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Respiratory 4 > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

What is Interstitial Lung disease?

A
  • Large diverse group of disorders (over 200)
  • Usually from injury to the alveolus and surrounding lung parenchyma
  • Diseases with many similarities in clinical presentation, radiographic appearance, and physiologic features
  • A major national health problem
  • Estimated prevalence – 81/100,000 for males and 67/100,000 for females
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2
Q

What is Idiopathic Pulmonary Fibrosis?

A

• A subset: Idiopathic Pulmonary Fibrosis (IPF)

– Accounts for about 28% of all ILD’s

– 14 to 43 people per 100,000

– 7 to 16 new cases per 100,000 yearly

– 15,000 to 40,000 deaths/year in the U.S.

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3
Q

What are the major categories of Interstitial lung disease?

A
  • Idiopathic Interstitial Pneumonias
  • Connective tissue disease/collagen vascular disorders
  • Occupational and Environmental exposures – Organic - Inorganic
  • Granulomatosis diseases
  • Treatment related – Drug Rx - Radiation pneumonitis - Immunosuppression
  • Infection
  • Interstitial pneumonia with autoimmune features
  • Primary (unclassified) ILD – Immunosuppression/transplant
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4
Q

ILD leads to what?

A
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5
Q

Idiopathic interstitial pneumonia’s are what?

A
  • Idiopathic pulmonary fibrosis – Most common ILD – A diagnosis of exclusion
  • Other idiopathic interstital pneumonias

– Non-specific interstitial pneumonia (NSIP)

– Desquamative interstitial pneumonia (DIP)

– Acute interstitial pneumonia/Hamman–Rich syndrome (AIP)

– Respiratory bronchiolitis interstitial lung disease (RBILD)

– Cryptogenic organizing pneumonia/BOOP (COP)

– Lymphocytic interstitial pneumonia (LIP)

– Idiopathic pleuroparenchymal fibroelastosis

– Interstitial pneumonia with autoimmune features

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6
Q

What are the pathologic components of ILD?

A
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7
Q

What are the stages of idiopathic pulmonary fibrosis?

A
  • Acute injury
  • Propagation
  • Chronic fibrosis
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8
Q

explain the pathogenesis of Idiopathic Pulmonary Fibrosis?

A
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9
Q

What is Diffuse paraenchymal lung disease?

A

–A group of disorders characterized by distinct cellular infiltrates and extracellular matrix deposition within the regions of the lung distal to the terminal bronchiole, i.e., the acinus

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10
Q

Provide an overview of idiopathic pulmonary fibrosis?

A
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11
Q

Explain the clinical approach to the ILD patient?

A
  • Clinical history
  • Clinical examination
  • Diagnostic procedures – Blood tests, serologies, etc. – Pulmonary functions test – Chest radiograph – High-resolution computed tomography (HRCT) – Bronchoscopic procedures as indicated
  • Endobronchial and transbronchial biopsies
  • Bronchoalveolar lavage (BAL) – Surgical biopsy video-assisted thorascopic surgery (VATS) if needed to establish a diagnosis
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12
Q

Explain common changes with interstitial lung disease?

A
  • increased Work of breathing
  • decreased FVC
  • decreased Lung volumes
  • decreased Diffusion capacity
  • decreased PO2
  • Abnormal CXR/HRCT
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13
Q

Explain Idiopathic Pulmonary Fibrosis?

A

Idiopathic Pulmonary Fibrosis (IPF) is a restrictive lung disease. It is a specific form of Idiopathic Interstitial Pneumonias (IIP’s). IPF is a chronic, progressive irreversible, fibrosing interstitial lung disease that occurs in adults. Its causes are unknown and its progression unpredictable, but fatal. To date, there is no known cure other than lung transplantation.

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14
Q

What are some risk factors for Interstitial Pulmonary Fibrosis?

A
  • Smoking history especially ≥ 20 packs/year
  • Gastroesophageal reflux disease (GERD) a very common finding
  • Male predominance
  • Age: > 50 years; median age 65-70 years
  • Infection
  • Genetic factors (familial IPF)
  • Environmental exposures
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15
Q

Symptoms of Idiopathic Pulmonary Fibrosis?

A
  • Dyspnea on exertion (progressive)
  • Dry cough
  • Bibasilar crackles (sounds like velcro being pulled apart)
  • Finger clubbing: Occurs in 25-50% of patients
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16
Q

What are some comorbidities associated with Idiopathic Pulmonary fibrosis?

A
  • GERD
  • COPD
  • Pulmonary hypertension
  • Lung cancer
  • Obstructive sleep apnea
  • Venous thromboembolism
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17
Q

What aspects of the history in idiopathic pulmonary fibrosis are important?

A
  • Past medical history
  • Medication/drug history
  • Occupational and environmental history
  • Smoking history
  • Family history
  • Pets
  • Hobbies
  • Travel
18
Q

usual symptoms and presentation of Interstitial lung disease? what symptoms are absent?

A
  • Dyspnea on exertion – Chronic-slowly progressive
  • Cough – Paroxysmal, dry
  • Bibasilar crackles (sounds like velcro being pulled apart)
  • Hemoptysis – Rare
  • Absent – Wheeze – Episodic chest tightness – Respiratory allergies
19
Q

What are some exceptions to the usual symptoms seen in ILD?

A
  • Acute onset of dyspnea, fever, malaise, headache and fatigue – Hypersensitivity pneumonias – Some drug-induced ILD’s – Infection in immunocompromised host – Toxic gas inhalation
  • Pulmonary involvement in collagen vascular disease – Scleroderma – Rheumatoid arthritis – Dermatomyositis
20
Q

Physical exam findings of ILD?

A
  • Tachypnea
  • Inspiratory crackles/rales
  • “Clubbed” fingers occurs in 25-50% of IPF cases
  • Cyanosis (late)
  • Extrapulmonary – Adenopathy – Arthritis – Systemic sclerosis – Rash
  • Signs of severe PHT (pulmonary hypertension) – Elevated JVD – Leg edema (late)
21
Q

What radiographic studies?

A
  • Chest x-ray
  • High resolution computed tomography (HRCT)
22
Q

Radiographic findings seen in CXR with ILD?

A

Chest x-ray is the initial study When looking at the chest x-ray assess:

• Location of abnormalities:

Upper central

Lower diffuse peripheral

• Pattern Alveolar linear Reticular reticular nodular Honeycombing

23
Q

High res CT is used how in suspected ILD?

A
  • Standard diagnostic study for all patients suspected to have ILD
  • Technique extremely important when doing the study – Thin slices – Prone and supine – Inspiratory and expiration
24
Q

Patterns of Opactities distribution in ILD?

A
  • Upper – Sarcoidosis, silicosis, pneumoconisosis, langerhans, hypersensitive pneumonitis, reactivation TB, PCP
  • Lower – IPF, collagen vascular disease, asbestosis, chronic aspiration
  • Peripheral – IPF, CEP, BOOP/COP
  • Central – Sarcoid, berylliosis, PCP
25
Q

Role of Pulmonary function tests in ILD?

A
  • Obtain baseline physiologic function
  • Confirm suspected diagnosis
  • Evaluate physiologic impairment
  • Assess course of disease
  • Assess effect of therapy
26
Q

Assessment of Pulmonary function in ILD?

A
  • Complete PFT – Spirometry (decrease FVC) – Lung volumes (decrease TLC, decrease RV) – DLCO decrease
  • Pulse oximetry
  • ABG (baseline)
  • 6 minute walk test (6MWT)
  • Cardiopulmonary exercise test
27
Q

Explain the changes seen in ABG’s with obstructive and interstitial diseases?

A
28
Q

Effect of exercise on ABG’s in interstitial lung disease?

A
29
Q

Explain the hypoxemia in shunts?

A
  • Primarily due to abnormal matching of ventilation/perfusion
  • “Diffusion Block” due to RBC’s rapid transit through pulmonary capillaries. However, the loss of the capillary bed is a more important cause
  • Rarely from shunts
30
Q

Lab studies in ILD?

A

CBC

Autoimmune serologies

ESR

ANA titer pattern,

ds DNA

ABG

RA factor

SSA SSB

CK

Serum ACE

Aldolase

Jo1 antibodies

Precipitins to organic antigens

31
Q

In granulomatous ILD what labs will be elevated?

A

ESR ANCA ACE

32
Q

Connective tissue diseases will show what elevated labs in ILD?

A

ANA RF, anti-CCP Scl-70 antibody Jo-1 antibody

33
Q

Hypersensitivity Pneumonia will show what with ILD?

A

Hypersensitivity panel (follow up with exposure history)

34
Q

IPF shows what on labs?

A

non-specific

35
Q

Characteristics of sarcoids, Eosinophilic granulomatosis, Rheumatoid lung disease, SLE, Polymyositis show what on labs?

A
36
Q

invasive studies for ILD?

A
  • Bronchoscopy – Endo/transbronchial biopsy – Bronchoalveolar lavage • Not commonly done not that helpful
  • Video-assisted thorascopic biopsy (VATS)
37
Q

Treatment of ILD?

A

Determined by etiology

Supplemental oxygen as indicated

Pulmonary rehab

Smoking cessation

Pharmacologic interventions

Lung transplant

38
Q

Pharmocologic agents in ILD?

A
  • Many agents studied – usually dismal results
  • Two new agents that slow progression

– Esbriet (Pirfenidone) • MOA: not fully elucidated • Felt to ↓ fibroblast proliferation production and cytokines

– OFEV (Nintedanib) • MOA: a tryosine kinase inhibitor • Shown to inhibit pathways involved in fibrosis

• Unfortunately, neither agent halts disease progression MOA: Mode of Action

39
Q

Human pentraxin for ILD?

A

Recombinant Human Pentraxin a possible new treatment for Idiopathic Pulmonary Fibrosis

Currently a phase 2 trial shows promise

Epithelial injury causing abnormal wound repair contributes to the pathogenesis of IPF

Purified serum amyloid P (Pentraxin 2) inhibits monocyte differentiation into profibrotic fibrocytes

Plasma Pentraxin 2 concentrations are reduced in patients with IPF and correlate with disease severity

The hope is that by replacing Pentraxin 2 with Recombinant Human Pentraxin it will result in decreased lung fibrosis

40
Q

What factors at the time of diagnosis of ILD determine prognosis?

A

Clinical • Dyspnea

Physiologic • DLCO • 6MWT desaturation • A-a gradient • Pulmonary hypertension • VO2MAX

Radiologic • HRCT pattern (honeycombing) • Extent of fibrosis

Emerging Markers

41
Q

What indicators for follow up determine the prognosis for ILD?

A

Physiologic • Forced vital capacity • DLCO • 6MWT desaturation

Clinical: dyspnea

42
Q

Prognosis of ILD?

A
  • Determined by etiology
  • Idiopathic pulmonary fibrosis – Very poor most patients dead 2 ½ to 5 years after diagnosis

– 5-year survival rate between 20% and 40%

– 3 to 9 cases per 100,000 persons/year

– Factors associated with increased survival • Younger age • Female gender • Less functional impairment • Cigarette smoking at time of diagnosis (unexplained)

Respiratory 4 (18 decks)

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