Obstructive/Restrictive Lung Diseases

Question 1

mild, moderate, vs. severe asthma pathology?

Answer 1

Mild Asthma: edema and hyperemia of the mucosa and infiltration of the mucosa with mast cells, eosinophils, and lymphocytes.

Moderate Asthma: chemokines eotaxin, RANTES, macrophage inflammatory protein 1 alpha, and interleukin 8 lead to inflammation and smooth muscle constriction

Severe: hypertrophy and hyperplasia of airway glands and smooth muscle lead to severe airway thickening

Question 2

asthma categories?

Answer 2

Mild intermittent: symptoms present for 2 days/week or less, or 2 nights/month or less (give shorta acting albuterol)

Mild persistent: symptoms present for > 2 days/week but less than once daily, or > 2 nights/ month (SABA, ihnaled corticosteroid)

Moderate persistent: symptoms present daily or greater than once/night (SABA as needed, low/med dose of corticosteroid, LABA)

Severe persistent asthma: symptoms are continual during the day and frequent at night (SABA as needed, high dose corticosteroid, LABA)

Question 3

Omalizumab

Answer 3

another tx for asthma - monoclonal antibody in patients with moderate to severe persistent asthma who have shown reactivity to an allergen and whose symptoms are inadequately controlled by an inhaled corticosteroid.

Question 4

bronchiectasis in upper lobe?

Answer 4

think CF

Question 5

bronchiectasis in lower lobe?

Answer 5

think aspiration

Question 6

bronchiectasis in right middle lobe with lingular predominance

Answer 6

MAI

Question 7

bronchiectasis in center of lung

Answer 7

bronchopulmonary aspergillosis

Question 8

acute farmer’s lung?

Answer 8

Develops after large exposure to moldy hay or contaminated compost

Symptoms often spontaneously resolve within 12 hours to days if antigen exposure is eliminated or avoided

Acute farmer’s lung manifest as new onset of fever, chills, nonproductive cough, chest tightness, dyspnea, headache , and malaise.

If the inhalational exposure is large, patients may develop acute respiratory failure

Question 9

subacute farmer’s lung?

Answer 9

Manifest as chronic cough, dyspnea, anorexia, and weight loss

Subacute disease is insidious in onset and may occur over weeks to months.

Question 10

Chronic Farmer’s Lung

Answer 10

Results from prolonged and continuous exposure to antigen
Patients may have irreversible lung damage
Patients may experience severe dyspnea at rest or with exertion

Question 11

Samter’s triad

Answer 11

ASA, NSAID sensitivity –> nasal polyps and asthma

Question 12

allergic bronchopulmonary aspergillosis?

Answer 12

tx: prednisone

Occurs in asthmatics and CF patients from a hypersensitivity reaction to Aspergillus colonization of the tracheobronchial tree

This syndrome may cause fever and pulmonary infiltrates that are unresponsive to antibacterial therapy.

Patients often have a cough and produce mucous plugs, which may form bronchial casts. Possible hemoptysis.

People with asthma who have ABPA are usually poorly controlled asthmatics with difficulty tapering off oral corticosteroids

ABPA may occur in conjunction with allergic fungal sinusitis, with symptoms including chronic sinusitis with purulent sinus drainage.

Question 13

sarcoidosis

Answer 13

Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized pathologically by the non-caseasting granuloma.

Can effect almost any organ

90% of patients present with mediastinal or hilar lymphadenopathy and parenchymal lung disease

Initial presentation may be an abnormal chest x-ray with mediastinal and hilar adenopathy

Question 14

Lofgren’s syndrome?

Answer 14

1. Acute manifestation of sarcoidosis
2. Erythema nodosum- More common in women
   Arthritis- More common in men
3. Bilateral Hilar
4. Lymphadenopathy

Prognosis is favorable

Question 15

sarcoidosis cxr staging?

Answer 15

Stage 0: Normal
Stage 1: Bilateral hilar adenopathy
Stage 2: Bilateral hilar adenopathy and parenchymal infiltrates
Stage 3: Parenchymal infiltrates without lymphadenopathy
Stage 4: Advanced parenchymal disease with fibrosis

Question 16

sarcoidosis tx?

Answer 16

prednisone, immunosuppressives if not responsive

Question 17

diseases associated with silicosis?

Answer 17

TB, COPD, chronic bronchitis, rheumatoid arthritis, lung cancer

Hilar adenopathy with eggshell calcification is strongly suggestive
Progressive massive fibrosis is characterized by coalescence of the nodules with larger mass lesions
Acute silicosis displays air space and interstitial pattern on x-ray

Question 18

CWP?

Answer 18

Usually no symptoms or signs
Can have symptoms of bronchitis
May lead to progressive massive fibrosis

no associated TB or cancer, however silicosis is also common in coal workers

Question 19

Caplan syndrome

Answer 19

rheumatoid arthritis with large cavitary pulmonary nodules associated with silicosis and coal worker’s pneumoconiosis

Question 20

asbestosis

Answer 20

Ferruginous bodies, asbestos bodies

Symptoms
Dyspnea
Dry cough
Chest tightness/pain

Signs
Inspiratory crackles
clubbing

associated with: mesothelioma, lung cancers, pleural effusions

Chest x-ray
Pleural plaques
Pleural effusion 10-15 year latency
Pleural thickening

Question 21

beryllium disease

Answer 21

at risk: Aerospace
Electronics
Ceramics
Metal
Nuclear

Acute Toxic Pneumonitis: high exposure can lead to a hypersensitivity response that is now rare due to better recognition of beryllium associated disease: see dyspnea, cough, c/p, blood tinged sputum, crackles

Chronic beryllium disease: clinical features are similar to sarcoidosis ranging from asymptomatic to severe granulomatous restrictive lung disease: see dyspnea, cough, c/p, w/l, fatigue, arthralgias

CXR: shows enlarged hilar/mediastinal nodes, multiple lung nodules or fibrosis, hyperinflation and honeycombing

Positive BeLPT( beryllium lymphocyte proliferation test performed on blood or BAL fluid

Question 22

Usual Interstitial Pneumonia

Answer 22

= same as idiopathic pulmonary fibrosis (UIP is the pathologic process of IPF)

• pathogenesis unknown- BUT SEE PATCHY areas of parenchymal fibrosis and interstitial inflammation interspersed between areas of relatively preserved lung tissue
• FIBROSIS is the most prominent component of the pathology (due to collagen deposition and type II hyperplasia)
• HONEYCOMBING – cystic airspace from retraction of surrounding fibrotic tissue

Most important disorder in this category is idiopathic pulmonary fibrosis

Question 23

Desquamative Interstitial pneumonia

Answer 23

“Smoker’s macrophages in alveoli” - see minimal fibrosis (not UIP precursor)

Pigmented macrophages in respiratory bronchioles secondary to smoking

***Smoking is believed to be an important underlying cause

• see ground glass appeaerance on imaging, lung biopsy shows macrophages with little fibrosis
• prognosis is better than IPF
• responds to corticosteroids usually and stopping smoking

Question 24

Respiratory bronchiolitis - (associated interstitial lung disease)

Answer 24

Related to DIP- see pigmented macrophages in bronchioles full of junk!

Interstitial inflammation is not present

Almost always associated with SMOKING!

Most important intervention is smoking cessation

Question 25

nonspecific interstitial pneumonia

Answer 25

** NOT as sick or progressive as IPF Mononuculear cell infiltration within the alveolar walls Uniform process Fibrosis is variable, but less than UIP - NO HONEYCOMBING Idiopathic or connective tissue disorder Better prognosis - all lesions are same age - ground glass appearance on imaging- lung biopsy shows predominantly inflammatory response in the alveolar walls , with little fibrosis - responds to corticosteroids usually

Question 26

Acute Interstitial pneumonia

Answer 26

** rapid onset (sx similar to ARDS), acute resp. failure following illness of interstitium filled with inflammatory cells Organizing or fibrotic stage of alveolar damage, which is the histologic pattern seen in ARDS. No initial trigger identified. Histology shows fibroblast proliferation and type II pneumocyte hyperplasia in the setting of what appears to be organizing alveolar damage Imaging with ground glass appearance Note: this one is on its own, and is associated with rapid lung failure due to ARDS like presentation!

Question 27

Cryptogenic organizing pneumonia

Answer 27

COP = Bronchiolitis obliterans organizing Pneomonia Organizing fibrosis (granulation tissue) in small airways - NO HONEYCOMBING, no interstitial fibrosis, just filling up of the airways - see cough and dysnpea Mild degree of chronic interstitial inflammation- Intraluminal airway involvement is a key feature (see alveoli filled with fibroblasts) Idiopathic Differential: infections, toxic inhalants, or connective tissue disease Chest x-ray often mimics pneumonia with one or more alveolar infiltrates Response to steroid is dramatic and occurs over days to weeks.

Question 28

idiopathic pulmonary fibrosis

Answer 28

= A type of usual interstitial pneumonia * No recognizable inciting agent - unpredictable disease * Presents between ages 50-70 * Insidious onset Dysregulated pattern of FIBROSIS in response to alveolar epithelial injury DYSPNEA is the most common complaint RALES on lung examination Patients frequently have clubbing CXR shows bilateral diffuse interstitial pattern thats more prominent at lung bases high resolution CT shows patchy interstitial densities and HONEYCOMBING indicative of irreversible fibrosis prognosis is poor: 2-5 years

Question 29

Rheumatoid arthritis

Answer 29

Primary manifestation is inflammatory joint disease Most common site of thorax involvement is the pleura Pleurisy, pleural effusions or both Lung parenchymal involvement includes one or multiple nodules or the development of interstitial lung disease Interstitial lung disease is usually mild, but can be severe in some cases

Question 30

SLE

Answer 30

Multisystem disease that affects joints and skin Serious organ involvement includes kidneys, lungs, nervous system, and heart Involvement of the thorax includes pleural disease presenting with pleuritic chest pain, pleural effusion, or both Acute pneumonitis in which infiltrates often involve the alveolar spaces as well as the alveolar walls Less frequently chronic interstitial lung disease – extensive fibrosis usually not prominent

Question 31

scleroderma "Progressive systemic sclerosis"

Answer 31

Pulmonary involvement tends to be severe with significant scarring of the pulmonary parenchyma Pulmonary fibrosis strongly associated with an autoantibody to topoisomerase I (antitopoisomerase I, also called Scl70) Pulmonary Artery Hypertension – small pulmonary vessel disease independent of the fibrosis

Question 32

polymyositis-dermatomyositis

Answer 32

Muscles and skin are the primary sites of the inflammatory process Interstitial lung disease is relatively infrequent Respiratory difficulty secondary to weakness of the diaphragm Involvement of the striated muscles in the proximal esophagus may lead to dysphagia and recurrent aspiration pneumonia

Question 33

Pulmonary Langerhans Histiocytosis

Answer 33

- proliferation of dendritic cells in response to smoking * Differential diagnosis of unexplained interstitial disease in the young or middle aged adult * **Disease occurs almost exclusively in smokers Histiocytic cell appears to be an antigen-presenting dendritic or phagocytic cell called a Langerhans cell Cytoplasmic rod like structures called X bodies (Burbeck Granules) which can be seen by electron microscopy Chest x-ray: Nodular or reticulonodular disease Upper lobe prominent HRCT Small cysts in addition to the nodular and reticulonodular changes Cysts may rupture and cause pneumothorax Some cases extensive honeycombing

Question 34

Lymphangioleiomyomatosis

Answer 34

Characterized by proliferation of atypical smooth muscle cells around lymphatics, blood vessels, and airways, accompanied by numerous small cysts throughout the pulmonary parenchyma Occurs almost exclusively in women of childbearing age Develops in 30-40% of female patients with the genetic condition tuberous sclerosis complex Clinical Manifestation Dyspnea and cough Vascular involvement may lead to hemoptysis Lymphatic obstruction may produce a chylous pleural effusion Airway involvement may lead to airflow obstruction Rupture of subpleural cysts can lead to spontaneous pneumothorax CXR: shows cystic changes in reticular pattern

Question 35

Goodpastures syndrome

Answer 35

- affects lungs and kidneys: with anti-GBM antibodies against type IV collagen plasmapheresis can be used to tx

Question 36

Wegener Granulomatosis

Answer 36

Upper respiratory tract, lungs, and kidneys– necrotizing small-vessel granulomatous vasculitis Chest x-ray: Nodules, infiltrates, cavitation Diffuse interstitial lung disease is not characteristic cANCA + (PR3 ANCA)

Question 37

Churg-Strauss Syndrome

Answer 37

Peripheral and lung eosinophilia Increased immunoglobulin E levels Systemic necrotizing vasculitis Affects upper and lower respiratory tracts Preceded by allergic disorders such as asthma , allergic rhinitis, sinusitis or drug reaction CXR: shows bilateral pathy, diffuse nodular infiltrates and reticulonodular

Question 38

Chronic eosinophilic pneumonia

Answer 38

Pulmonary interstitium and alveolar spaces are infiltrated by eosinophils and to a lesser extent macrophages Clinical Manifestations Occurs over weeks to months Fever, weight loss, dyspnea, and productive cough Increased eosinophils in the peripheral smear BAL with increased eosinophils tx: corticosteroids

Question 39

pulmonary alveolar proteinosis

Answer 39

* see cough and sputum with gelatinous yellow chunks and bilateral patchy distribution on CXR Primary pathologic process affects the alveolar spaces, not the alveolar cells Alveolar spaces are filled with a proteinaceous phospholipid material (acellular surfactant) that represents components of pulmonary surfactant Clinically: Dyspnea and cough Bilateral alveolar infiltrates HRCT crazy paving pattern produced by thickening of interlobular septa accompanied by ground –glass alveolar filling Superimposed to respiratory infections - Nocardia tx: whole-lung lavage

Question 40

Hypersensitivity pneumonitis

Answer 40

Hyperimmune respiratory syndrome caused by inhalation of a wide variety of allergic antigens that are usually organic (see Type III and Type IV reaction - i.e. immune complex deposition and delayed response hypersensitivity --- this is not a Type I where you would see IgE levels high) suspect if: Intermittent pulmonary and systemic symptoms, associated with a certain environmental exposure i.e. farmers, bird keepers, woodworkers, cheese makers Farming and agriculture – especially dairy cattle Thermophillic actinomycetes in moldy hay, grain, or silage Ventilation/water-related contamination: Thermophillic actinomycetes Mycobacterium avium-intracellulare complex organisms CXR: diffuse reticulondoular, see ground glass opacities

Question 41

amiodarone lung

Answer 41

Usually 2-4 months of treatment at doses greater than 400 mg/day, in elderly - present with exertional dysnpnea as major problem, may mimic pulmonary infection. - See Lipid laden foamy alveolar macrophages - see chronic interstitial pneumonia, organising pneumonia and AIP that can even lead to ARDS on CXR: see multiple areas of dense air space with interstitial fibrosis

Question 42

what things cause increased DLCO?

Answer 42

diffuse alveolar hemorrhage, polycythemia

Question 43

diagnostic criteria for Churg Strauss?

Answer 43

* Asthma * Eosinophils greater than 10% of a differential white blood cell count * Presence of mononeuropathy or polyneuropathy * Non-fixed pulmonary infiltrates * Presence of paranasal sinus abnormalities * Histological evidence of extravascular eosinophils Churg–Strauss syndrome (CSS, also known as eosinophilic granulomatosis with polyangiitis [EGPA] or allergic granulomatosis) is an autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy). It usually (but not always) manifests in three stages. The early (prodromal) stage is marked by airway inflammation; almost all patients experience asthma and/or allergic rhinitis; the second stage is characterized by abnormally high numbers of eosinophils (hypereosinophilia), which causes tissue damage, most commonly to the lungs and the digestive tract. The third and final stage consists of vasculitis, which can eventually lead to cell death and can be life-threatening.

Question 44

causes of decreased DLCO?

Answer 44

DLCO is decreased in any condition which affects the effective alveolar surface area: Hindrance in the alveolar wall. e.g. fibrosis, alveolitis, vasculitis Decrease of total lung area, e.g. Restrictive lung disease Chronic obstructive pulmonary disease (COPD), due to large residual air stuck in the lungs Uneven spread of air in lungs, e.g. emphysema Pulmonary embolism Cardiac insufficiency Pulmonary hypertension Bleomycin (upon administration of more than 200 units) Chronic heart failure[4] Anemia-due to decrease in blood volume Amiodarone high cumulative dose; more than 400 milligrams per day

Question 45

criteria for exudate?

Answer 45

1. Pleural fluid protein/serum protein > 0.5 2. Pleural fluid LDH/Serum LDH > 0.6 3. Pleural fluid LDH more than two-thirds normal upper limit for serum

Question 46

Heerfordt's Disease

Answer 46

A variant of Sarcoidosis | Characterized by nonsuppurative parotitis, uveitis, mild fever, and facial nerve paralysis

Question 47

IPF immunology?

Answer 47

- restrictive pattern of fibrosing - corticosteroids not indicated (though are useful in nonspecific interstitial pneumonia) - see fibroblast proliferation and deposition of fibrin injury to lung -->increased TGFbeta --> Type II AEC proliferates injury to lung --> vasodilation/coagulation/vascular remodeling --> Type II AEC proliferation * there is evidence of cellular and humoral immunity in IPF: CD4 T cells and autoantibodies Genetic contribution: telomerase mutations, MUC5B mutations

Question 48

sarcoidosis immunology?

Answer 48

- multisystemic noncaseating epithelioid granulomas - can progress to fibrosis with loss of alveolar and bronchial tissue and vascular surface area ** see increase in TNFalpha, IL7, MMP12 - T cells surround granulomas full of macrophages

Question 49

Hypersensitivity Pneumonitis immunology?

Answer 49

- due to inhalation of exogenous organic material - see transient fever, hypoxemia, myalgias, arthrlagias, dyspnea, cough - sx resolve w/out tx provided there isn't rexposure to ag - ex: Bird Breeder's lung - in course of exposure: around 4-12 hours see decreased VC and DLCO2, that resolves in 24 hours after exposure * this is not associated with IgE or eosinophils - higher prevalence in smokers - lymphocytosis is common *** see increased TGFbeta, IL1, IL12, TNFalpha, IL2, IFNgamma, IL6, IL17, IL22 - if persistant --> fibrosis tx with corticosteroids aids recovery initially

Question 50

presence of Eosinophils in BAL?

Answer 50

eosinophilic lung disease - see infiltration in airways, alveoli, or interstitium

Question 51

wheeze

Answer 51

a continuous musical sound that is generated by flow through critically narrowed collapsible bronchi - this is heard in asthma, COPD, pulmonary edema, bronchiolitis, bronchiectasis

Question 52

what do restrictive vs. obstructive diseases show?

Answer 52

R: decreased VC, decreased TLC, RV, FRC O: decreased VC, increased TLC,RV, FRC

Question 53

mild intermittent asthma?

Answer 53

sx presents less than 2 days per week, or less than 2 nights per month tx: SABA as needed

Question 54

mild persistent asthma?

Answer 54

sx present more than 2 days per week - but less than once daily tx: SABA as needed, inhaled corticosteroid or montelukast

Question 55

moderate persistent asthma?

Answer 55

sx are daily or more than once per night tx: SABA as needed, low/medium dose corticosteroid, LABA

Question 56

severe persistent asthma?

Answer 56

sx are continual during the day and freq. at night tx: SABA as needed, high dose corticosteroid, LABA

Question 57

centriacinar emphysema

Answer 57

affects resp. bronchioles distal to terminal bronchiole - occurs w/ smoking

Question 58

panacinar emphysema

Answer 58

affects alveolar ducts, alveoli and coalesce to form bullae - occurs with alpha1 antitrypsin deficiency and with lots of smoking

Question 59

what causes emphysema

Answer 59

imbalance of elastase-antielastase in lung

Question 60

nodular pattern in upper lobes? eggshell calcifications?

Answer 60

Silicosis - seen with mining/sandblasting | - increased risk of TB, COPD, lung cancer, bronchitis

Question 61

ferruginous bodies?

Answer 61

asbestosis

Question 62

signs of asbestosis?

Answer 62

dyspnea, dry cough, c/p insipratory basal crackles/clubbing mesothelioma, lung cancer, pleural effusion and pleural plaques

Question 63

which disease is similar to sarcoidosis? see granulomatous restrictive lung disease, dyspnea, cough, c/p, w/l, fatigue, arthralgias? enlarged hilar lymph nodes, fibrosis

Answer 63

beryllium exposure disease - think aeronautics, electronics, ceramics

Question 64

infectious etiologies leading to bronchiectasis? genetic etiologies? Immune etiologies?

Answer 64

childhood pertussis TB MAI (mycobacterium avium intracellular) - involves the right middle lobe and lingula genetic: CF, ciliary dyskinesia, alpha1at Immune: hypogammaglobulinemia, IgG defciency, HIV, Sjogren's syndrome, RA

Question 65

upper lobe bronchiectasis? lower lobe? right middle lobe? central?

Answer 65

upper: CF lower: aspiration right middle: MAI central: allergic bronchopulmonary aspergillosis

Question 66

tx for sarcoidosis?

Answer 66

prednisone for 3 mos

Question 67

p-anca and IgE elevated along with BUN and creatinine - CXR shows bilateral patchy infiltrates - has asthma and allergies and now has neuritis and maculopapules with marked abdominal pain?

Answer 67

churg- strauss syndrome

Question 68

charcot-leyden crystals? curschmann spiral?

Answer 68

all indicators of asthma - see eosinophils in the serum

Question 69

what drug used to treat rheumatoid arthritis can cause lung disease?

Answer 69

methotrexate lung disease: see SOB, cough, fever - can result in fibrotic inflammatory disease, pulmonary infections or AIP - CT shows diffuse parenchymal opacification and reticular opacities - NSIP is the most common presentation