slide1
Download
Skip this Video
Download Presentation
Pulmonary Fibrosis & Bronchiectasis Omer Alamoudi, MD, FRCP, FCCP, FACP

Loading in 2 Seconds...

play fullscreen
1 / 70

Pulmonary Fibrosis & Bronchiectasis Omer Alamoudi, MD, FRCP, FCCP, FACP - PowerPoint PPT Presentation


  • 123 Views
  • Uploaded on

Pulmonary Fibrosis & Bronchiectasis Omer Alamoudi, MD, FRCP, FCCP, FACP Professor, consultant Pulmonologist Department of Medicine, KAUH Email: [email protected] Pulmonary fibrosis, Idiopathic (IPF). Definition Causes Clinical presentation pathology Diagnosis Management.

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'Pulmonary Fibrosis & Bronchiectasis Omer Alamoudi, MD, FRCP, FCCP, FACP' - ginata


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
slide1
Pulmonary Fibrosis &Bronchiectasis

Omer Alamoudi, MD, FRCP, FCCP, FACP

Professor, consultant Pulmonologist

Department of Medicine, KAUH

Email: [email protected]

pulmonary fibrosis idiopathic ipf
Pulmonary fibrosis, Idiopathic (IPF)
  • Definition
  • Causes
  • Clinical presentation
  • pathology
  • Diagnosis
  • Management
idiopathic pulmonary fibrosis ipf definition
Chronic progressive disease of unknown etiology

Characterized by inflammation and fibrosis of the lung parenchyma

Lung interstitium and alveoli

Idiopathic Pulmonary fibrosis (IPF)Definition
pulmonary fibrosis idiopathic ipf1
Pulmonary fibrosis, Idiopathic (IPF)
  • Definition
  • Causes
  • Clinical presentation
  • Pathology
  • Diagnosis
  • Management
ipf causes
Unknown (90%)

Familial (AD)

Viral (Epstein Barr virus)

Collagen vascular disorder (RA, scleroderma, SLE, dermatomyositis)

Asbestosis

Drugs (Amiodarone, Busulphan, Bleomycin)

PCP

Radiation pneumonitis

Recurrent Intraalveolar hemorrhages

Chronic aspiration pneumonia

Smoking, wood, metal dust exposure

Granulomatosis (Sarcoidosis, histoplasmosis)

IPFCauses
pulmonary fibrosis idiopathic ipf2
Pulmonary fibrosis, Idiopathic (IPF)
  • Definition
  • Causes
  • Clinical presentation
  • pathology
  • Diagnosis
  • Management
pulmonary fibrosis idiopathic ipf clinical presentation
Pulmonary fibrosis, Idiopathic (IPF)Clinical presentation
  • Onset: Usually between 50 and 70 yr
  • Clinical presentation
    • Progressive dyspnea on exertion
    • Paroxysmal cough, usually nonproductive
    • Clubbing of the fingers (50%)
    • Fine inspiratory basal crackles chest auscultation
    • Abnormal chest x-ray or HRCT
    • Restrictive pulmonary physiology with reduced lung volumes and DLCO and widened AaPO2
pulmonary fibrosis idiopathic ipf clinical presentation1
Pulmonary fibrosis, Idiopathic (IPF)Clinical presentation
  • Signs of pulmonary hypertension
    • Loud P 2
    • Right ventricular heave
    • RBBB
  • Signs of Corpulmonale and Rt heart failure
    • Raised JVP
    • Hepatomegaly, ascitis
    • Lower limb edema
  • Signs of underlying causes (RA, scleroderma)
pulmonary fibrosis idiopathic ipf3
Pulmonary fibrosis, Idiopathic (IPF)
  • Definition
  • Classification
  • pathogenesis
  • Causes
  • Clinical presentation
  • Pathology
  • Diagnosis
  • Management
ipf pathology
IPF: Pathology
  • UIP is essential to diagnosis of IPF
    • Idiopathic, progressive, diffuse fibrosing inflammatory process
    • Involves lung parenchyma
  • Surgical lung biopsy
    • suspected IPF
    • Atypical clinical or radiographic features
  • Major purpose of histologic examination is to distinguish UIP from other histologic subsets of IIP
pulmonary fibrosis idiopathic ipf4
Pulmonary fibrosis, Idiopathic (IPF)
  • Definition
  • Causes
  • Clinical presentation
  • Pathology
  • Diagnosis
  • Management
ipf diagnosis
IPFDiagnosis
  • Major criteria
    • Exclusion of other known causes of ILD
    • Restrictive pulmonary function studies
    • Bibasilar reticular abnormalities on HRCT scan
    • No histologic or cytologic features on transbronchial lung biopsy or BAL analysis supporting another diagnosis
  • Minor criteria
    • Age >50 yr
    • Insidious onset of otherwise unexplained exertional dyspnea
    • Duration of illness 3 mo
    • Bibasilar, dry (“Velcro”) inspiratory crackles
diagnosis ipf
Chest radiograph

Early: ground glass appearance

Late: reticular, reticulonodular, honeycombing at the periphery

Deviation of trachea to the right

Diagnosis IPF

Bilateral lower lobe opacities and possible mild decrease in lung volumes.

diagnosis ipf1
Chest radiograph

Reduction of the lung volume

Pleural involvement, adenopathy, localized parenchymal densities (very rare)

Diagnosis IPF
diagnosis ipf high resolution ct hrct
Diagnosis IPFHigh Resolution CT (HRCT)
  • HRCT
    • Useful to differentiate IPF from other ILD
    • Determine the severity and extent of the disease
    • Select the place for biopsy
  • Findings
    • Patchy, ground glass attenuation, thickened interlobular septae
    • Traction bronchiectasis
    • Reticular pattern, honeycombing
diagnosis ipf hrct
Diagnosis IPFHRCT
  • Distortion of the pulmonary architecture
  • Thickening of pulmonary interstitium
  • Ground-glass attenuation.
  • No obvious honeycombing is present
diagnosis ipf hrct1
Diagnosis IPFHRCT
  • Advanced stage of pulmonary fibrosis
  • Reticular opacities
  • Honeycombing, predominantly subpleural distribution.
diagnosis ipf pulmonary function tests1
Diagnosis IPFPulmonary function tests
  • Restrictive pattern
  • Lung volumes ( TLC, RV, FRC ↓), DLCO ↓
diagnosis ipf2
Diagnosis IPF
  • ABG
    • PO2 Reduced (V/Q mismatch)
    • PCO2 Normal or Reduced

Increased (End stage)

diagnosis ipf3
Diagnosis IPF
  • Bronchoscopy & Bronchoalveolar lavage (BAL)
    • Assess progression of disease
    • Assess response to therapy
    • Assess prognosis of disease
      • Lymphocyte Good prognosis/ respond to therapy
      • Eosinophil &
      • neutrophils Poor prognosis/ no response to steroid
diagnosis ipf4
Diagnosis IPF
  • Other tests
    • Lung biopsy (open vs TBBB) most important
    • Gallium-67 increased (not useful)
    • PET (position emission tomography)
      • Glucose uptake increased
    • ESR, ANA, RF
pulmonary fibrosis idiopathic ipf5
Pulmonary fibrosis, Idiopathic (IPF)
  • Definition
  • Causes
  • Clinical presentation
  • Pathology
  • Diagnosis
  • Management
therapeutic approaches to ipf
Corticosteroids

Other immunosuppressives

Azathioprine

Cyclophosphamide

Antifibrotic agents

Colchicine

D-Penicillamine

IFN-

IFN-

Pirfenidone

Antioxidant agents

Glutathione

N-acetylcysteine

Others

Agents that block neutrophil adhesion molecules

Inhibitors of specific fibrogenic cytokines and growth factors

THERAPEUTIC APPROACHES TO IPF
ipf management
IPFManagement
  • Should started as early as possible
  • Duration of therapy 3-6 months
  • Corticosteroids
    • Prednisone tablets
      • Dose 1-1.5mg/kg (30Mg)
    • Methyl prednisone (pulse therapy 3-5 days)
      • Dose 1 gm/day
ipf management1
IPFManagement
  • How to assess the response to therapy
    • Reduction of symptoms
    • Improvement of lung function tests
    • Improvement of DLCO
    • Improvement of chest x ray (alveolitis)
ipf management2
IPFManagement
  • Cyclophosphamide
    • Dose 2 mg/kg/day (max 200 mg/day)
    • Leucopenia (WBC < 3000)
    • Opportunistic infection
    • Ca bladder
    • Hemorrhagic cystitis
ipf management3
IPFManagement
  • Azathioprine
    • 1-2mg/kg/day (max 200Mg/day)
    • GIT symptoms
    • Bone marrow suppression
    • Increase liver enzymes
    • Leucopenia, and thrombocytopenia (WBC< 3000)
  • Colchicine (anti-inflammatory)
    • Dose 0.5mg BID
ipf management4
IPFManagement
  • Oxygen supply (rest and exercise) if PO2< 55 mmHg
  • Lung transplantation
    • Age <60
    • Progressive disease
    • Lack of response to therapy
  • Psychosocial support
  • Influenza vaccine
cause of death
CAUSE OF DEATH

IPF

[N=543]

1-7 year FU

60% Died

[N=326]

Lung

cancer

10%

Pulmonary

embolism

3%

Pulmonary

infection

3%

Cardiovascular

disease

27%

Other

18%

Respiratory

failure

39%

slide35
RISK FACTORS FOR PROGRESSIVE DISEASE

Age: >50 yr

Gender: male

Dyspnea: moderate to severe with exertion

History of cigarette smoking

Lung function: moderate to severe loss (especially gas exchange with exercise)

BAL fluid: neutrophilia or eosinophilia at presentation

HRCT scan: reticular opacities or honeycomb changes

Response to corticosteroids: poor

Pathology: more fibrosis, fibroblastic foci

bronchiectasis
Bronchiectasis
  • Definition
  • Etiology
  • Clinical findings
  • Diagnosis
  • Management
bronchiectasis1
Definition

Acquired disorder affecting the major bronchi and bronchioles

Permanent dilatation and destruction of bronchial wall

Bronchiectasis
bronchiectasis2
Bronchiectasis
  • Definition
  • Etiology
  • Pathophysiology
  • Clinical findings
  • Diagnosis
  • Management
bronchiectasis3
Bronchiectasis
  • Factors trigger bronchiectasis
      • Infection
      • Impairment of drainage
      • Airway obstruction
      • Defect of host defense
causes of bronchiectasis
Causes of Bronchiectasis
  • Etiology
  • Pulmonary infections
    • Tuberculosis
    • Viral, Mycoplasma Pneumonia
    • Pertussis (whooping cough)
    • Mycobacterium Aviumintracellulare (MAI)
causes of bronchiectasis1
Causes of Bronchiectasis
  • Airway obstruction
    • FB aspiration
      • Rt lung, lower lobes
      • Obstructive pneumonia, focal bronchiectasis
    • LN enlargement
      • Rt middle lobe syndrome
causes of bronchiectasis2
Causes of Bronchiectasis
  • Defective host defenses
    • Local
      • Ciliary Dyskinesia
    • Systemic
      • Hypogammaglobulinemia
      • AIDS
causes of bronchiectasis3
Causes of Bronchiectasis
  • Rheumatic diseases
      • Rheumatoid arthritis
      • Sjogren’s syndrome
  • Inflammatory bowel diseases
      • Ulcerative colitis
      • Crohns disease
causes of bronchiectasis4
Causes of Bronchiectasis
  • Kartagener’s syndrome
    • Immotile cilia
      • (Dextrocardia, Sinusitis, Bronchiectasis)
  • Young’s Syndrome
      • (Sinusitis, Obstructive azoospermia, Bronchiectasis)
  • Cystic fibrosis
causes of bronchiectasis5
Causes of Bronchiectasis
  • Allergic bronchopulmonary aspergillosis (ABPA)
      • Central bronchiectasis
      • High IgE level
      • Precipitating, specific antibodies to Aspergillus
      • Long standing asthma
  • Cigarette smoking
  • Idiopathic (50%)
bronchiectasis4
Bronchiectasis
  • Definition
  • Etiology
  • Clinical findings
  • Diagnosis
  • Management
bronchiectasis5
Bronchiectasis
  • Clinical findings
    • Symptoms
      • Cough
      • Daily sputum production
      • Dyspnea
      • Hemoptysis
      • Recurrent pleurisy
bronchiectasis6
Bronchiectasis
  • Clinical findings
    • Signs
      • Coarse crackles
      • Finger Clubbing (50%)
      • Rhonchi
bronchiectasis7
Bronchiectasis
  • Definition
  • Etiology
  • Clinical findings
  • Diagnosis
  • Management
bronchiectasis8
Bronchiectasis
  • Diagnostic evaluation
      • Complete blood count, differential
      • Immunoglobulin levels (IgG, IgM, IgA)
      • Sputum culture, smear (TB, Fungi)
bronchiectasis9
Bronchiectasis
  • Diagnostic evaluation
    • Chest radiography (PA, lateral)
      • Abnormal in most patients
      • Linear atelectasis
      • Dilated , thickened airways (tram, parallel lines, ring shadows)
      • Central → ABPA
      • Upper lobe (TB, cystic fibrosis)
bronchiectasis10
Bronchiectasis
  • Diagnostic evaluation
    • High resolution CT scanning (HRCT)
      • Airway dilatation
      • Bronchial wall thickening
      • Cystic changes
bronchiectasis11
Bronchiectasis
  • Definition
  • Etiology
  • Clinical findings
  • Diagnosis
  • Management
bronchiectasis12
Bronchiectasis
  • Treatment of infection
    • Acute exacerbation
      • Streptococcus pn., H. influenzae
      • Amoxycillin-clavulinic acid
      • Clarithromycin
      • Cefuroxime
      • Duration 10-14 days
bronchiectasis13
Bronchiectasis
  • Pseudomonas
    • Extensive bronchiectasis
    • Difficult to eradicate
    • Poor quality of life
    • Increased no of hospitalization
      • Ciprofloxacin orally or IV, aerosolized
      • Bronchodilator
bronchiectasis14
Bronchiectasis
  • Aspergillus
    • Itraconazole 400mg/day
bronchiectasis15
Bronchiectasis
  • Bronchial hygiene
    • Hydration and mucus clearance
      • Oral liquids
      • Nebulization with saline solution, mucolytic agents (DNase)
    • Physiotherapy
      • Chest percussion technique
      • Mechanical vibrator
      • 15-30 min/session, 2-3 times/day
bronchiectasis16
Bronchiectasis
  • Bronchodilators
    • Airway reactivity
      • Nebulized Salbutamol
  • Anti-inflammatory medications
      • Inhaled corticosteroids
      • Beclomethasone, budesonide
bronchiectasis17
Bronchiectasis
  • Surgery
    • Indications
      • Removal destroyed lung
      • Reduction in overwhelming purulent sputum production
      • Uncontrolled hemorrhage
      • Removal of area harboring resistant organism
bronchiectasis18
Bronchiectasis
  • Bronchial artery embolization
    • Intractable bleeding
      • Preserve lung tissue
      • Avoid thoracotomy
  • Lung transplantation
    • Controversial
ipf vs bronchiectasis
IPF vs Bronchiectasis
  • Bilateral, Basal
  • Sputum –ve
  • Dry cough
  • Alveoli / interstitium
  • Hypoxemia ++
  • Fine crackles
  • Restrictive pattern
  • Unilateral or bilateral
  • Sputum ++++
  • Productive cough
  • Airways
  • Hypoxemia ±
  • Coarse crackles
  • Obstructive/restrictive
slide70
Suggested Text book to read
  • Davidson’s principle of internal medicine
ad