L13 Interstitial Lung disease Flashcards

1
Q

Diffuse Parenchymal Lung Disease aka

A

Interstitial lung disease

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2
Q

honeycombing

A

clustered cystic air spaces

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3
Q

Interstitial lung disease pathophysiology

A

Extension distortion of the airway and alveolar compartment along with the interstitium causing progressive scarring of lung tissue surrounding the alveoli
Areas of fibrosis alternate with areas of normal lung
Honeycombing

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4
Q

Causes of Interstitial lung disease

A

Idiopathic
Autoimmune
Exposure
Drugs

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5
Q

exposure causes

A

asbestos, silica, bat/bird droppings, radiation, hot tubs

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6
Q

drug causes

A
amiodarone
propranolol
nitrofurantoin
methotrexate
rituximab
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7
Q

autoimmune causes

A

dermatomyositis,RA, scleroderma, sarcoidosis, sjogrens

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8
Q

Interstitial lung disease is ______ so the treatment goal is to ______

A

IRREVERSIBLE

prevent progression/alleviate sx

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9
Q

what symptoms are uncommon in Interstitial lung disease

A

wheezing, chest pain

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10
Q

main sx of Interstitial lung disease

A

Progressive dyspnea on exertion

Persistent non-productive cough

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11
Q

extra-pulmonary symptoms may indicate

A

connective tissue disease→ musculoskeletal pain, weakness, joint pain/swelling, fevers, dry eyes/mouth

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12
Q

heard on lung exam of a patient with Interstitial lung disease

A

velcro crackles at bases

inspiratory squeaks

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13
Q

late stage Interstitial lung disease signs

A
cyanosis, digital clubbing
cor pulmonale (middle/late)
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14
Q

Extrapulmonary manifestations: erythema nodosum

A

Sarcoidosis

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15
Q

Extrapulmonary manifestations: gottron’s papules

A

Dermatomyositis

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16
Q

highest yield for noninvasive test to diagnose Interstitial lung disease

A

High resolution chest CT (HRCT)

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17
Q

Gold standard to diagnose Interstitial lung disease

A

Tissue biopsy

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18
Q

findings on CXR that could indicate Interstitial lung disease

A

Ground glass appearance early, nonspecific
Reticular “netlike” (most common), nodular, or mixed pattern of opacities
Honeycombing: small cystic spaces: poor prognosis

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19
Q

reticular opacities are

A

small irregular opacities

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20
Q

Micronodular pattern is

A

small rounded opacities

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21
Q

HRCT: reticular opacities

A

IPF

Asbestosis

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22
Q

HRCT: ground glass attenuation

A

Drug toxicity

Respiratory bronchiolitis

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23
Q

HRCT: upper/central lobes

A

Sarcoidosis

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24
Q

Serologic studies to rule out subclinical autoimmune disease

A

ANA, RF

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25
Q

when to evaluate for vasculitis and how

A

pulmonary hemorrhage or suspicious systemic symptoms

Antineutrophil cytoplasmic antibodies (ANCA)

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26
Q

signs of restrictive disease on PFTs

A

Decreased Total lung capacity
Decreased FEV1, FVC
Normal/increased FEV1/FVC

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27
Q

PFTs obstructive disease

A

Increased TLCC
Normal FVC
Decreased FEV1
Decreased FEV1/FVC ratio

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28
Q

If PFTs indicate restrictive disease, obtain

A

DLCO: low=ILD

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29
Q

ILD with obstructive pattern

A

sarcoidosis

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30
Q

Extension of bronchoscopy that allows sampling→ cell counts, culture (atypical/typical), cytology (identify maligancy)

A

Bronchoalveolar lavage

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31
Q

Is bronchoalveolar lavage typically performed with HRCT findings consistent with IPF

A

no

32
Q

gold standard for diagnosis

A

lung biopsy

33
Q

contraindication for lung biopsy

A

honeycombing

34
Q

Transbronchial biopsy is a broncoscope + biopsy used to assess ____

A

central locations

NOT periphery

35
Q

2 types of surgical lung biopsies

A
  1. Video assisted thoracoscopic surgery: 2 incisions in lateral chest wall
  2. Thoracotomy: 5-6 cm incision
36
Q

Bronchoscope used to evaluated hilar and mediastinal lymph nodes
useful if sarcoid suspected

A

Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA)

37
Q

2 biopsy methods that can be done in conjunction if a different scope is passed

A

Endobronchial ultrasound guided transbronchial needle aspiration (EBUS-TBNA)
-and-
Transbronchial lung biopsy

38
Q

UIP=“Usual interstitial pneumonia”

A

Idiopathic pulmonary fibrosis

Abestosis

39
Q

most common interstitial lung disease

A

Idiopathic pulmonary fibrosis

40
Q

what is HRCT used for in idiopathic pulmonary fibrosis

A

Determine if: probable/indeterminate UIP pattern, alternative diagnosis→ Bronchoalveolar lavage/lung biopsy

41
Q

treatment for idiopathic pulmonary fibrosis

A

Treatment for GERD

Nintedanib: tyrosine kinase inhibitor

42
Q

Nintedanib is a _____ and works by

A

tyrosine kinase inhibitor

downregulation of production of growth factors and procollagens

43
Q

sarcoidosis extrapulmonary findings

A

erthema nodosum
lupus pernio
granulomatous uveitis
arthralgias

44
Q

Non-caseating granulomas

A

sarcoidosis
→ secrete 1,25 dihydroxyvitamin D→ serum Ca++ elevated
→ secrete ACE

45
Q

labs of sarcoidosis

A

Elevated serum ACE +/- elevated serum calcium, hypercalciuria
Elevated alkaline phosphatase

46
Q

lupus pernio

A

idk some weird nose nodule

sarcoidosis

47
Q

CXR staging of sarcoidosis

A
  1. Normal
  2. Hilar adenopathy
  3. Hilar adenopathy + diffuse infiltrates
  4. Only diffuses parenchymal infiltrates
  5. Pulmonary fibrosis
48
Q

when to treat sarcoidosis

A

stage 2 and symptomatic

49
Q

Sarcoidosis tx

A

high dose corticosteroids + PJP prophylaxis
methotrexate (alternative)
chloroquine/hydroxychloroquine: cutaneous lesions, neuro sx, hypercalcemia
topical corticosteroids for ocular disease
lung transplant: stage IV

50
Q

most common CXR pattern of sarcoidosis LAD

A

bilateral symmetrical hilar and right paratracheal mediastinal adenopathy

51
Q

inhalation and deposition of mineral dust

A

pneumoconiosis: asbestosis, silicosis, coal worker’s

52
Q

Chronic simple silicosis

A

10-12 years exposure
+/- asymptomatic
Nonprogressive once exposure eliminated
Hilar node calcification: eggshell pattern

53
Q

Chronic complicated silicosis

A

> 20 years exposure
Progression even after exposure eliminated
Tachypnea, prolonged expiration, rhonchi, wheezing, rales
Advanced disease: cyanosis, cor pulmonale

54
Q

silicosis classic CXR finding

A

eggshell calcifications

55
Q

asbestosis presentation

A

15-20 years after exposure, dose dependent
40-75 year old males
Smoking increases risk of bronchogenic cancer

Presentation: Insidious onset, dyspnea, reduced exercise tolerance, chest discomfort, end inspiratory rales
Digital clubbing which does not correlate with severity

56
Q

asbestosis imaging

A

HRCT: reticular opacities
Restrictive pattern
CXR: opacities in lower lungs, thickened pleura, pleural plaques

Open-lung biopsy not required but is definitive diagnosis: fibrosis, asbestos bodies through light microscopy

57
Q

reliable indicator of asbestosis on CXR, not always present

A

calcified pleural plaques in diaphragmatic pleura of the 6th-9th ribs

58
Q

asbestosis treatment

A

NO immunotherapy drugs or steroids alter disease course

basically prevent exposure and treat symptoms

59
Q

asbestosis has a high risk of

A

Cancer
risk increased by smoking→ bronchogenic carcinoma
→ cancers of upper respiratory tract, esophagus, biliary system, kidneys

60
Q

Mesothelioma

A

form of cancer almost always associated with asbestos exposure, even short term (1-2) years
Cancer develops in mesothelium: protective lining covering organs: pleura (most common), peritoneum, pericardium
Not caused by smoking, poor prognosis

61
Q

granulomatosis with polyangiitis aka

A

“ANCA-associated vasculitis”

62
Q

granulomatosis with polyangiitis

A

Multisystem autoimmune disease
Systemic vasculitis of small/medium vessels
Necrotizing granulomas of upper and lower respiratory tracts
RARE, 35-55 year old northern europeans
Relapse common

63
Q

granulomatosis with polyangiitis presentation

A

recurrent respiratory infections, constitutional symptoms (fever, weight loss, night sweats, anorexia, fatigue/lethargy)
Ocular: conjunctivitis, episcleritis, uveitis, RAO
ENT: chronic sinusitis, rhinitis, epistaxis, saddle nose deformity
Pulmonary: infiltrates, cough, hemoptysis, dyspnea, stridor
Renal: failure, erythrocyte casts
Peripheral nervous system: cranial nerve palsies, sensorimotor polyneuropathy
Skin: palpable purpura, skin ulcers, necrotic, blistering purpura
Cardiac: pericarditis, coronary vasculitis
Polyarticular arthralgias

64
Q

specific granulomatosis with polyangiitis findings that distinguish it

A

saddle nose deformity
infiltrates of CXR
a million systemic symptoms of vasculitis
(+) C-ANCA
CT: stellate shaped peripheral pulmonary arteries= “vasculitis sign”

65
Q

granulomatosis with polyangiitis lab findings

A

(+) C-ANCA +/- RF, ANA

Elevated ESR/CRP
Normocytic anemia
Thrombocytosis 
Leukocytosis
Increased BUN/Cr
UA: proteinuria, RBC cast
66
Q

granulomatosis with polyangiitis treatment

A

Cyclophosphamide: immunosuppressant which improves prognosis
Toxicities: CHF, thrombocytopenia, acute hemorrhagic cystitis, acute tubular necrosis, N/V/D
Corticosteroids

67
Q

Hypersensitivity Pneumonitis

A

Repetitive inhalation of antigens in a susceptible host → inflammation
Antigens: bacteria, fungi, mold, proteins, chemicals, environments: bird/bat droppings, animal fur/feathers, hot tubs, hardwood dusts
4th-6th decade

68
Q

Hypersensitivity Pneumonitis acute disease

A

remits without treatment

69
Q

subtypes of Hypersensitivity Pneumonitis

A

Acute: flu-like syndrome within hours of exposure
Remits without therapy
Sub-acute: insidious onset of cough, dyspnea, fatigue over weeks
Chronic: progressive dyspnea, cough, fatigue, malaise

70
Q

Hypersensitivity Pneumonitis symptoms

A

Diffuse, fine bibasilar crackles, fever, tachypnea, muscle wasting, clubbing, weight loss

71
Q

Hypersensitivity Pneumonitis imaging

A

Interstitial inflammation
Honeycombing
Centrilobular fibrosis
Peribronchiolar fibrosis

72
Q

Hypersensitivity Pneumonitis tx

A

Avoid antigen
Corticosteroids: speed recovery, chronic
adjunctive therapy: Bronchodilators, antihistamines, inhaled corticosteroids

73
Q

Pulmonary hypertension/Cor pulmonale/CV disease

A

complication of interstitial lung disease

Lungs stiffen→ pulmonary arteries become dilated→ right ventricular dysfunction and enlargement

74
Q

complications of interstitial lung disease

A
Pulmonary hypertension/Cor pulmonale/CV disease
Pneumothorax
PE
Pulmonary infection
Elevated cancer risk
Progressive respiratory insufficiency
75
Q

Granulomatosis with polyangitis imaging

A

CXR: variable, nodules which may cavitate
CT chest:
→ stellate shaped peripheral pulmonary arteries= “vasculitis sign”
→ Feeding vessels to nodules/cavities
→ Diffuse alveolar hemorrhage
Tissue biopsy: histopathologic evidence of vasculitis, granulomatous inflammation
Labs: