bodies

Silicosis (rock quary, sand blasting),

Berylliosis (aeronautics, electronics)

HP - Bird Fancier's lung (antigen mediated)

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Chronic aspiration

Aspiration is often subclinical

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Connective tissue diseases causing DPLD:

Rheumatoid arthritis

Progressive systemic sclerosis

Polymyositis/dermatomyositis

Asbestosis

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Rheumatoid arthritis

10%-20% of patients with rheumatoid arthritis (mostly men) are affected. May affect the pleura (pleuritis and pleural effusion), parenchyma, airways (bronchitis, bronchiectasis), and vasculature. The parenchymal disease can range from BOOP-type pattern to usual interstitial pneumonitis.

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Progressive systemic sclerosis

Nonspecific interstitial pneumonia pathology; may be progressive in 50% of patients. May be exacerbated by aspiration due to esophageal involvement; antibody to Scl-70 or pulmonary hypertension portends a poor prognosis. Monitoring of diffusing capacity for early involvement is warranted.

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Polymyositis/dermatomyositis

Many different types of histology; poor prognosis

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Asbestosis

Pleural plaques are the most common radiographic finding in patients exposed to asbestos. Ninety percent of asbestos-induced pleural abnormalities are caused by pleural plaques (well-circumscribed lesions) and diffuse pleural thickening.

Dx: The diagnosis of asbestosis is based on two essential findings: a convincing history of asbestos exposure with an appropriately long latent period and definite evidence of interstitial fibrosis. 

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Other connective tissue diseases:

Sjögren syndrome, Behçet disease

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DPLD Causing Granulomatous Changes:

Known Causes

Hypersensitivity pneumonitis

Unknown Causes:

Sarcoidosis

Granulomatosis with polyangiitis (Wegener)

 

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Hypersensitivity pneumonitis

An allergic, inflammatory lung disease that is also called extrinsic allergic alveolitis.   It results from exposure to airborne allergens that cause cell-mediated immunologic sensitization.

Immune reaction to an inhaled low-molecular-weight antigen; may be acute, subacute, or chronic.

Hx: Patients typically present with dyspnea, cough, fatigue, anorexia, malaise, and weight loss. Most patients who are exposed to an inhalational antigen have symptoms within 4 to 12 hours.

Dx: Pulmonary function testing may show obstructive and restrictive defects. Noncaseating granulomas are seen.

Chronic hypersensitivity pneumonitis has a poor prognosis.

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Sarcoidosis

Variable clinical presentation, ranging from asymptomatic to multiorgan involvement. ranging from acute disease with erythema nodosum, fever, arthralgia, and hilar lymphadenopathy (Löfgren syndrome), to a more indolent course. Ninety percent of patients have pulmonary involvement. 

Stage 1: hilar adenopathy. Stage 2: hilar adenopathy plus interstitial lung disease. Stage 3: interstitial lung disease. Stage 4: fibrosis. Noncaseating granulomas are hallmarks.

Sarcoidosis is a diagnosis of exclusion based on multisystem involvement and histologic evidence of noncaseating granulomas when all other causes have been excluded. Most patients require tissue diagnosis, but some cases do not warrant histologic confirmation. These include classic clinical presentations of known sarcoid syndromes, such as Löfgren syndrome and Heerfordt syndrome (uveitis, parotid gland enlargement, and fever). 

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Granulomatosis with polyangiitis (Wegener)

May be associated with upper airway involvement and other systemic findings.