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Pulmonology > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

Total lung capacity =_____+_____.

A

FVC + RVC

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2
Q

Asbestosis:

  • cause
  • disease manifestation
A

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)
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3
Q

How does one acquire asbestosis?

A

-direct toxic effect of the fibers on pulmonary cells and release of mediators from inflamm cells

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4
Q

Clinical findings of asbestosis

A
  • 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**
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5
Q

Dx of Asbestosis consists of what tests?

A
  • 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
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6
Q

In what order does asbestosis progress in the lungs?

A

-begins in the lower lung zones with small parenchymal opacities with multinodular or reticular pattern

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7
Q

Bronchiolitis Obliterans

-how do you get this?

A
  • 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)

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8
Q

Presentation of Bronchiolitis Obliterans

A
  • 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
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9
Q

Dx of Brochiolitis Obliterans

A
  • requires transbronchial biopsies w/ Bronchiol Alveolar Lavage (BAL)
  • must rule out infection (viral and bacterial cultures must be negative)
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10
Q

Tx of Bronchiolotis Obliterans

A
  • changing anti-immune medications
  • photopheresis
  • retransplantation
  • Prevention: make sure pt takes immunosuppression drugs and they are staying healthy)
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11
Q

Hypersensitivity Pneumonitis

  • aka
  • what is this?
  • causes
A

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
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12
Q

Who gets Hypersensitivity pneumonitis?

WHat may actually be associated with decreased risk of HP?

A
  • Farmers lung: the farming population
  • bird fanciers

-smoking is associated with a decreased risk of HP

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13
Q

List some etiologic agents that may cause HP

A
  • 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
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14
Q

Acute HP

  • presentation
  • PE findings
  • Tx
  • what would you see on x-ray
A

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

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15
Q

Subacute or intermittent HP

  • presentation
  • lab findings
  • PFT findings
  • Xray finding
  • Tx
A

-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.

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16
Q

CHronic Progressive HP

  • sx
  • PE findings
  • Labs
  • PFT
  • XRAY
  • Tx
A

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.
17
Q

Dx of HP

A
  • 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)
18
Q

Prevention of HP

A
  • 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)
19
Q

What are the 5 acute side effects of steroids?

A
  • insomnia
  • stomach upset
  • psychosis
  • elevated BS
  • elevated BP
20
Q

What are the long term effects of steroids?

A
  • osteoporosis
  • psychosis
  • weight gain
  • cataracts
  • immunosuppression
  • adrenal axis suppression
21
Q

Interstitial Lung Disease

-definition

A

def: diffuse parenchymal lung disease

- a group of disorders that are classified together because of clinical xray, physiologic or pathologic manifestations.

22
Q

Interstitial Lung Disease

  • 50% are _____.
  • clinical presentation
  • prognosis
  • presentation on CXR
A

-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

23
Q

Idiopathic pulmonary fibrosis

  • what is this?
  • cause?
  • risk factors
A

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
24
Q

Idiopathic Pulmonary Fibrosis

-progression of disease

A

alveolitis is dominated by inflamm cells early on, these cells are:

  • alveolar mfs
  • neutrophils
  • eosinophils
  • lymphocytes
  • increased basophils and mast cells
25
Q

Idiopathic pulmonary fibrosis:

  • presentation
  • dx
A

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)
26
Q

Idiopathic Pulmonary Fibrosis

-txx

A

no tx is beneficial

may use;

  • glucocorticouds
  • immunosuppressives
  • -azathiprine
  • -cyclophosphamide
  • -methotrexate
  • antioxidants
  • -acetylcysteine
27
Q

Sarcoidosis

  • definition
  • who does this usually affect?
  • presents with what on CXR?
A 
- 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
28
Q

Sarcoidsosis Presentation

A
  • dyspnea
  • cough
  • chest pain
29
Q

Tx Sarcoidosis

what are some indications for tx

A
  • 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
30
Q

Drug Induced Pulmonary Dz: Eosinophilic Pneumonias

  • sx
  • drugs associated with this
  • Tx
A

Sx: dry cough, fever, chills, dyspnea

Drugs:

  • Nitrofurantoin
  • TCA’s
  • Sulfonamides
  • Penincillin
  • Thiazides
  • hydralazine

Tx: take them off the medication

31
Q

Radiation-induced Lung Injury

  • what are the two types
  • both types are seen in patients who have undergone thoracic radiation for??
A

Types:

  • radiation pneumonitis
  • radiation fibrosis

-breast, lung, esophogeal, lymphoma, stomach cancer

32
Q

Pathogenesis of Radiation-induced Lung injury

A
  • 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
33
Q

Many factors affect the development of radiation-induced lung disease, what are some?

A
  • method of radiation
  • volume of lug irradiated
  • dosage of radiation
  • induction chemotherapy
34
Q

Clinical manifestations of Radiation induced lung injury

A
  • 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
35
Q

PE findings of Radiation induced Lung injury

A
  • fine crackles or pleural rub
  • pleural friction rub*
  • dullness to percussion
  • tachypnea
  • cyanosis or signs of sever pulm HTN
36
Q

CXR findings of radiation induced lung injury

A
  • may be normal
  • patchy alveolar filling defects
  • straight line effect, not conforming to anatomical units but to confines of radiation port
  • small pleural effusion
37
Q

Tx radiation induced lung injury

A
  • corticosteroids
  • inhibition of collagen synthesis
  • stop radiation
38
Q

Pneumoconiosis

  • definition
  • examples
A

def: non-neoplastic rxn of the lung to inhaled mineral or organic dust

examples:
- silicosis
- coal workers
- can be complicated by infection

39
Q

Coal workers pneumoconiosis

  • sx
  • may cause what other dz
  • whats seen on radiograph?
A
  • 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

Pulmonology (15 decks)

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