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IPAP JUNIOR SEM. > ILD And Vacular Dz > Flashcards

ILD And Vacular Dz Flashcards

1
Q

What is the definition of ILD

A

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

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2
Q

What are the common S/s of ILD

A

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

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3
Q

What is the CXR finding in ILD

A

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

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4
Q

Does ILD effect the airways proximal to the bronchioles

A

NO!

Leads to obliteration of capillaries & fibrosis

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5
Q

What is the pathophy of ILD

A

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

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6
Q

What is the general etiology of ILD

A

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

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7
Q

What are the common CXR findings of ILD

A

Diffuse ground glass pattern

Progression to Reticular/linear infiltrates

Nodules (reticulonodular infiltrates)

Ill-defined nodules with air bronochograms
(Acinar rosettes)

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8
Q

What is the common CXR finding in sarcoidosis

A

hilar and mediastinal adenopathy

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9
Q

What is the common CXR finding in Berylliosis

A

hilar and mediastinal adenopathy

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10
Q

What is the common CXR finding in Silicosis

A

hilar and mediastinal adenopathy

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11
Q

What is the common CXR finding in chronic eosinophilic pneumonia

A

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

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12
Q

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

A

Chronic eosinophilic pneumonia

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13
Q

What are the adv/ disadvantages of CXR

A

can be helpful but often not diagnostic, low radiation

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14
Q

What are a the adv/ disadv of CT

A

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

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15
Q

What are the procedural complications of Tissue Bx

A

Bleeding, pneumothorax, truama

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16
Q

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

Think

A

ILD- Idiopathic pulm fibrosis

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17
Q

What type of infiltrates are seen in crypogenic organizing pneumonia

A

Migratory infiltrates

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18
Q

What type of infiltrates are seen in chronic eosinophilic pneumonia

A

Migratory infiltrates

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19
Q

What is the most common type of ILD

A

Idiopathic pul fibrosis

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20
Q

What is the appraoch to idiopathic interstitium pneumonias

A

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

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21
Q

What is “usual” interstitium pneumonia

A

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

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22
Q

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?

A

UIP, usually interstitium pneumonia

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23
Q

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

A

Idio pulm fibrosis

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

24
Q

What is the dx standard for IPF and its tx

A

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

25
Q

What is the general prognosis of IPF

A

Progressive decline in pulmonary function ultimately resulting in death

5 year survival 50%

Average survival from time of dx: 2-5 yrs

26
Q

What is the MOA of nintedanib

A

Tyrosine kinase inhibitor

27
Q

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?

A

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

28
Q

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

A

Switch them to methotrexate

29
Q

What is stage 1 sarcoidosis on CXR

A

Bilateral hilar adenopathy

30
Q

What is stage II sarcoidosis on CXR

A

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

31
Q

What is stage III sarcoidosis on CXR

A

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

32
Q

What is stage IV sarcoidosis on CXR

A

Advanced fibrotic upper lobe changes (stage IV)

33
Q

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

A

Sarcoidosis

34
Q

How can sarcoidosis effect the heart

A

Can lead to restrictive cardiomyopathy and dys-rhythmias

35
Q

What major lung complications can sarcoidosis cause

A

Lung complications: fibrosis, pneumothorax, hemoptysis, respiratory failure

36
Q

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

A

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

37
Q

A pt presents with bilateral hilar lymphadenopathy, erythema nodosum and migratory poly arthralgias

What is this the triad for and what is the tx

A

Lofgren syndrome- Sacroidosis

No need to perform Bx

treat with NSAIDS, low dose glucocorticoids, colchicine and hydroxychlorquine

38
Q

A pt present with fever uveitis and parotisis

Think?

A

Heerfordt Waldenstrom Sydome

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

39
Q

What pattern should sarcoidosis have on PFT

A

Restrictive with a decreased diffusing capacity

40
Q

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

Think?

A

Sarcoidosis

41
Q

You find hemosiderin-laden macrophages on bronch lavage

What dz is this the Dx for

How would you treat this Dz

A

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

42
Q

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

A

CXR reveals diffuse, bilateral alveolar infiltrates

. A chronic interstitial infiltrate may develop after repeated episodes, infrequently with hilar and mediastinal adenopathy

43
Q

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

A

Good pasture has postive anti GBMs and renal involvement with microscopic hematauria

Hemosiderosis does not

44
Q

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 ?

A

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)

45
Q

What is the dx for eosiphilic Granulamatosis with polyangitis

A

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

46
Q

What is the Dx for granulomatic with polyangitis

A

Serologic testing (C-ANCA)

and biopsy of lung, sinus tissue, or kidney with demonstration of necrotizing granulomatous vasculitis

47
Q

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?

A

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

48
Q

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

A

Increased risk for pulmonary infection with Nocardia or fungi

49
Q

What medications increase the risk of Eosinophilic pulmonary syndromes

A

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

50
Q

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

A

Eosinophilic pulm syndomre

51
Q

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?

A

Acute Eosinophilic Pneumonia

CXR: abnormal, but nonspecific

Tx: Response to corticosteroids is usually dramatic

52
Q

A woman presents with fever, night sweats, and wt loss, and Dyspnea

Is a non smoker

BAL reveals marked increased eospinophils

Think? CXR? Tx?

A

Chronic Eosinophilic Pneumonia

CXR: Infiltrates commonly involve upper lung fields

TX: oral prednisone and taper

53
Q

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?

A

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
54
Q

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

Think? TX?

A

Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)

Tx: Cyclophosphamide and prednisone

RITUXIMAB can be used
And Bactrim for relapsing cases

55
Q

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?

A

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

IPAP JUNIOR SEM. (43 decks)

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