ILD And Vacular Dz

Question 1

What is the definition of ILD

Answer 1

A group of respiratory disorders with many potential causes featuring variable degrees of pulmonary inflammation and fibrosis

Question 2

What are the common S/s of ILD

Answer 2

Cough, dyspnea, restrictive pattern on PFTS
Can be with a co existing airflow obstruction (mixed) pattern
Decreased DLCO

INCREASED alveolar-arterial oxygen difference at rest or during exertion

Question 3

What is the CXR finding in ILD

Answer 3

Dyspnea, late inspiratory crackles and CXR with septal thickening and reticulonodular changes.

Question 4

Does ILD effect the airways proximal to the bronchioles

Answer 4

NO!

Leads to obliteration of capillaries & fibrosis

Question 5

What is the pathophy of ILD

Answer 5

injury leading to attempted repair causing fibrosis and a honeycomb pattern on CXR and secondary pulm hypertension

Question 6

What is the general etiology of ILD

Answer 6

No specific cause found in most patients

Most common known causes are medications & inorganic/organic dusts, radiation

Strong correlation with connective tissue disease
—Rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, dermatomyositis, Sjogren syndrome

Question 7

What are the common CXR findings of ILD

Answer 7

Diffuse ground glass pattern

Progression to Reticular/linear infiltrates

Nodules (reticulonodular infiltrates)

Ill-defined nodules with air bronochograms
(Acinar rosettes)

Question 8

What is the common CXR finding in sarcoidosis

Answer 8

hilar and mediastinal adenopathy

Question 9

What is the common CXR finding in Berylliosis

Answer 9

hilar and mediastinal adenopathy

Question 10

What is the common CXR finding in Silicosis

Answer 10

hilar and mediastinal adenopathy

Question 11

What is the common CXR finding in chronic eosinophilic pneumonia

Answer 11

peripherally located pulmonary infiltrates in the upper and middle lung zones with relatively clear perihilar and central zones

Question 12

You see peripherally located pulmonary infiltrates in the upper and middle lung zones with relatively clear perihilar and central zones CXR think ?

Answer 12

Chronic eosinophilic pneumonia

Question 13

What are the adv/ disadvantages of CXR

Answer 13

can be helpful but often not diagnostic, low radiation

Question 14

What are a the adv/ disadv of CT

Answer 14

can be much more diagnostic than CXR , higher radiation (but still low risk)

Question 15

What are the procedural complications of Tissue Bx

Answer 15

Bleeding, pneumothorax, truama

Question 16

You see decreased lung volumes, honeycombing, perihilar reticular changes with a lower lobe predominance

Think

Answer 16

ILD- Idiopathic pulm fibrosis

Question 17

What type of infiltrates are seen in crypogenic organizing pneumonia

Answer 17

Migratory infiltrates

Question 18

What type of infiltrates are seen in chronic eosinophilic pneumonia

Answer 18

Migratory infiltrates

Question 19

What is the most common type of ILD

Answer 19

Idiopathic pul fibrosis

Question 20

What is the appraoch to idiopathic interstitium pneumonias

Answer 20

1st step—identify pts whose disease is truly idiopathic
i.e., not infectious, med-related, environmental, occupational or connective tissue related

Careful medical hx

CXR & high-resolution CT may be diagnostic

Lung biopsy often required for definitive dx

Question 21

What is “usual” interstitium pneumonia

Answer 21

lung injury characterized by patchy collagen fibrosis with associated scarring distributed in a peripheral, sub-pleural fashion with honeycomb changes.

Question 22

You see lung injury characterized by patchy collagen fibrosis with associated scarring distributed in a peripheral, sub-pleural fashion with honeycomb changes.

What is this?

Answer 22

UIP, usually interstitium pneumonia

Question 23

A pt comes in recently diagnosed with asthma, on your workup you hear bibasilar inspiratory crackles and note clubbing of the fingers

The pt has a NML SPO2 at rest with exertional desat

There is a restrictive pattern on PFTS

And the CXR shows bilateral reticualr opacities.

What is this? And what would you expect to see on CT

Answer 23

Idio pulm fibrosis

CT: Patchy reticular opacities and honeycombing with a peripheral, bibasilar predominance
And
Minimal ground-glass opacities

Question 24

What is the dx standard for IPF and its tx

Answer 24

Dx: Lung Bx
(May not be needed if classis s/s and Ct are high index)

Tx: Prednisone is often ineffective

Nintedanib and pirfenidone are more effective but very very $$$

Tx the pt with supplemental O2 if sat is below 89

Only definite Tx is Transplant

Question 25

What is the general prognosis of IPF

Answer 25

Progressive decline in pulmonary function ultimately resulting in death 5 year survival 50% Average survival from time of dx: 2-5 yrs

Question 26

What is the MOA of nintedanib

Answer 26

Tyrosine kinase inhibitor

Question 27

This is the second most common ILD Presents with granulomatous disease of unknown etiology Can often involve the skin, eyes, lymph, neuro, liver, kidney, and heart Highest incidence is in African America women S/s Cough, dyspnea, chest pain, fatigue, fever, and wt loss With erythema nodosum, lupus perino , iritis, arthritis, peripheral neuropathy, parotid enlargement, hepatosplenomegaly, and muscle weakness Think? Dx? CXR? Tx?

Answer 27

Sarcoidosis CXR: Often asymptomatic with abnormal CXR —Bilateral hilar adenopathy common —Right paratracheal lymphadenopathy Dx: biopsy on noncaseating granulomas Tx: oral corticosteroids or methotrexate if unable to handle steroids

Question 28

You are treating a pt with sarcoidosis and they develop myopathy, difficult to control DM, excessive wt gain, osteoporosis from corticosteroid tx What should you do

Answer 28

Switch them to methotrexate

Question 29

What is stage 1 sarcoidosis on CXR

Answer 29

Bilateral hilar adenopathy

Question 30

What is stage II sarcoidosis on CXR

Answer 30

Hilar adenopathy w/ parenchymal involvement mainly in middle or upper lobes (stage II)

Question 31

What is stage III sarcoidosis on CXR

Answer 31

Parenchymal involvement alone mainly in upper lobes (stage III) —Diffuse reticular infiltrates

Question 32

What is stage IV sarcoidosis on CXR

Answer 32

Advanced fibrotic upper lobe changes (stage IV)

Question 33

A young black woman with erythema nodosum should raise high index of suspicion for what dz

Answer 33

Sarcoidosis

Question 34

How can sarcoidosis effect the heart

Answer 34

Can lead to restrictive cardiomyopathy and dys-rhythmias

Question 35

What major lung complications can sarcoidosis cause

Answer 35

Lung complications: fibrosis, pneumothorax, hemoptysis, respiratory failure

Question 36

What is the minim follow up criteria for a pt with sarcoidosis

Answer 36

At minimum-- annual physical exam, PFTs, CMP, ophthalmologic exam, CXR, ECG

Question 37

A pt presents with bilateral hilar lymphadenopathy, erythema nodosum and migratory poly arthralgias What is this the triad for and what is the tx

Answer 37

Lofgren syndrome- Sacroidosis No need to perform Bx treat with NSAIDS, low dose glucocorticoids, colchicine and hydroxychlorquine

Question 38

A pt present with fever uveitis and parotisis Think?

Answer 38

Heerfordt Waldenstrom Sydome Rare form of sarcoidosis that can lead to sicca that can mimic shorten syndrome

Question 39

What pattern should sarcoidosis have on PFT

Answer 39

Restrictive with a decreased diffusing capacity

Question 40

A pt lanes present with leukopenia, elevated ESR, hypercalcemia, and hypercaliuria, and elevated ACE levels Think?

Answer 40

Sarcoidosis

Question 41

You find hemosiderin-laden macrophages on bronch lavage What dz is this the Dx for How would you treat this Dz

Answer 41

Idiopathic pulmonary hemosiderosis Common in children and y/a Causes recurrent pulm hemorrhage Iron deficieny is typical Treatment: acute hemorrhage episodes--corticosteroids may be useful

Question 42

What CXR findings would you expect to see in a pt with Idio pulm hemosiderosis

Answer 42

CXR reveals diffuse, bilateral alveolar infiltrates . A chronic interstitial infiltrate may develop after repeated episodes, infrequently with hilar and mediastinal adenopathy

Question 43

How can you tell the difference between Goodpasture and Idiopathic pulm hemosiderosis

Answer 43

Good pasture has postive anti GBMs and renal involvement with microscopic hematauria Hemosiderosis does not

Question 44

A 30-40 year old man presents with hemoptysis, anemia. Dyspnea, cough and fever, with hypoxia CXR show diffuse bile alveolar infiltrates Labs reveal iron def anemia, and microscopic hematauria What is the Dx for this condition and Tx ?

Answer 44

This the Anti-glomerular basement membrane disease (Goodpasture Syndrome) Dx: IgG on immunofluorescence & anti-glomerular basement membrane antibodies Tx: combo of corticosteroids, cyclophosphamide, plasmapharesis to remove antibodies DDX: idiopathic Pulm Hemosiderosis However hemosiderosis doesnt have urine involvement or antiBM antibodies)

Question 45

What is the dx for eosiphilic Granulamatosis with polyangitis

Answer 45

Lung, skin or nerve biopsy: histologic features of fibrinoid necrotizing epithelioid & eosinophilic granulomas

Question 46

What is the Dx for granulomatic with polyangitis

Answer 46

Serologic testing (C-ANCA) and biopsy of lung, sinus tissue, or kidney with demonstration of necrotizing granulomatous vasculitis

Question 47

This is a rare dz where phospholipids accumulate witching the alveolar spaces Can be idiopathic or 2/2 immunocomp, CA, inhalation of silica, titanium, or aluminum, or post infectious with TB S/s progressive dyspnea, and cough THink? DX? CXR? Tx?

Answer 47

pulmonary alveolar proteinosis Dx: Bronchoalveolar lavage -Characteristic findings of milky appearance and PAS-positive lipoproteinaceous material Some cases, will need transbronchial or surgical lung bx —Amorphous intra-alveolar phospholipid CXR: bilateral alveolar infiltrates suggestive of pulmonary edema Tx: periodic whole lung lavage with warm saline, under general anesthesia with supplement O2 post treatment

Question 48

What infection are pts with pulm alveolar proteinosis at an increased risk for

Answer 48

Increased risk for pulmonary infection with Nocardia or fungi

Question 49

What medications increase the risk of Eosinophilic pulmonary syndromes

Answer 49

Exposure to medications (nitrofurantoin, phenytoin, ampicillin, acetaminophen, ranitidine)

Question 50

infections with helmith s (Ascaris, hookworms, Strongyloides) or filariae (Wuchereria bancrofti, Brugia malayi) Lead to what kind of pulmonary syndrome e

Answer 50

Eosinophilic pulm syndomre

Question 51

A pt present with acute, febrile illness, cough, and dyspnea wit rapid progresision to resp failure Often assoc with recent initiative or resumption of smoking BAL reveals increased eosinophils without serum eosinophilia Think>? CXR? Tx?

Answer 51

Acute Eosinophilic Pneumonia CXR: abnormal, but nonspecific Tx: Response to corticosteroids is usually dramatic

Question 52

A woman presents with fever, night sweats, and wt loss, and Dyspnea Is a non smoker BAL reveals marked increased eospinophils Think? CXR? Tx?

Answer 52

Chronic Eosinophilic Pneumonia CXR: Infiltrates commonly involve upper lung fields TX: oral prednisone and taper

Question 53

This is an idiopathic disease characterized by -Glomerulonephritis -necrotizing granulomatous vasculitis of upper & lower resp. tracts (pulmonary infiltrates, nodules or cavitations), and varying degree of small vessel vasculitis S/s chronic sinusitis, chronic serous otitis (fluid in middle ear), with fever, and skin rash and wt loss +/- hemoptysis, cough, CP and dyspnea Think? DX? CXR? Tx?

Answer 53

Granulomatosis with polyangiitis Aka Wegeners Dx: serologic testing (C-ANCA) and biopsy of lung, sinus tissue, or kidney with demonstration of necrotizing granulomatous vasculitis CXR: Characteristic sign of lung disease is nodular pulm. infiltrates w/ cavitation Tx: - Cyclophosphamide - Oral corticosteroids (prednisone) - Rituximab (Truxima) – antineoplastic, -Anti-CD20 (monoclonal antibody) - Bactrim to prevent relapses in some cases

Question 54

You are evaluating a pt and you find Tracheal stenosis, facial deformities (saddle nose), strawberry gums, petechiae/purpura Think? TX?

Answer 54

Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) Tx: Cyclophosphamide and prednisone RITUXIMAB can be used And Bactrim for relapsing cases

Question 55

This is a Idiopathic multisystem vasculitis of small and medium-sized arteries in pts w/ asthma S/s chronic rhinosinusitis, asthma and prominent peripheral blood eosinophilia Think? Dx? CXR? Tx?

Answer 55

Eosinophilic granulomatosis with polyangiitis AKA Churg-Strauss syndomome Dx: Serum eosinophilia Lung, skin or nerve biopsy: histologic features of fibrinoid necrotizing epithelioid & eosinophilic granulomas CXR: transient opacites to multiple nodules TX; Corticosteroids and Medolizumab (interlukin 5 antagonist) And cyclophosphamide for severe disease