Interstitial Lung Disease

Question 1

Definition of idiopathic interstitial pneumonia

Answer 1

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

Question 2

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

Answer 2

Idiopathic interstitial pneumonia

Question 3

MOA of idiopatich interstitial pneumonia

Answer 3

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

Question 4

Sx of idiopathic interstitial pneumonia

Answer 4

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

Question 5

Diagnostic w/u for idiopathic interstitial pneumonia

Answer 5

H&P

ABG

PFTs

Labs

Bronchoscopy

High-resolution CT

Biopsy (usually surgical, occasional transbronchial)

Question 6

Success of transbronchial biopsy for diagnosis in idiopathic interstitial pneumonia

Answer 6

25%

Question 7

Treatment plan for idiopathic interstitial pneumonia

Answer 7

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

Question 8

Cornerstone of therapy for interstitial lung disease

Answer 8

Steroids

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

Question 9

Treatment of interstitial lung disease after failure of medical managment

Answer 9

Lung transplantation

Question 10

SOC for intersitial lung disease (IPF)

Answer 10

Single lung transplant

Question 11

Criteria for transplant

Answer 11

• 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)

Question 12

5- year survival after transplant for IPF

Answer 12

]50-60%

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

Question 13

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

Answer 13

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

Question 14

UIP / IPF demographics

Answer 14

males

age > 50

Question 15

Etiologies of UIP / IPF

Answer 15

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

Question 16

Most common CT findings with UIP / IPF

Answer 16

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

Question 17

Diagnosis and Treatmetn of UIP/IPF

Answer 17

• Diagnosis:
  
  • surgical biopsy
• Treatment:
  
  • High dose steroids
  • Transplant

Question 18

Favorable prognostic factors for UIP / IPF

Answer 18

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

Question 19

5 - year survival for UIP / IPF

Answer 19

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

Question 20

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

Answer 20

Desquamative interstitial pneumonia (DIP)

Question 21

MC affected patient population with DIP

Answer 21

Smokers aged 40-50 years old

Question 22

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

Answer 22

Nonspecific interstitial pneumonia (NIP)

Question 23

Characteristic imaging appearance of Desquamative Interstitial Pneumonitis?

Answer 23

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.

Question 24

Treatment of Choice for Desquamative Interstitial Pneumonitis?

Answer 24

Steroids (more amenable to therapy than UIP/IPF)

May progress and require lung transplantation

Question 25

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

Answer 25

Improved response rate Better overall survival

Question 26

MC affected population with BOOP/COP

Answer 26

Men and Women equally Age 40-60 years

Question 27

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

Answer 27

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

Question 28

Imaging appearance/characteristics of BOOP/COP

Answer 28

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

Question 29

TOC for BOOP/COP

Answer 29

Steroids (rapid resolution of disease process typical) Surgical role: biopsy for diagnosis

Question 30

Outcomes for BOOP/COP

Answer 30

Mortality ~ 12% Relapses after steroid therapy common (respond favorably to addnl steroid rx)

Question 31

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

Answer 31

Lymphocytic insterstial pneumonia (LIP) Often associated with: * Immune disorders: * Sjogren's syndrome * SLE * Myasthenia gravis * Chronic active hepatitis

Question 32

MC affected patient populations with LIP

Answer 32

Children (50% of HIV+ children with pulmonary disease) Immunocompromised Women (40-80 yo)

Question 33

Imaging appearance/characteristics of LIP

Answer 33

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

Question 34

TOC and Outcomes for LIP

Answer 34

Treatment: steroids (surgery for diagnostic bx) 33-50% mortality within 5 years of diagnosis 5% progress to lymphoma

Question 35

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

Answer 35

Sarcoidosis

Question 36

MC affected patient population with sarcoidosis

Answer 36

Females African ancestery Age: 20-40 years

Question 37

Causal theories for sarcoidosis

Answer 37

* 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

Question 38

Sarcoidosis involves lungs in \_% of cases

Answer 38

~ 94%

Question 39

MC extrapleural sites invovled in sarcoidosis

Answer 39

* Eyes * uveitis * conjunctivitis * retinitis * Skin * nodules * plaques

Question 40

Sarcoidosis occasionally associated with what other conditions

Answer 40

* Rheumatoid arthritis * SLE * Progressive systemic sclerosis * Lofgren's syndrome

Question 41

Imaging appearance/characteristics of sarcoidosis

Answer 41

Lymphadenopathy Upper lobe predominance Variable interstitial/aciner lesions Occasional nodules

Question 42

Serum and/or BAL fluid marker elevated in sarcoidosis

Answer 42

ACE (angiotensin coverting enzyme)

Question 43

Clinical presentation of sarcoidosis

Answer 43

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

Question 44

Radiographic staging of sarcoidosis

Answer 44

* 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

Question 45

Dx workup for sarcoidosis

Answer 45

* PFTs * Diagnostic bronchoscopy (BAL and transbronchial bx) * High resoluation CT scan * Medistinoscopy or VATS bx (_non-caseating granulomas, elevated tissue ACE_)

Question 46

Natural history of pulmonary sarcoidosis

Answer 46

Most remain stable 20% suffer progressive pulmonary deterioration

Question 47

TOC for sarcoidosis

Answer 47

Steroids Alternative rx: metotrexate, cyclosporine, chlorambucil

Question 48

Therapeutic recommendatons for sarcoidosis

Answer 48

High remission rate: Asymptomatic stage I or II with normal PFTS (expectant managment) Symptomatic stage II or any stage III (treatment) Extrapulmonary manifestations (treatment)

Question 49

Mortatliy rate for sarcoidosis

Answer 49

4% (most deaths attributable to pulmonary disease)

Question 50

Answer 50

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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Question 55

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