Interstitial Lung Disease.ppt - Approach To Interstitial Lung

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					       Approach To
 Interstitial Lung Diseases
               or
Diffuse Parenchymal Lung
          Diseases

         Jihane A. Faress M.D
     Assistant Professor of Medicine
Pulmonary, Critical Care and Sleep Division
           October 13th, 2008
Objectives

• Review the spectrum of ILD or DPLD
• Identify clues on presentation to make
  the diagnosis
• Review common radiographic findings
  in ILD
• Role of BAL,TBBX and OLB in the
  diagnosis of ILD
• Come up with an algorithm to make
  the diagnosis
Clinical Presentation

• Dyspnea on exertion or a persistent non
  productive cough
• Abnormal CXR
• Pulmonary symptoms associated with
  another disease, such as CVD
• PFT abnormalities
    What is the Pulmonary
    Interstitium?

• Interstitial compartment is
  the portion of the lung
  sandwiched between the
  epithelial and endothelial
  basement membrane


• Expansion of the interstitial
  compartment by
  inflammation with or without
  fibrosis
    – Necrosis
    – Hyperplasia
    – Collapse of basement
      membrane
    – Inflammatory cells
                                                             Approach to DPLD




                                                                                                    Granulomatous
                      DPLD of known                              Idiopathic Interstitial
                                                                                                    Lung Diseases                      Others
                         Cause                                       Pneumonias
                                                                                                     (Sarcoidosis)




                                                                                                                                         LAM
Drugs                      Exposure                    CVD    IPF                      IIP other than IPF                          Histiocytosis X
                                                                                                                                    Malignancy




        Hypersensitivity                                        Desquamative Interstitial            Respiratory Bronchiolitis-
                                      Pneumoconiosis                 Pneumonia                       Interstitial Lung disease
         Pneumonitis

                                                                                                                                  IPF: 47-64%

                                                                                                                                  NSIP: 14 to 36%

                                                                                                                                  RBILD/DIP: 10-
                                                                                                     Cryptogenic Organizing       17%
                                                                       Acute Interstitial
        Toxic Inhalation                Radiation                        Pneumonia
                                                                                                           Pneumonia
                                                                                                                                  COP: 4-12%

                                                                                                                                  AIP: 2%

                                                                                                                                  LIP: 2%



                                                                    Lymphocytic Interstitial         Non Specific Interstitial
                                                                      Pneumonia                           Pneumonia
Incident Cases of ILD

                                  Occupation
                                     11%       DILD
            Sarcoidosis
                                                5%    DAH
                8%
                                                       4%

                                                             CTD
                                                             9%




                                                               Other
                                                                11%




      Pulmonary Fibrosis
            52%




(Incidence of IPF=26-31 per 100,000)
                                                      Coultas AJRCCM 1994; 150:967
     Historical Classification of IIP

                                                                     UIP/IPF


                                                                            RB-
     UIP                        UIP
                                                                     DIP    ILD    2002
     DIP                                                                           ATS/ERS
                                NSIP
   UIP-BO                    DIP-RBILD
     LIP                                                             AIP
                                AIP
 Giant cell IP
                                                                                  Cellular
                                                                     NSIP
                                                                                  Fibrotic


     1970                       1997                                 COP
Liebow and Carington             Katzenstein


Adapted from Ryu JH, et al. Mayo Clin Proc. 1998;73:1085-1101.       LIP
Adapted from ATS/ERS. Am J Respir Crit Care Med. 2002;165:277-304.
Clinical Assessment

•   History
•   Physical Exam
•   Chest Radiograph
•   Pulmonary Function Testing
    – At Rest
    – Exercise
• Serologic Studies
• Tissue examination
History
•   Age
•   Gender
•   Smoking history
•   Medications
•   Duration of symptoms
•   Environmental exposure
•   Occupational exposure
•   Family history
History: Age and Gender
   Age             Gender


                 – LAM
                 – Tuberous sclerosis
                 – Pneumoconiosis
    History: Smoking

•    All of the following • In Goodpasture’s
     DPLD are associated    syndrome
     with smoking except:    – 100% of smokers vs. 20%
    a)   IPF                    of nonsmokers
    b)   RBILD                  experience pulmonary
                                hemorrhage
    c)   DIP
    d)   HP                • Individuals exposed to
    e)   Histiocytosis X
                             asbestos who smoke are
                             more likely to develop
                             asbestosis
History: Medications




               www.pneumotox.com




                             Schwartz, ILD text book, 4th edition
History: Occupational and
Environmental
             INORGANIC
ORGANIC: Hypersensitivity Pneumonitis
Occupational ????
History: Duration of Illness

2.   Acute Diseases (Days to weeks)
       • DAD (AIP), EP, Vasculitis/DPH, Drug, CVD
       ________________________________________________________________________________________________________________


3.   Subacute Diseases (weeks to months)
       • HSP, Sarcoid, Cellular NSIP, Drug,
       “Chronic” EP, Bronchiolitis/ SAD
     __________________________________________________________________________________________________________________


4.   Chronic Diseases (months to years)
       • UIP, Fibrotic NSIP, Pneumoconioses,
       CVD-related, Chronic HSP
       Smoking (RBILD and PLCH), SAD
Modified Liebow classification of the idiopathic
interstitial pneumonias (Katzenstein)
• Acute
   • Acute interstitial pneumonia             (AIP)

• Subacute
   •   Nonspecific interstitial pneumonia     (NSIP)
   •   Lymphocytic Interstitial Pneumonia     (LIP)
   •   Cryptogenic Organizing Pneumonia       (COP)
   •   Desquamative interstitial pneumonia/   (DIP)
       Respiratory bronchiolitis-associated
       interstitial lung disease              (RBILD)


• Chronic
   • Usual interstitial pneumonia             (UIP)
Physical Findings

•   Resting Tachypnea
•   Shallow breathing
•   Dry crackles
•   Digital clubbing
•   Pulmonary HTN
•   Non-pulmonary
    findings
Laboratory
ILD: Evaluation

• Rdiographic
  – CXR
  – HRCT
• Physiologic testing
  – PFT
  – Exercise test
• Lung Sampling
  – BAL
  – Lung biopsy: (TBBx, Surgical)
CXR: LlMITATIONS

• CXR is normal:
  – in 10 to 15 % of symptomatic patients with
    proven infiltrative lung disease
  – 30% of those with bronchiectasis
  – ~ 60 % of patients with emphysema
• CXR has a sensitivity of 80% and a
  specificity of 82% percent for detection
  of DPLD
• CXR can provide a confident diagnosis
  in ~ 23 % of cases
A normal CXR does not rule
out the presence of DPLD
CXR CLUES
Alveolar Filling
• Air-bronchograms
• Acinar rosettes
• Diffuse consolidation
• Nodule like, poor
  boarder definition
• Silhouetting:
  obliteration of normal
  structures
CXR CLUES

Interstitial Infiltrates
• Nodular
• Linear or reticular
• Mixed
• Honeycomb
• Cysts and traction
  bronchiectasis
• GGO
HRCT

• 2 essential technical factors:
  – Narrow collimation
  – Use of a high spatial frequency reconstruction
    algorithm
• Does not use contrast
• Prone and supine
• Inspiratory and expiratory
Conventional    HRCT




 Supine        Prone
Secondary pulmonary lobular
anatomy
The terminal bronchiole in the center
divides into respiratory bronchioles with   Centrilobular area in blue (left)
acini that contain alveoli.                 and perilymphatic area in yellow
Lymphatics and veins run within the         (right)
interlobular septa
HRCT Clues
• What is the dominant HR-pattern:
   –   Reticular
   –   Nodular
   –   High attenuation (ground-glass, consolidation)
   –   Low attenuation (emphysema, cystic)
• Where is it located within the secondary lobule
  (centrilobular, perilymphatic or random)
• Is there an upper versus lower zone?
• Central versus peripheral predominance
• Are there additional findings (pleural involvement,
  lymphadenopathy, traction bronchiectasis)
HRCT: Radiographic Pattern
HRCT Findings in Late IPF




               Slide courtesy of G Raghu, MD.
Honeycomb lung
Predominant HRCT pattern?


                       Sjogren’s
      “NSIP”
                       Syndrome
Peripheral Location




    COP               IPF
Chronic Alveaolar Infitrates
W (Wegner’s)
E (Eosinophilic pneumonia)
B (BOOP, BAC)
A (PAP, Aspiration)
L (Lymphoma)
L (Lipoid Pneumonia)
S (Sacroidosis)
                                          IPF: CXR




             Reduced lung volume               Basal and peripheral reticulation

Images courtesy of W. Richard Webb, MD.
        Classic IPF HRCT



                        Basal and subpleural predominance




            Reticular opacities             Traction      Honeycombing
                                         bronchiectasis
Image courtesy of W. Richard Webb, MD.
   DIP: Radiographics

• CXR:
  – Normal: 3-22%
  – Patchy GGO
  – lower zone predilection
• HRCT:
  – GGO
     • LL distribution (73 %)
     • peripheral distribution (59%)
Ground Glass Pattern

                 •   HP
                 •   PCP pneumonia
                 •   DIP
                 •   NSIP
                 •   PAP
                 •   DAH
                 •   Fluid
Cysts or Cyst Like

                Bronchiectasis
LAM




               EG

E
     Radiographic Patterns in ILD

Pleural Involvement           Adenopathy          Kerley B lines
Lymphangitic Carcinomatosis   Sarcoidosis       Chronic LV failure
LAM                           Lymphoma          Lymphangitic CA
Drug Induced                  Lymphangitic CA   Lymphoma
Radiation Pneumonitis         LIP               LAM
Asbestosis                    Amyloidosis       Veno-occlusive disease
     Effusion                 Berylliosis       Acute Eosinophilic Pneumonia
     Thickening               Silicosis
     Plaques
     Mesothelioma
Collagen vascular disease
PFT: Lung Volumes
Restrictive Disease



       VC
                             VC
 TLC              VC
            TLC        TLC


       RV                    RV
                  RV

  Normal      ILD      NM Disease
Probability of Histologic Diagnosis of Diffuse Diseases
                                            Transbronchial   Surgical
                                                Biopsy       Biopsy
         1. Granulomatous diseases
         2. Malignant tumors/lymphangitic
         3. DAD (any cause)
         4. Certain infections                      Often
         5. Alveolar proteinosis
         6. Eosinophilic pneumonia
         7. Vasculitis
         8. Amyloidosis
         9. EG/HX/PLCH                              Sometimes
       10. LAM
       11. RB/RBILD/DIP
       12. UIP/NSIP/LIP COP
       13. Small airways disease                    Never
       14. PHT and PVOD
       Courtesy of Kevin O. Leslie, MD.
Video Assisted Thoracic Surgery (VATS)
• VATS is the preferred procedure for obtaining a lung biopsy
      High diagnostic accuracy
      Less morbidity and mortality than open lung biopsy
      BAL and TBBx limited to excluding other IPF mimickers
• Ideal biopsy
      Two or more surgical wedge biopsies with areas of normal lung
      Samples should measure 35 cm in length and 23 cm in depth
• Outpatient thoracoscopic lung biopsy can be a safe and effective
  procedure for patients with interstitial or focal lung disease
      Diagnosis obtained in 61/62 patients
      72.5 % discharged home within 8 hours
      22.5% discharged home within 23 hours


       ATS/ERS Consensus Statement. Am J Respir Crit Care Med.
       2000;161:646-664.
       Chang AC, et al. Ann Thorac Surg. 2002.74;1942-1946.
       Rena O, et al. Eur J Cardiothorac Surg. 1999;16:624-627.
                        Approach to the ILD Patient
                               Patient with Suspected
                                         ILD

                              Hx, PE, CXR, PFT, Labs

                                    Dx likely by          Yes              Is bronch               Yes
                                     bronch?                              diagnostic?
                                      No
                                                                                                   STOP
                                        HRCT



    Hx and HRCT                Hx and HRCT                                                       Atypical
                                                            Suspected
     consistent                 Dx of other                                                   clinical or CT
                                                             other ILD
      with IPF                      ILD                                                      features of IPF

                                                                                Yes
                                                           Dx likely by                       Is bronch          Yes
         STOP                       STOP
                                                            bronch?                          diagnostic?
                                                             No                                      No
                                                                                                                  STOP
                                                                VATS




                UIP          NSIP          RBILD          DIP          DAD              OP           LIP        Non IIP

Martinez F, Flaherty K. Available at: http://www.chestnet.org/education/online/pccu/vol18/lessons03_04/lesson03.php.

				
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