Chapter 18- Interstital Lung Diseases Flashcards
What are 3 common symptoms of ILD?
dyspnea on exertion
dry cough
constitutional symptoms sometimes
What does PFT show with ILD?
restriction and gas-exchange abnormalities
Lymphangioleiomyomatosis commonly presents in what people?
women of childbearing age
Langerhans cell histiocytosis commonly presents in what people?
young male smokers
What is the most common Idiopathic interstitial pneumonia?
cryptogenic fibrosing alveolitis (IPF)
What is IPF (cryptogenic fibrosing alveolitis)?
progressive fibrosis of the lungs resulting in nonproductive cough and SOB that worsens with exertion and eventually causes hypoxemic respiratory failure
What age is assoc. w/ IPF?
50-70y/o
What will be found on PE, PFT, and X-ray with IPF?
crackles
PFT- decreased lung volumes, poor oxygenation, infiltrates on Xray
What is the treatment for IPF?
Corticosteroids + azathioprine
Case by case decision
For acute exacerbation of IPF, what is the treatment?
corticosteroids
Poor prognosis
What interstitial pneumonia is assoc. with connective tissue disorders and hypersensitivity pneumonitis?
Nonspecific interstitial pneumonia (NSIP)
What are S/S of NSIP?
slowly progressive dyspnea and bilateral interstitial infiltrates?
What is treatment for NSIP?
immunosuppressants
What population is desquamative interstitial pneumonia (DIP) assoc. with?
young individuals that smoke
What are the S/S of DIP?
progressive SOB and bilateral infiltrates on chest radiograph
How do you diagnose DIP?
biopsy (shows macrophages in alveolar spaces)
How do you treat DIP/
avoid tobacco
immunosuppressants
What is acute interstitial pneumonitis (AIP)?
dyspnea that progresses from days to weeks and leads to respiratory failure
usually fatal
What are s/s of Cryptogenic organizing pneumonia (COP)?
subacute or chronic dyspnea first noted on exertion and sometimes triggered after acute illness, like URI
Treatment of COP
immunosuppressants
Who is lymphocytic interstitial pneumonia (LIP) common in?
women
What are s/s of lymphocytic interstitial pneumonia (LIP)?
gradual onset dyspnea and cough
occasionally fever, CP, arthralgias, weight loss
How to you treat lymphocytic interstitial pneumonia (LIP)?
corticosteroids
What is the most common granulomatous disorder?
sarcoidosis
What are the associated risks of sarcoidosis?
Northern European
10-40 y/o
Women > men
Characterized by formation in tissues of noncaseating granulomas that organize in an inner core of epithelioid cells, macrophages, and giant cells, surround by a rim of lymphocytes, fibroblasts, and connective tissue
sarcoidosis
What organs are affected by sarcoidosis?
virtually any organ,
What are two problems assoc. with acute sarcoidosis?
Lofren syndrome
Uveoparotid fever
Lofren syndrome
erythema nodosum, arthritis, hilar adenopathy
Uveoparotid fever
uveitis, parotitis, facial nerve palsy
How is sarcoidosis treated?
corticosteroids
What are the usually s/s and PFTs for interstitial lung diseases related to connective tissue disorders?
extertional dyspnea and dry cough or asymptomatic
Bibasilar crackles
PFTs: restrictive pattern with decreased diffusion capacity
What is the treatment for interstitial lung diseases related to connective tissue disorders?
immunosuppressants
2 causes of drug induced lupus
procainamide or hydralazine
Drug that causes dyspnea, hemoptysis, pulmonary infiltrates
crack cocaine (“crack lung”)
Characterized by disruption of alveolar capillary membranes resulting in bleeding into alveolar spaces
Pulmonary vasculitis and diffuse alveolar hemorrhage
S/S: Pulmonary vasculitis and diffuse alveolar hemorrhage
abrupt onset of cough, fever, dyspnea, hemoptysis
What are 3 abnormalities associated with systemic vasculitis?
ocular, nasopharyngeal, cutaneous
What do you hear on auscultation with Pulmonary vasculitis and diffuse alveolar hemorrhage
inspiratory crackles, systolic murmur
What are 3 histologic patterns of Pulmonary vasculitis and diffuse alveolar hemorrhage
bland pulmonary hemorrhage
diffuse alveolar damage
pulmonary capillaritis
Define pneumoconiosis
accumulation of mineral dusts in the lungs usually leading to fibrosis
What professions can lead to silicosis?
mining, stone cutting, carving, polishing, sandblasting
What are s/s of silicosis?
dyspnea or asymptomatic
What can chronic silicosis result in besides fibrosis?
simple nodular silicosis
Formation of pigmented lesions surrounded by emphysema (coal macules)
coal worker’s pneumoconiosis
What s/s are associated with coal worker’s pneumoconiosis?
chronic productive cough
diffuse small rounded opacities on Xray
Where can you be exposed to abestos?
fibrous silicate used for insulation,, friction bearing surfaces or to strengthen materials
Characterized by pleural plaques, effusion, fibrosis, gradual onset of dyspnea
Asbestosis
Leads to acute chemical bronchitis and pneumonitis
berylliosis
How to treat berylliosis?
corticosteroids
What pneumoconiosis is associated with RA?
coal worker’s pneumoconiosis
What pneumoconiosis has elevated risk of TB?
silicosis
What pneumoconiosis has increased incidence of malignancy?
asbestosis
Fever, chills, cough, dyspnea, malaise that occurs several hours after inhaling organic antigens. treated with corticosteroids
acute hypersensitivity pneumonitis
What are the s/s of subacute or chronic hypersensitivity pneumonitis?
chronic dyspnea and cough
What can be heard and seen on CT for hypersensitivity pneumonitis
Heard: diffuse crackles and wheezes
CT: ground glass opacities, centrilobular nodules, mosaic attenuation patterns
“eosinophilic granuloma” seen in young to middle aged adults that are mainly white male smokers. Associated with dyspnea on exertion, cough, possible hemoptysis
Pulmonary Langerhans Cell Hisitocytosis
What does imaging and PFT show for Pulmonary Langerhans Cell Hisitocytosis?
PFT: obstructive pattern, impaired diffusion capacity
Imaging: micronodular lesions, cysts in mid and upper lung zones
Treatment for Pulmonary Langerhans Cell Hisitocytosis
stop smoking
corticosteroids
Abnormal proliferation of smooth muscle cells
Lymphangioleiommyomatosis
What are S/S of Lymphangioleiommyomatosis?
dyspnea, PNEUMOTHORAX, possible chylous pleural effusions and hemoptysis
How to treat Lymphangioleiommyomatosis?
manage pleural complications, bronchodilator, O2 therapy, avoid estrogen
Presence of pulmonary infiltrates and eosinophilia of peripheral blood or lung
eosinophilic lung disease
Acute eosinophilic lung disease
Fever, nonproductive cough, dyspnea <7 days leading to respiratory failure
Healthy Men 20-40 y/o
What do diagnostics show for Acute eosinophilic lung disease?
imaging: bilateral infiltrates
abundant eosinophils in lavage fluid
Treatment of Acute eosinophilic lung disease
corticosteroids
Productive cough, dyspnea, malaise, weight loss, NIGHT SWEATS, fever. Predominantly middle aged women. Treated with corticosteroids
Chronic eosinophilic lung disease
Simple pulmonary eosinophilia (Loffler syndrome)
Transient migratory infiltrates that last <1 month
Causes: Idiopathic, drugs, PARASITES
Treatment: remove cause, treat parasites, corticosteroids if idiopathic
When aspergillosis colonizes the airways in patients with asthma or CF
Allergic bronchopulmonary aspergillosis
Allergic bronchopulmonary aspergillosis S/S
fever, malaise, cough productive of THICK BROWN MUCUS PLUGS, some hemoptysis
Diagnostics for Allergic bronchopulmonary aspergillosis
Xray: pulmonary infiltrates, central bronchiectasis
Antibodies to aspergillus present
Treatment of Allergic bronchopulmonary aspergillosis
corticosteroids, itraconazole
Lipoproteinaceous material accumulates in alveoli. Caused by congenital or disorders with decreased macrophages. Can also be idiopathic.
Pulmonary Alveolar proteinosis
S/S of Pulmonary Alveolar proteinosis
progressive dyspnea on exertion, malaise, low-grade fever, cough
Pulmonary Alveolar proteinosis: findings of chest X-ray, CT, lavage
XRAY: bilateral perihilar opacities
CT: thickening of interlobular and interlobular septa
LAVAGE: milky, opaque fluid, looks foamy
Treatment of Pulmonary Alveolar proteinosis if hypoxia or severe dyspnea
whole lung lavage
What are the clinical manifestations of the pulmonary system in sarcoidosis?
Dyspnea, cough, wheezing, hemoptysis, endobronchial lesions
What are the clinical manifestations of the dermatological system in sarcoidosis?
Erythema nodosum, papules, plaques
What are the clinical manifestations of the otolaryngologic system in sarcoidosis?
Saddle nose deformity, sinusitis, laryngeal lesions, parotitis
What are the clinical manifestations of the ocular system in sarcoidosis?
Uveitis, choriorectinitis, lacrimal gland enlargement, chronic tearing, optic neuritis
What are the clinical manifestations of the Neurologic system in sarcoidosis?
Cranial nerve palsy, headache, diabetes insipidus, mass lesions, seizures, meningitis, encephalitis
What are the clinical manifestations of the rheumatologic system in sarcoidosis?
Arthralgias, arthropathy, myopathy
What are the clinical manifestations of the gastrointestinal system in sarcoidosis?
Elevated transaminases, abdominal pain, jaundice
What are the clinical manifestations of the cardiologic system in sarcoidosis?
Arrhythmias, conduction abnormalities, sudden cardiac death, pulmonary hypertension, congestive heart failure
What are the clinical manifestations of the hematologic system in sarcoidosis?
Lymphadenopathy (especially hilar), hypersplenism
What are the clinical manifestations of the Endocrine system in sarcoidosis?
Hypercalcemia, hypercalciuria, epididymitis
What are the clinical manifestations of the renal system in sarcoidosis?
Renal calculi, interstitial nephritis, renal failure
What are the syndromes associated with sarcoidosis?
Lofgren syndrome Heerfordt syndrome (uveoparotid fever)
What does lofgren syndrome consist of?
Fever, arthralgias, bilateral hilar adenopathy, erythema nodosum
What does Heerfordt syndrome consist of?
Fever, swelling of parotid gland and uveal tracts, cranial nerve VII palsy
What connective tissue disorders are involved in pleural effusion?
RA (5-40%)
Lupus (30-40%)
What connective tissue disorders are involved in necrobiotic nodules?
RA
What connective tissue disorders are involved in fibrosis?
RA (20--60%) Lupus (3%) Systemic Sclerosis (15-90%) Polymyotisis/dermatomyotisis (10-40%) Sjogren syndrome (33%)
What connective tissue disorders are involved in bronchiolitis?
RA
Lupus
Sjogren syndrom
What connective tissue disorders are involved in pulmonary arthropathy?
RA
Lupus
Systemic sclerosis
Polymyotisis/dermatomyotisis
What connective tissue disorders are involved in pneumonitis hemorrhage?
Lupus
What connective tissue disorders are involved in diaphragm dysfunction?
Lupus
What connective tissue disorders are involved in aspiration?
Systemic sclerosis
Polymyotisis/dermatomyotisis (14%)
What connective tissue disorders are involved in secondary carcinoma?
Systemic sclerosis
