Module 7 pulmonary

Question 1

The next restrictive lung disease is called nonspecific interstitial pneumonia (NSIP), what are the features of this disease?

Answer 1

1. Idiopathic
2. Better prognosis
3. Younger patients
4. Uniform fibrosis process (Chicken wire)
5. Two variants: cellular (more inflammation) and fibrosing (more fibrosis)
6. Lacks honey combing change, fibroblast foci and heterogeneity

Question 2

The third fibrosing restrictive lung disease is called cryptogenic organizing pneumonia aka bronchiolitis obliterans organizing pneumonia (BOOP), what are some features?

Answer 2

1. Idiopathic
2. Cough, SOB and recover either spontaneously or with steroids
3. CXR: patchy peribronchial/sub-pleural consolidation
4. Histology: Masson bodies: polypoid plugs of loose CT in alveolar ducts, alveoli and bronchioles ; Alveolar architecture not destroyed

Question 3

The fourth fibrosing restrictive lung disease is called Collagen Vascular disease (CVD). what are some characteristics?

Answer 3

1. group of diseases that affect collagen
2. Disease of which inflammation or weakness in collagen tends to occur
3. Associated with collagen and blood vessel abnormalities; often autoimmune
4. Can affect joints,skin ,blood vessels or other organs

Question 4

What are examples of CVD that have pulmonary manifestations?

Answer 4

1. Sjogren’s sydrome
2. Lupus
3. RA
4. Systemic Sclerosis
5. Dermatomyositis- polymyositis

Question 5

What are some pathologic patterns with CVD?

Answer 5

1. Usual interstitial pneumonia (UIP): pattern of fibrosis
2. non specific interstitial pneumonia (NSIP)
3. Organizing pneumonia
4. Bronchiolitis +/- fibrosis
5. Pleural involvement can occur: pleuritis, pleural nodules (thickening) and pleural effusion

Question 6

Lymphoid Interstitial pneumonia (LIP) is included in the CVD category, what are some features?

Answer 6

1. Idiopathic
2. Expansion of the interstitium by sheets of lymphoid cells
3. Associated with: CT diseases, Autoimmune diseases, HIV
4. May transform to lymphoma

Question 7

What are some other cause of interstitial fibrosis?

Answer 7

Anticancer drugs 
Radiation pneumonitis 
Intravenous heroin 
Autoimmune disorders 
Wegener granulomatosis: inflammation of the blood vessels with granulomas

Question 8

The next category of restrictive lung disease is granulomatous, what two diseases are included in this?

Answer 8

Sarcoidosis

HP: hypersensitivity Pneumonia

Question 9

Sarcoidosis is a restrictive lung disease that affects what part of the population?

Answer 9

Young female
non smokers
african american and danish/swedish women

Question 10

What is the etiology and pathogenesis for sarcoidosis?

Answer 10

Etiology: Idiopathic, dx of exclusion
Path: type IV HSR with non caseating granulomas (also called non necrotizing granuloma) with lymphangetic distribution

Question 11

What is the most common symptom for sarcoidosis?

Answer 11

Asymptomatic

• –found incidentally on CXR: bilateral hilar lymphadenopathy which eventually leads to honey comb lung with pul fibrosis (restrictive lung disease)
• -patients much later on in disease will get dry cough and SOB on exertion

Question 12

Sarcoidosis starts in the lung but spreads. What are the organs and associated symptoms for spreading?

Answer 12

Skin: Erythema Nodosum (painful rash on anterior aspect of LE)
Neuro: facial nerve paralysis
Kidney: fibrosis and therefore renal failure
Liver: Fibrosis and therefore cirrhosis and HCC
Heart: Restrictive cardiomyopathy (which can lead to arrhythmia’s)
Parotid and Lacrimal Glands (sicca syndrome – dry eyes and mouth)
Milkulicz syndrome: Uveitis with bilateral parotid gland involvement
Arthritis in joints

Question 13

Sarcoidosis can also spread to the blood, what do you find in the blood?

Answer 13

Lymphopneia (using up all your CD4 T cells in formation of the granulomas)
Elevated calcium
Why metastatic calcification? Epitheloid histocytes (Activated macrophages) activate vit D precursors – this gives you hypercalcemia
Elevated ACE: not useful for dx just treatment

Question 14

What is the best investigation for sarcoidosis?

Answer 14

Biopsy of the lung or hilar lymph nodes, see granuloma with on central necrosis (this is in big pic on slide 8) and mutli nucleated giant cells

Question 15

On histology for sarcoidosis, what types of inclusion bodies are seen in the granuloma?

Answer 15

Pic on bottom shows ASTEROID BODIES - proteinaceous inclusions of giant cells
Pic on top shows Schaumann body - laminated calcium concretions due to metastatic calcifications

Question 16

What are the complications for sarcoidosis?

Answer 16

1. Cor Pulmonale
2. Contraction atelectasis
3. Facial nerve paralysis
4. Restrictive cardiomyopathy
5. Renal Failure
6. Liver cirrhosis and HCC

Question 17

What is the treatment for sarcoidosis?

Answer 17

Steroids 
Lung transplant (problem is that high chance of recurrence after transplant)

Question 18

Review: What other diseases do non caseating granulomas?

Answer 18

1. Sarcoidosis
2. Berryliosis
3. Crohns
4. Leprosy
5. Hypersensitvity Pneumonitis

Question 19

The next granulomatous restrictive lung disease is hypersensitivity pneumonia (HP) also called extrinsic allergic alveolitis (EAA), what is this disease?

Answer 19

Inflammation of alveoli caused by HSR to inhaled organic antigens (Cotton, hay, grain or compost)

Question 20

What is the etiology and pathogenesis of HP/EAA?

Answer 20

Etiology: Inhaled organic antigens(often called farmers lung and related to occupational exposures) — damage to level of alveoli — restrictive lung disease —– decreased capacity, lung, compliance and total lung volume.
Pathogenesis: immune complexes mediated (type III) and cell mediated (type II) HSR to an extrinsic antigen

Question 21

What is the histopathology of HP/EAA?

Answer 21

1. Airway centered process
2. Chronic inflammatory process
3. Organizing pneumonia
4. GIant cells and poorly formed non-necrotizing granuloma

Question 22

In HP/EAA acute symptoms occur soon after exposure and symptoms improve when what happens?

Answer 22

when patient leaves the environment where Ag is located

Question 23

The next set of restrictive lung diseases are smoking related, what are the associated diseases?

Answer 23

Respiratory Bronchiolitis-Interstitial Lung Disease (RBILD)

Desquamative Interstitial Pneumonia (DIP)

Question 24

In RBILD, what are some features?

Answer 24

1. Seen in smokers in their 40s and 50s
2. Accumulation of smoker’s macrophages within the lumen of respiratory bronchioles
3. Associated with peribronchiolar fibrosis
4. Responds to steroids and smoking cessation

Question 25

In DIP ,what are some features?

Answer 25

1. Rare pneumonia, seen in smokers 2. Prominent filling of alveolar spaces by alveolar macrophages 3. Minimal alveolar fibrosis

Question 26

Now moving on to the infectious diseases of the lung, first lets talk about pneumonia. In general what is pneumonia?

Answer 26

1. Alveoli fill with fluid, hindering oxygenation 2. Infection of the lung --acute or chronic 3. 1/6th of all US deaths 4. Fever, cough, SOB and CP

Question 27

Review: what are the stages of bacterial pneumonia?

Answer 27

Acute congestion: exudate rich in fibrin, RBC and PMNs in alveolar lumen Red hepatization: more PMNs controlled infection Grey Hepatization: congestion and fibrin disappear, PMNs replaced by macrophages Resolution: 10-14 days complete (not that lobar will resolve with or without abx just might take longer)

Question 28

What are some complications in bacterial pneumonia?

Answer 28

``` Abscess Empyema Organization (Scar tissue) Bacteremia Recurrent pneumonias, bronchiolitis obliterans ```

Question 29

Typical pneumonia involves the alveolar spaces and includes lobar and lobular (Bronchopneumonia), what are some features of lobar pneumonia?

Answer 29

Lobar: - -young immunocompetent - --strep. pneumonia - --complete resolution (with or without abx) - -spreads via the pores of kohn (Architecture stays intact, confluent consolidation of entire lobe and bronchi are not involved)

Question 30

what are some features of lobular or bronchopneumonia

Answer 30

Lobular: Patchy and bronchi & bronchioles are damaged --Klebsiella: alcoholics, diabetics --- aspiration (red currant jelly sputum) ---Pseudomonas: CF, ventilators, burns, lung abscesses and cavitations NOTE: Klebsiella and Pseudomonas both cause necrotizing pneumonia because they are angio invasive --- endothelial damage --S. aureus: following viral infection (like flu) --Legionella: contaminated H2O and ACs, become septic very fast GI and pulmonary and liver (hep) and Kidney (oliguria) and neuro (because of SIAHD --- retain excess water and retain sodium so hyponatremia)

Question 31

What are complications of bronchopneumonia?

Answer 31

``` Lung abscess Aspergilloma Empyema Compression atelectasis Septicemia --- DIC and pleural thickening ```

Question 32

In community acquired acute pneumonia, what are the symptoms and associated bacterial agents?

Answer 32

High fever, shaking, chills, pleuritic chest pain 1. S. pneumonia: most common, lower lobe or right middle lobe. Underlying chronic diseases: CHF, COPD, DM. patient with decreased splenic function -- give vaccine 2. H. Influenza: most common cause of COPD exacerbation. Underlying: COPD, CF and Bronchiectasis Capsule B present: target for vaccine 3. Moraxella Catarrhalis: 2nd most common cause of COPD exacerbation (old ppl) 4. S. Aureus: most likely to cause abscesses after a viral resp illness (measles in kids and flu in adults) . IVDA = right sided endocarditis. 5. K. pneumonia: extensive destruction: malnourished, chronic alcoholics and DM. Red currant jelly 6. P. aeruginosa: progressive necrotizing pneumonia in CF and burn patients 7. L. pneumophilia: prevents phagosome fusion: aquatic environments

Question 33

In community acquired atypical pneumonia (aka walking pneumonia) this involves the interstitium, what is the etiology?

Answer 33

Obligate intracellular bacteria: most common= mycoplasma Chlamydia (psittaci (in parrots) and pneumonia) and Coxiella Burnetti (from sheep; no rash in Q fever) Virus: CMV and Parainfluenza

Question 34

What is the hematological complication of mycoplasma?

Answer 34

Cold autoimmune hemolytic anemia (Happens in cold temps in tips of finger and toes)

Question 35

what is the pathogenesis for community acquired atypical pneumonia?

Answer 35

Alveolar spaces are free empty, inflammation in alveolar wall (Thickened septae) ---- cough is dry

Question 36

What is the presentation for community acquired atypical pneumonia?

Answer 36

mild symptoms in the beginning (dry cough and low grade fever) hyaline membrane

Question 37

Pneumocystis Jirovecii, is a fungal cause to community acquired atypical pneumonia, however how is the histology different?

Answer 37

Alveolar spaces are filled with this acellular cotton candy exudate (so no production of mucus because the exudate is so thick)

Question 38

What would you find on examination, CXR and serology for a patient with community acquired pneumonia?

Answer 38

No dullness on percussion --- alveolar spaces are empty Worse than what xray shows Xray: interstitial infiltrates; ground glass opacity Serology: IgM and IgG (most accurate) with antibody titers if bacteria If viral PCR If p. jirovecii: then bronchoalveolar lavage

Question 39

What is a complication of ARDS?

Answer 39

Hyaline membrane on autopsy | (damage to type II pneumocysts because the organism is right there is the interstitium

Question 40

Does atypical pneumonia respond to abx?

Answer 40

Yeh if its a bacterial cause it will

Question 41

What cells are seen in atypical pneumonia?

Answer 41

Lymphocytes called lymphomono nuclear | no PMNs