Multidisciplinary Approach to Interstitial Lung...
Transcript of Multidisciplinary Approach to Interstitial Lung...
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Multidisciplinary Approach to Interstitial Lung Diseases
RISHI RAJ
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Case Study: Mr. D.S
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Mr. D.S
• 64 YEAR OLD MALE
• SLOWLY PROGRESSIVE COUGH AND DYSPNEA OVER LAST FEW YEARS, MORE FOR
THE LAST ONE YEAR
• BASILAR CRACKLES ON EXAM
• CXR SHOWED INTERSTITIAL OPACITIES
• REFERRED TO YOUR CLINIC FOR A FORMAL EVALUATION
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Broad/Simplistic categories of ILDs
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Survival differs in ILDs
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Treatment of non-IPF related ILDs
IMMUNOSUPPRESSIVE/CYTOTOXIC MEDICATIONS ARE USEFUL IN TREATING NON-IPF ILDS
INCLUDING CRYPTOGENIC ORGANIZING PNEUMONIA, HYPERSENSITIVITY PNEUMONITIS,
CONNECTIVE TISSUE ASSOCIATED ILD ETC.
Corticosteroids
Azathioprine
Mycophenolate
Cyclophosphamide
Others
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Patients with IPF should generally not be treated chronically with corticosteroids
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Patients with IPF on prednisone and azathioprine are more likely to die or be hospitalized than those on placebo
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Nintedanib reduces rate of FVC decline in IPF patients
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Pirfenidone reduces the rate of decline of FVC
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Mr. D.S
• 64 YEAR OLD MALE
• SLOWLY PROGRESSIVE COUGH AND DYSPNEA OVER LAST FEW YEARS, MORE FOR
THE LAST ONE YEAR
• BASILAR CRACKLES ON EXAM
• CXR SHOWED INTERSTITIAL OPACITIES
• REFERRED TO YOUR CLINIC FOR A FORMAL EVALUATION
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Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
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Clinical exam is THE most important tool in the
diagnosis of Interstitial Lung Diseases
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ILD from one etiology can present with different radiologic and histopathologic patterns
RADIOLOGIC PATTERNS HISTOPATHOLOGIC PATTERNS
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ILDs from different etiologies share the same radiologic and histopathologic patterns
RADIOLOGIC PATTERNS HISTOPATHOLOGIC PATTERNS
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ILD Questionnaires
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Radiographs and other workup as indicated
SEROLOGIC TESTING
Rheumatoid factor
Anti-Scl 70
Etc.
FORMAL RHEUMATOLOGY CONSULTATION
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Inhalational Exposures (Hypersensitivity Pneumonitis)
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Medications and Occupations
OCCUPATIONAL LUNG DISEASES
Occupational history
ALL occupations
DRUGS
Common drugs
Nitrofurantoin
Methotrexate
Amiodarone
Etc.
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Mr. D.S: Additional history
• SMOKED 1 PACK/DAY UNTIL 15 YEARS AGO
• INTERMITTENT WOODWORKING, BUT NOW WEARS MASK
• SOME MOLD IN BATHROOM IN HOME BUT OTHERWISE NO SIGNIFICANT MOLD
INFESTATION
• DOWN CLOTHING AND BEDDING AT HOME
• NO DIAGNOSIS OF A CONNECTIVE TISSUE DISEASE BUT COMPLAINTS OF JOINT PAIN IN
HANDS AND FEET WITHOUT ASSOCIATE SWELLING
• PHYSICAL EXAM DID NOT SHOW ANY EVIDENCE OF ACTIVE OR PAST CONNECTIVE
TISSUE DISEASE
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Mr. D.S: Working Diagnostic Considerations
• IDIOPATHIC PULMONARY FIBROSIS
• CHRONIC HYPERSENSITIVITY PNEUMONITIS
• RHEUMATOID ARTHRITIS ASSOCIATED CONNECTIVE TISSUE DISEASE
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Mr. D.S
• THE CRP, ESR, ANA, ANTI SCL 70, SSA, SSB, MYOSITIS PANEL WERE ALL
NEGATIVE EXCEPT FOR SLIGHT ELEVATION IN RF AND A POSITIVE ANTI CCP
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Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
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Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
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HIGH RESOLUTION CT CHEST
VARIOUS PATTERNS
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CT Chest: Usual Interstitial Pneumonia Pattern
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CT Chest: Probable Usual Interstitial Pneumonia Pattern
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CT Chest: Indeterminate Pattern
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CT Chest: Alternative Diagnosis Pattern
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HIGH RESOLUTION CT CHEST
INTERSTITIAL LUNG DISEASE PROTOCOL
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Conventional vs High Resolution CT
CONVENTIONAL HIGH RESOLUTION CT
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Prone vs. Supine Images
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Mr. D.S: HRCT Images
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Mr. D.S: HRCT Images
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Mr. D.S: HRCT Images
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Mr. D.S:
• CT CHEST SHOWED AN INDETERMINATE UIP PATTERN
• NOT HELPFUL IN NARROWING THE DIFFERENTIAL DIAGNOSIS
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Mr. D.S: Working Diagnostic Considerations
• IDIOPATHIC PULMONARY FIBROSIS
• CHRONIC HYPERSENSITIVITY PNEUMONITIS
• RHEUMATOID ARTHRITIS ASSOCIATED CONNECTIVE TISSUE DISEASE
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Mr. D.S:
• REFERRED TO RHEUMATOLOGY
• THE PATIENT DID NOT MEET CRITERIA FOR CONNECTIVE TISSUE DISEASE AND
RHEUM RECOMMENDED TO DIAGNOSE AND TREAT THE ILD AS IF IT WAS NOT RELATED
TO A CONNECTIVE TISSUE DISEASE
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Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
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Bronchoscopy, lavage and biopsy
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Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
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Surgical Lung BiopsyTHORACOSCOPIC (VATS) LUNG BIOPSY
3 incisions (5-10 mm)
Access to all aspect of the chest
Favored approach if patients will tolerate anesthesia
POSTOPERATIVE CARE
Chest tube in place (overnight
Majority are home in 1-2 days (>90% in our practice)
Primary concern is air leak
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Mortality following surgical lung biopsy
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Mr. D.S:
• REFERRED FOR A SURGICAL LUNG BIOPSY (VATS)
• UNEVENTFUL PROCEDURE AND RECOVERY
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Mr. D.S: Surgical Lung Biopsy
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Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
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Agreement on the final diagnosis increases with multidisciplinary discussion
91 ILD PATIENTS
STEP 1 Expert clinicians and radiologists independently
reviewed HRCT
Opinion: Definite, probable, possible and not UIP
STEP 2 Clinicians and radiologists reviewed HRCT with clinical
information
No discussion between participants
STEP 3 Clinician and radiology conference; discussed results
with each other
STEP 4 Conference: Clinicians, radiologists and pathologists
discussing cases and their diagnoses
STEP 5 All discussants tried to reach a consensus diagnosis
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Stanford Multidisciplinary Interstitial Lung Disease Conference
• PULMONARY MEDICINE
• THORACIC RADIOLOGY
• PULMONARY PATHOLOGY
• RHEUMATOLOGY
• LUNG TRANSPLANT
• THORACIC SURGERY
• CLINICAL RESEARCH
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Mr. D.S: Final Diagnosis
CONSENSUS MULTIDISCIPLINARY DIAGNOSIS: IDIOPATHIC PULMONARY FIBROSIS
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Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
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Mr. D.S: Clinical Course
• STARTED ON ANTI-FIBROTIC MEDICATIONS
• TOLERATED WELL EXCEPT FOR MILD AND MANAGEABLE SYMPTOMS
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Mr. D.S: Clinical Course
QUALIFIED FOR, AND ENROLLED IN A TRIAL FOR NOVEL THERAPEUTIC AGENT FOR
IDIOPATHIC PULMONARY FIBROSIS
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Mr. D.S: Clinical Course
• STABLE FOR 3 YEARS, AND THEN PROGRESSED CLINICALLY
• RECEIVED A DOUBLE LUNG TRANSPLANT AND DOING WELL 1 YEAR
POSTOPERATIVELY
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Questions