Restrictive Lung Disease Flashcards
Restriction” in lung disorders always means what?
What else may be reduced?
What is increased or normal?
a decrease in lung volume (FVC)
TLC and FEV1
FEV1/FVC
What is the cause of Asbestosis?
inhalation of asbestos fibers
What are the two categories of fibers?
Which is less toxic and more common?
chrysotile and amphibole
Chrysotile less toxic
and accounts for 90% of asbestos use in U.S.
Asbestosis disease manifestations?
What can it lead to? 2
Characterized by slowly progressive (years), diffuse pulmonary fibrosis
- Malignancies: non-small cell and small cell carcinoma of the lungs
- malignant mesothelioma (cancer of the pleural lining of the lungs)
Asbestosis pathogenesis?
2
- direct toxic effect of the fibers on pulmonary cells and
- release of mediators from inflammatory cells
(inflammatory to fibrous scaring)
Clinical findings for Asbestosis?
3
If cough, sputum production, or wheezing are present more likely secondary to what?
- most patients asymptomatic for 20-30 yrs after initial exposure:
- Dyspnea on exertion
- Progresses to fine bibasilar end expiratory crackles and clubbing
smoking
How do we diagnose asbestosis and what would these tests show?
2 (3 and 4)
PFTs:
- Reduced lung volumes—VC and TLC
- Decreased pulmonary compliance
- Absence of airflow obstruction (normal ratio of FEV1 to FVC)
Radiographs
- Begins in lower lung zones w/ small parenchymal opacities w/ a multinodular or reticular pattern
- Often associated pleural abnormalities
- “Shaggy” heart and “ground” glass appearance
- Honeycombing and upper lobe involvement late stage disease
Bronchiolitis Obliterans (BO) pathogensis?
Chronic airway rejection in lung transplant patients
BO: Chronic airway rejection in lung transplant patients is due to what? 5
- Episodes of acute rejection
- Primary graft dysfunction
- CMV pneumonitis
- Noncompliance w/ immunosuppressive meds
- Lymphocyte bronchitis or bronchiolitis
What can BO develop from?
lung transplant (5 years out 45% of recipients develop BO)-slowly progressing
BO presentation
4
- Usually indolent symptoms similar to URI
- Exertional dyspnea and decline in spirometry
- Initially radiographs and exam only help exclude other illnesses
- Advanced stages see bronchioectasis w/ obstruction and hyperinflation, often colonized w/ pseudomonas
Diagnosis of BO requires what?
Requires transbronchial biopsies with BAL
Need a good bronchoscopy technique and adequate bronchio-alveloar lavage
Transbronchial biopsies with BAL are usually made on a pt who presents with what?
What do we have to rule out?
Usually made on a patient who presents w/ declining spirometry without an acute illness
infection
Treatment of BO?
4
- Changing anti-immune medications
- Photopheresis
- Retransplantation
- Prevention!
What is hypersensitivity pneumonitis also know as?
extrinsic allergic alveolitis
Hypersensitivity pneumonitis represents what kind of response to an inhaled agent?
What kind of agent usually?
Where does it occur?
immunological reaction
Usually an organic antigen
Occurring within the pulmonary parenchyma
Inciting agents include for HP include?
3
Agricultural dusts
Bioaerosols
Reactive chemical species
What is associated with a decreased risk of HP?
Increased risk?
smoking
genetic factors
HP Etiologic Agents
Farming, vegetable and dairy cattle workers
Ventilation and water-related contamination
Bird and poultry handling (exposure to down)
Veterinary work and animal handling
Grain and flour processing and loading (grain can become colonized w/ microorganisms and insects, grain is easily aerosolized so exposure to antigens can occur easily)
Lumbar milling, construction, wood stripping etc.: mold exposure
Plastic manufacturing
Painting
Electronics industry
Presentation types of HP?
3
Acute
Subacute or intermittent
Chronic progressive
Acute presentation of HP? 2
Onset?
Symtpoms? 4
PE findings? 2
Treatment? 1
- May follow heavy exposure to antigen
- May be confused w/ viral or bacterial infection
Abrupt onset (4-6 hrs after exposure) of:
- fever and chills
- Nausea
- chest tightness and dyspnea
- without wheezing
PE: tachypnea and diffuse fine rales
TX: Removal from antigen—symptoms subside in 12 hrs to several days/disease may recur w/ re-exposure
What would an XRAY look like for HP?
Labs to order?
may show a micronodular, interstitial pattern, frequently normal; sometimes do HRCT
CBC to rule out infection maybe
How is it acquired?
Describe the onset of subacute or intermittent HP?
4
Low level exposure over time
Gradual development of
- productive cough,
- dyspnea, fatigue,
- anorexia and
- weight loss
Subacute HP PE findings? 2
Lab findings? 2
PFT findings? 2
Xray findings? 2
Treatment?2
PE: tachypnia, diffuse rales
Lab: lymphocytosis on bronchial alveolar lavage, mild hypoxemia
PFTS: restriction pattern or mixed restriction/obstruction pattern
X-rays: normal or reticular opacities in middle and upper lung zones
TX: removal from antigen and glucocorticosteroids, takes weeks to months to resolve
Chroic progressive HP generally has no report of what?
Insideous onset of what things? 4
PE findings? 1
What will it be hard to differentiate from? 1
Lab findings? 3
PFT findings? 3
Xray findings? 3
Generally no report of acute episodes
Insidious onset of
- cough,
- dyspnea,
- fatigue
- wt. loss
PE:
1. digital clubbing may be seen
Differential from idiopathic pulmonary fibrosis is difficult
Lab: lymphocytosis, also neutrophilia or eosinophilia on BAL
PFTs: restrictive, obstructive often seen with it, resting and exertional hypoxemia
X-rays:
- fibrotic changes,
- loss of lung volume,
- emphysema pattern changes
Differential Diagnosis for HP?
5
- Inhalation fever
- Organic dust toxic syndrome
- Chronic bronchitis
- Asthma
- Chronic airflow limitation
What would give us a high index of expoure for an HP diagnosis?
What tests would we use to diagnose HP?
3
Careful review of patient’s occupational, avocational and domestic exposures
- A normal CXR doesn’t rule it out
- Inhalation challenge by re-exposure
- HRCT and BAL
HP treatment?
2
Antigen avoidance
Glucocorticoids used to accelerate initial recovery, however, the long-term outcome is relatively unchanged
How would we prevent HP?
4
- Reduction of antigenic burden (wetting compost)
- Design facilities: maintain humidity less than 60%, avoid having stagnant water or carpet that is likely to get moist
Maintenance: routinely inspect all heating, ventilation, an air conditioning equipment that it is clean and water is drained daily from humidifiers and vaporizers
Protective devices: masks, filters
Definition of interstitial lung disease?
2
- Diffuse parenchymal lung diseases
2. Most of these disorders are associated w/ extensive alteration of alveolar and airway architecture
Short term side effects of steroids
4
- insomia
- stomach upset
- high blood pressure and blood sugars
- psychosis
take in the morning and noon
Long term side effects of steroids
7
- osteoporosis
- immunosuppression
- adrenal axis suppression (give them more for trauma)
- ulcers
- weight gain
- cataracts
- diabetes
What is Idiopathic Pulmonary Fibrosis?
Affects adults of which age?
Cause?
- Chronic, relentlessly progressive fibrotic disorder of the lower respiratory tract
- Affects adults > 40YO
- Precise factors that initiate and maintain inflammatory and fibrotic responses in IPF are unknown
Risk factors for IPF?
4
Smoking
Infections
environmental pollutants
chronic aspiration and drugs
Early in IPF alveolitis is dominated by inflammatory cells including:
5
- Alveolar macrophages—secrete proinflammatory and profibrotic cytokines which affect mesenchymal cell proliferation and promote collagen depositions
- Neutrophils
- Eosinophils
- Lymphocytes
- Increased numbers of basophils and mast cells are also found
IPF presentation
3
- Dyspnea on exertion
- Persistent nonproductive cough
- Abnormal CXR
Diagnosis of IPF?
4
- Routine blood tests including serologic studies and autoimmune testing to r/o other diseases
- Radiographs, HRCT
- PFTs—restrictive pattern
- Bronchoalveolar lavage (looking for neutrophilia)
Treatment of IPF
2
Meds?
3
- Prognosis of the disease dismal
- Trials have no proof that prognosis of the disease is dismal
- Glucocoricoids
- Immunosuppressives:
Azathiprine
Cyclophosphamide
Methotrexate - Antioxidants:
Acetylcysteine
Definition of sarcoidosis?
Who does it typically affect?
And it presents with one or more of the following: (3)
It is characterized pathologically by the presence of noncaseating granulomas in involved organs (multisystem granulomatous disorder)
Typically affects young adults, initially presents w/ one or more of the following:
- Bilateral hilar lymphadenopathy
- Pulmonary reticular opacities
- Skin, joint and/or eye lesions
Sarcoidosis presentation?
3
Dyspnea
Cough
Chest pain
Treatment of pulmonary sarcoidosis:
Many pts experience remission how?
Why is it dificult to guide treatment and follow progression of the disease?
What is the cause of the disease?
A large number of patients undergo spontaneous remission or have a benign clinical course
No easy way to assess dz activity and severity, so predicting clinical course and prognosis of disease is difficult
Marked variability in presentation and clinical course make it difficult to develop treatment guidelines
The cause of the dz is unknown so no specific treatment exists
Indications for treatment of pulmonary sarcoidosis? 3
What would we treat with?
- Worsening pulmonary sx: cough, dyspnea, chest pain or discomfort, and hemoptysis
- Deteriorating lung function
- Progressive radiographic changes
Daily glucocorticoids
If improvement slow taper
If reactivation of disease increase to last effective dose and treat for 3-6 months, some patient’s require maintenance dose
Describe the pic on slide 58 of the lecture that shows a stage two sarcoidosis. What two things characterize it as this?
Bilateral pleural effusions and hilar lymphadanopathy
Eosinophilic pneumonias presents how many days after the drug has started?
What are the symtpoms? 4
What will the radiograph show? 2
Present 2-10 days after drug started
Symptoms: dry cough, fever, chills and dyspnea
Radiograph: eosinophilic pleural effusion + patchy or diffuse pulmonary infiltrates
What drugs are associated with this?
8
Nitrofurantoin -Tricyclic antidepressants Sulfonamides -Hydralazine Penicillin -Isonaizid Thiazides -Gold salts
What are the two types of radiation induced lung injury?
Both are seen in patient’s who have undergone thoracic radiation for ? 6
How is it limited?
Radiation pneumonitis
Radiation fibrosis
breast cancer lung cancer lyphoma esophygeal cancer stomach cancer pancreatic
dose wise
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
What does radiation induced lung injury result from?
The cytotoxic effect is largely due to what?
Radiation-induced lung injury results from the combination of
- direct cytotoxicity upon normal lung tissue and the
- development of fibrosis triggered by radiation-induced cellular signal transduction
DNA damage that causes clonagenic death in normal lung epithelial cells
Many factors affect the development of radiation-induced lung disease:
6
- Method of irradiation
- Volume of lung irradiated
- Dosage of radiation
- Time-dose factor
- Concurrent chemotherapy
- Induction chemotherapy
Radiation-induced Lung Injury clinical manifestations?
5
- Early nonproductive cough
- Dyspnea on exertion or inability to take a deep breath
- Low grade fever
- Chest pain: pleuritic, substernal
- Malaise and weight loss may be seen
Radiation-induced Lung Injury PE findings?
5
- Fine crackles or a pleural rub, sometimes normal
- Pleural friction rib
- Dullness to percussion
- Tachypnea,
- cyanosis or signs of pulmonary HTN (severe)
Radiation-induced Lung Injury:
Need to distinguish from other pulmonary dz such as?
5
- Infection
- Lymphangitic or direct extension of tumor
- Drug-induced pneumonitis
- Hemorrhage
- Cardiogenic edema
Radiation-induced Lung Injury:
Chest XRAy findings?
4
- May be normal
- Patchy alveolar filling defects
- Straight line effect, not conforming to anatomical units but to confines of radiation port is diagnostic (square matching radiation field)
- Small pleural effusions
Treatment of Radiation-induced Lung Injury?
2
Corticosteroids
Inhibition of collagen synthesis
Pneumoconiosis definition?
Examples?
3
nonneoplastic reaction of the lung to inhaled mineral or organic dust
Silicosis
Coal workers
Can be complicated by infection
Coal Worker’s Pneumoconiosis presents how initially?
What may it eventually cause (2) and how does this change the diagnosis?
What will the Xray show?
Asymptomatic
May cause chronic bronchitis and COPD so is then known as industrial bronchitis and is compensable
Radiographically: small opacities can progress to larger opacities and fibrosis
Things that are very vascular are usually suspected as what?
cancer
