Restrictive Lung Disease Flashcards
Total lung capacity =_____+_____.
FVC + RVC
Asbestosis:
- cause
- disease manifestation
Cause:
-inhalation of asbestos fibers; chrysotile(less toxic, 90% of cases) & amphibole
Manifestation:
- slowly progressive (20-30years), diffuse FIBROSIS
- spectrum : asbestosis, pleural dz, malignancies (non-small cell and small cell carcinoma of lungs, malignant mesothelioma)
How does one acquire asbestosis?
-direct toxic effect of the fibers on pulmonary cells and release of mediators from inflamm cells
Clinical findings of asbestosis
- asymptomatic for 20-30yrs after exposure
- dyspnea on exertion
- progress to bibasilar end expiratory crackles and clubbing
- if cough, sputum production, or wheezing are present more likely secondary to smoking**
Dx of Asbestosis consists of what tests?
- PFTs
- -reduced lung volumes (VC and TLC)
- -decreased pulmonary compliance
- -absence of airflow obstruction
- Radiographs
- -pleural abnormalities
- -shaggy heart and ground glass appearance
- -honeycombing and upper lobe involvement late stage disease
In what order does asbestosis progress in the lungs?
-begins in the lower lung zones with small parenchymal opacities with multinodular or reticular pattern
Bronchiolitis Obliterans
-how do you get this?
- chronic airway rejection in lung transplant patients d/t:
- -episodes of acute rejection
- -primary graft dysfunction
- -CMV pneumonitis
- -Noncompliance w/ immunosuppressive meds
- -lymphocyte bronchitis or bronchiolitis
-can develop farther out from transplant (5yrs after lung transplant)
Presentation of Bronchiolitis Obliterans
- indolent sx, similar to URI (cough, tired, fatigue, exertional dyspnea)
- decline in spirometry
- advanced stages we see bronchiectasis w/ obstruction and hyperinflation, often colonized w/ pseudomonas d/t their immunosuppression
Dx of Brochiolitis Obliterans
- requires transbronchial biopsies w/ Bronchiol Alveolar Lavage (BAL)
- must rule out infection (viral and bacterial cultures must be negative)
Tx of Bronchiolotis Obliterans
- changing anti-immune medications
- photopheresis
- retransplantation
- Prevention: make sure pt takes immunosuppression drugs and they are staying healthy)
Hypersensitivity Pneumonitis
- aka
- what is this?
- causes
aka: extrinsic allergic alveolitis
- this is an immunologic rxn to an inhaled agent occuring within the pulmonary parenchyma, a restrictive lung dz
Causes:
- agricultural dusts
- bioaerosols
- reactive chemical species
Who gets Hypersensitivity pneumonitis?
WHat may actually be associated with decreased risk of HP?
- Farmers lung: the farming population
- bird fanciers
-smoking is associated with a decreased risk of HP
List some etiologic agents that may cause HP
- farming, vegtable and dairy cattle workers
- ventilation and water-related contamination
- bird and poultry handling
- veterinary worka nd animal handling
- Grain and flour processing and loadign
- lumber milling, construction, wood stripping
- painting
Acute HP
- presentation
- PE findings
- Tx
- what would you see on x-ray
Presentation :
- abrupt onset 4-6hrs of:
- fever and chills
- nausea
- chest tightness & without wheezing***
PE:
-tachypnea and diffuse fine rales(crackles)
Tx:
-removal from Ag, sx subside in 12hrs to sever days, disease may recur with re-exposure
See on CXR/HRCT:
-may show micronodular, interstitial pattern
Subacute or intermittent HP
- presentation
- lab findings
- PFT findings
- Xray finding
- Tx
-gradual development of cough, dyspnea, fatigue, anorexia, and weight loss, tachypnea, diffuse rales
Lab findings:
-lymphocytosis on bronchioal alveolar lavage, mild hypoxemia
PFT:
-restriction patter or mixed restriction/obstruction pattern
Xray:
-normal or reticular opacities in middle and upper lung zones
Tx: removal from ag and glucocorticosteroids, take weeks to months to resolve.
CHronic Progressive HP
- sx
- PE findings
- Labs
- PFT
- XRAY
- Tx
sx: insidious onset of cough, dyspnea, fatigue and weight loss
PE: digital clubbing
Labs:
-lymphocytosis, neutrophilia or eosinophilia on BAL.
PFT: restrictive, obstructive often seen with it, resting and exertional hypoxemia
XRay:
-fibrotic changes, loss of lung volume, emphysema pattern changes
Tx:
- antigen avoidance
- glucocorticoids used to accelerate initial recovery.
Dx of HP
- high index of suspicion from careful review of occupational, avocational, and domestic exposures
- normal CXR doesnt rule it out, need to have HRCT
- inhalation challenge by re-exposure
- HRCT and BAL (if chronic intermittent or chronic progressive they may need these tests)
Prevention of HP
- reduction of antigenic burden (wetting compost)
- design facilities (maintain humidty, avoid having stagnant water or carpet)
- Maintenance: routinely inspect all heating, ventilation, and air conditioning equipment.
- protective devices (masks and filters)
What are the 5 acute side effects of steroids?
- insomnia
- stomach upset
- psychosis
- elevated BS
- elevated BP
What are the long term effects of steroids?
- osteoporosis
- psychosis
- weight gain
- cataracts
- immunosuppression
- adrenal axis suppression
Interstitial Lung Disease
-definition
def: diffuse parenchymal lung disease
- a group of disorders that are classified together because of clinical xray, physiologic or pathologic manifestations.
Interstitial Lung Disease
- 50% are _____.
- clinical presentation
- prognosis
- presentation on CXR
-50% are idiopathic (Idiopathic pulmonary Fibrosis)
- Clinical presentation:
- progressive exertional dyspnea and nonproductive cough
Prognosis: usually die of resp failure within 3-6 years once x-ray changes
Presentation on CXR:
-haziness progresses to nodules then linear opacities, shaggy heart border, blunted costophrenic angle
Idiopathic pulmonary fibrosis
- what is this?
- cause?
- risk factors
What is this?
-chronic relentless progressive fibrotic disorder of the lover resp. tract
-cause: idiopathic, we dont know what causes this.
Risk factors:
- smoking
- infections
- environmental pollutants
- chronic aspiration and drugs
Idiopathic Pulmonary Fibrosis
-progression of disease
alveolitis is dominated by inflamm cells early on, these cells are:
- alveolar mfs
- neutrophils
- eosinophils
- lymphocytes
- increased basophils and mast cells
Idiopathic pulmonary fibrosis:
- presentation
- dx
Presentation:
- dyspnea on exertion
- persistent nonproductive coughs
- abnormal
Dx:
- routine blood tests inclluding serologic studies and autoimmune testing to r/o other disease
- radiographs, HRCT
- PFT (restrictive pattern)
- bronchoalveolar lavage (looking for neutrophilia)
Idiopathic Pulmonary Fibrosis
-txx
no tx is beneficial
may use;
- glucocorticouds
- immunosuppressives
- -azathiprine
- -cyclophosphamide
- -methotrexate
- antioxidants
- -acetylcysteine
Sarcoidosis
- definition
- who does this usually affect?
- presents with what on CXR?
- multisystem granulomatous disorder of unknown etiology (inflamm response that results in a collect of immune cells ) noncaseating granulomas (caseating: a form of necrosis in which tissue is changed into a dry mass of dead cells resembling cheese)
-usually effects young adults
- Presents w/ one or more of the following:
- bilateral hilar lymphadenopathy
- pulmonary reticular opacities
- skin, joint, and/or eye lesions
Sarcoidsosis Presentation
- dyspnea
- cough
- chest pain
Tx Sarcoidosis
what are some indications for tx
- some undergo spontaneous remission or have a benign clinical course
- cause of dz is unknown so no specific tx exists
- glucocorticoids may help
Indications for Tx:
- worsening of pulm sx: cough, dyspnea, chest pain, hemoptysis
- deteriorating lung function
- progressive radiograph changes
Drug Induced Pulmonary Dz: Eosinophilic Pneumonias
- sx
- drugs associated with this
- Tx
Sx: dry cough, fever, chills, dyspnea
Drugs:
- Nitrofurantoin
- TCA’s
- Sulfonamides
- Penincillin
- Thiazides
- hydralazine
Tx: take them off the medication
Radiation-induced Lung Injury
- what are the two types
- both types are seen in patients who have undergone thoracic radiation for??
Types:
- radiation pneumonitis
- radiation fibrosis
-breast, lung, esophogeal, lymphoma, stomach cancer
Pathogenesis of Radiation-induced Lung injury
- ionizing radiation localized release of sufficient energy to break strong chemical bonds and generate highly reactive free radical species*
- development of fibrosis triggered by radiation-induced cellular signal transduction
- the cytotoxic effect is d/t DNA damage that causes clonagenic death in normal lung epithelial cells
Many factors affect the development of radiation-induced lung disease, what are some?
- method of radiation
- volume of lug irradiated
- dosage of radiation
- induction chemotherapy
Clinical manifestations of Radiation induced lung injury
- early nonproductive cough
- dyspnea on exertion or inability to take a deep breath
- low grade fever
- chest pain; pleuritic or substernal
- malaise and weight loss
PE findings of Radiation induced Lung injury
- fine crackles or pleural rub
- pleural friction rub*
- dullness to percussion
- tachypnea
- cyanosis or signs of sever pulm HTN
CXR findings of radiation induced lung injury
- may be normal
- patchy alveolar filling defects
- straight line effect, not conforming to anatomical units but to confines of radiation port
- small pleural effusion
Tx radiation induced lung injury
- corticosteroids
- inhibition of collagen synthesis
- stop radiation
Pneumoconiosis
- definition
- examples
def: non-neoplastic rxn of the lung to inhaled mineral or organic dust
examples:
- silicosis
- coal workers
- can be complicated by infection
Coal workers pneumoconiosis
- sx
- may cause what other dz
- whats seen on radiograph?
- asymptomatic
- may cause chronic bronchitis and COPD, then k nown as industrial bronchitis and is compensable
Radiograph:
- small opacities can progress to larger opacities and fibrosis
- alveoli coalesce and the alveoli die within the fibrotic lesions
