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General Thoracic Surgery > 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)

21
Q

MC affected patient population with DIP

A

Smokers aged 40-50 years old

22
Q

Characteristic imaging appearance of DIP

A

Bilateral ground glass opacities in a linear pattern

23
Q

TOC for DIP

A

Steroids (more amenable to therapy than UIP/IPF)

May progress and require lung transplantation

24
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)

25
Q

Compared to UIP/IPF, NIP has (improved or worse) response rate to steroid therapy and (better or worse) overal survival?

A

Improved response rate

Better overall survival

26
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)

27
Q

MC affected population with BOOP/COP

A

Men and Women equally

Age 40-60 years

28
Q

Imaging appearance/characteristics of BOOP/COP

A

Airspace disease with bilateral diffuse ground-glass opacities

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

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)

General Thoracic Surgery (36 decks)

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