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General Thoracic Surgery Notecards > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

Definition of idiopathic interstitial pneumonia

A

Spectrum of pulmonary disorders with characteristic alveolitic change secondary to infilration of immune cells into pulmonary interstitium

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2
Q

Alveolitic pattern of interstitial lung disease characterized by activated macrophages, T-cells, and inflammatory cytokines that activate B-cells to secrete antibodies

A

Idiopathic interstitial pneumonia

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3
Q

MOA of idiopatich interstitial pneumonia

A

antibody/antigen complex deposition in lung parenchyma > activation of complement system > inflammatory reaction with parenchymal tissue injury

further destruction by neutrophils, macrophages (TNF, IL-1), prostaglandins, and cytokines

ultimatley, inflammation and injury may result in irreversible fibrosis

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4
Q

Sx of idiopathic interstitial pneumonia

A

progressive dyspnea and non-productive cough (over months - years)

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5
Q

Diagnostic w/u for idiopathic interstitial pneumonia

A

H&P

ABG

PFTs

Labs

Bronchoscopy

High-resolution CT

Biopsy (usually surgical, occasional transbronchial)

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6
Q

Success of transbronchial biopsy for diagnosis in idiopathic interstitial pneumonia

A

25%

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7
Q

Treatment plan for idiopathic interstitial pneumonia

A
  • Oxygen supplementation
  • Removal of causitive agents
  • Early intervention to prevent progression of fibrosis
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8
Q

Cornerstone of therapy for interstitial lung disease

A

Steroids

  • 20% of IPF patients will respond to steroids
  • Alternative therapies include:
    • cyclophosphamide
    • azathioprine
    • methotrexate
    • penicillamine
    • cochicine
    • cyclosporine
    • INF-gamma
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9
Q

Treatment of interstitial lung disease after failure of medical managment

A

Lung transplantation

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10
Q

SOC for intersitial lung disease (IPF)

A

Single lung transplant

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11
Q

Criteria for transplant

A
  • Progressive dyspnea/hypoxia despite maximal medical therapy
  • VC <= 60 - 70% predicted
  • DLCO <= 50 - 60% predicted
  • Age < 60 yrs (double lung transplant) or <65 yrs (single lung transplant)
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12
Q

5- year survival after transplant for IPF

A

]50-60%

(28-month median survival for IPF with medical treatment alone)

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13
Q

Defined by thickened, fibrotic alveolar interstitium with lymphocytic and plasmacytic infiltration

A

Usual interstial pneumonia (UIP) / Idiopathic pulmonary fibrosis (IPF)

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14
Q

UIP / IPF demographics

A

males

age > 50

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15
Q

Etiologies of UIP / IPF

A

Etiology largely unknown

  • Infectious
    • hepatitis C
    • EBV
  • Environmental
    • heavy metal dusts
    • solvents
    • cigarette smoking
  • Genetic
    • HLA-B15, B8, B12
  • Immunologic
    • Rheumatoid arthritis
    • SLE
    • Systemic sclerosis
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16
Q

Most common CT findings with UIP / IPF

A
  • Mediastinal lymphadenopathy
  • Lower lobe/subpleural predominance
    • Reticurlar or reticulnodular pattern
    • Honeycombing cysts
    • Ground glass opacities
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17
Q

Diagnosis and Treatmetn of UIP/IPF

A
  • Diagnosis:
    • surgical biopsy
  • Treatment:
    • High dose steroids
    • Transplant
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18
Q

Favorable prognostic factors for UIP / IPF

A
  • young age
  • disease < 1 year
  • active inflammation
  • lymphocytosis on BAL
  • presence of immune complexes
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19
Q

5 - year survival for UIP / IPF

A
  • Responders (43%)
  • Non-responders (20%)
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20
Q

ILD subtype characterized by mildly thickened intersitium, spare infiltration of inlfammatory cells, mild fibrosis and filling of alveoli with macrophages

A

Desquamative interstitial pneumonia (DIP)

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21
Q

MC affected patient population with DIP

A

Smokers aged 40-50 years old

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22
Q

ILD subtype characterized by mildly thickened interstitium, infiltration of inflammatory cells, and mild fibrosis in patchy distribution with uniform histology

A

Nonspecific interstitial pneumonia (NIP)

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23
Q

Characteristic imaging appearance of Desquamative Interstitial Pneumonitis?

A

Bilateral ground glass opacities in a linear pattern

The radiologic patterns are nonspecific and include patchy ground-glass opacities with a lower lung and peripheral predominance.

24
Q

Treatment of Choice for Desquamative Interstitial Pneumonitis?

A

Steroids (more amenable to therapy than UIP/IPF)

May progress and require lung transplantation

25
Q

Compared to UIP/IPF; Nonspecific Interstitial Pneumonia/Fibrosis (NIP) has (improved or worse) response rate to steroid therapy and (better or worse) overall survival?

A

Improved response rate

Better overall survival

26
Q

MC affected population with BOOP/COP

A

Men and Women equally

Age 40-60 years

27
Q

ILD subtype characterized by chronic inflammation of alveoli, granulation tissue in bronchioles/alveoli, and accumulation of macrophages within alveoli with patchy distribution and uniform histology

A

Brochiolitis obliterans organizing pneumonia (BOOP) / cryptogenic organizing pneumonia (COP)

28
Q

Imaging appearance/characteristics of BOOP/COP

A

Airspace disease with bilateral diffuse ground-glass opacities

CT findings are far more extensive than expected from a review of the plain chest radiograph. The lung abnormalities show a characteristic peripheral or peribronchial distribution, and the lower lung lobes are more frequently involved

29
Q

TOC for BOOP/COP

A

Steroids (rapid resolution of disease process typical)

Surgical role: biopsy for diagnosis

30
Q

Outcomes for BOOP/COP

A

Mortality ~ 12%

Relapses after steroid therapy common (respond favorably to addnl steroid rx)

31
Q

ILD subtype characterised by infilration of lymphocytes and plasma cells in lung parenchyma without alveolar damage

A

Lymphocytic insterstial pneumonia (LIP)

Often associated with:

  • Immune disorders:
    • Sjogren’s syndrome
    • SLE
    • Myasthenia gravis
    • Chronic active hepatitis
32
Q

MC affected patient populations with LIP

A

Children (50% of HIV+ children with pulmonary disease)

Immunocompromised

Women (40-80 yo)

33
Q

Imaging appearance/characteristics of LIP

A

Reticular/reticulonodular pattern and airspace consolidation

High-resolution CT is the radiologic procedure of choice and shows bilateral abnormalities that are diffuse or have a lower lung predominance. The dominant high-resolution CT feature in patients with LIP is ground-glass attenuation, which is related to the histologic evidence of diffuse interstitial inflammation (,Fig 25,,) (,63). Another frequent finding is thin-walled perivascular cysts

34
Q

TOC and Outcomes for LIP

A

Treatment: steroids (surgery for diagnostic bx)

33-50% mortality within 5 years of diagnosis

5% progress to lymphoma

35
Q

Chronic systemic disorder of unknown etiology characterized by noncaseating granulomas within multiple organ systems

A

Sarcoidosis

36
Q

MC affected patient population with sarcoidosis

A

Females

African ancestery

Age: 20-40 years

37
Q

Causal theories for sarcoidosis

A
  • Enviornmental
    • Workplace clusters
  • Infectious
    • lymphadenopathy
    • M. tuberculosis
  • Hereditary
    • HLA-A1 and -B1
  • Immunologic
    • altered T cell ratios
    • impaired systemic immunity
    • hyperactive B-cell lines
    • altered macrophage productin of INF-G and RANTES
38
Q

Sarcoidosis involves lungs in _% of cases

A

~ 94%

39
Q

MC extrapleural sites invovled in sarcoidosis

A
  • Eyes
    • uveitis
    • conjunctivitis
    • retinitis
  • Skin
    • nodules
    • plaques
40
Q

Sarcoidosis occasionally associated with what other conditions

A
  • Rheumatoid arthritis
  • SLE
  • Progressive systemic sclerosis
  • Lofgren’s syndrome
41
Q

Imaging appearance/characteristics of sarcoidosis

A

Lymphadenopathy

Upper lobe predominance

Variable interstitial/aciner lesions

Occasional nodules

42
Q

Serum and/or BAL fluid marker elevated in sarcoidosis

A

ACE (angiotensin coverting enzyme)

43
Q

Clinical presentation of sarcoidosis

A

Asymptomatic (30-50%)

  • Hilar or mediastinal adenopathy (80%)
  • Constitutional symptoms (fever, chills, malaise, weight loss)
  • Elevated ACE levels (serum and BAL fluid)
  • Restrictive lung function (occasionally obstructive)
  • Hypercalcemia
  • Peripheral lymphopenia
44
Q

Radiographic staging of sarcoidosis

A
  • Stage 0 (8%): normal
  • Stage I (50%): lymphadenopathy
  • Stage II (30%): lymphadenopathy and parenchymal infiltrates
  • Stage III (12%): parenchymal infiltrates only
  • Stage IV (rare): end-stage honey-comb lung
45
Q

Dx workup for sarcoidosis

A
  • PFTs
  • Diagnostic bronchoscopy (BAL and transbronchial bx)
  • High resoluation CT scan
  • Medistinoscopy or VATS bx (non-caseating granulomas, elevated tissue ACE)
46
Q

Natural history of pulmonary sarcoidosis

A

Most remain stable

20% suffer progressive pulmonary deterioration

47
Q

TOC for sarcoidosis

A

Steroids

Alternative rx: metotrexate, cyclosporine, chlorambucil

48
Q

Therapeutic recommendatons for sarcoidosis

A

High remission rate:

Asymptomatic stage I or II with normal PFTS (expectant managment)

Symptomatic stage II or any stage III (treatment)

Extrapulmonary manifestations (treatment)

49
Q

Mortatliy rate for sarcoidosis

A

4%

(most deaths attributable to pulmonary disease)

50
Q
A

Distribution (a), CT image (b), and CT pattern (c) of UIP. The distribution is subpleural with an apicobasal gradient (red area in a). CT shows honeycombing (green areas in c), reticular opacities (blue areas in c), traction bronchiectasis (red area in c), and focal ground-glass opacity (gray area in c).

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General Thoracic Surgery Notecards (38 decks)

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