Pulmonary Fibrosis &
Sponsored Links
This presentation is the property of its rightful owner.
1 / 70

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


  • 87 Views
  • Uploaded on
  • Presentation posted in: General

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

Download Presentation

Pulmonary Fibrosis & Bronchiectasis Omer Alamoudi, MD, FRCP, FCCP, FACP

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


Pulmonary Fibrosis &Bronchiectasis

Omer Alamoudi, MD, FRCP, FCCP, FACP

Professor, consultant Pulmonologist

Department of Medicine, KAUH

Email: dramoudi@yahoo.com


Pulmonary fibrosis, Idiopathic (IPF)

  • Definition

  • Causes

  • Clinical presentation

  • pathology

  • Diagnosis

  • Management


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 (IPF)

  • Definition

  • Causes

  • Clinical presentation

  • Pathology

  • Diagnosis

  • Management


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 (IPF)

  • Definition

  • Causes

  • Clinical presentation

  • pathology

  • Diagnosis

  • Management


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 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 (IPF)

  • Definition

  • Classification

  • pathogenesis

  • Causes

  • Clinical presentation

  • Pathology

  • Diagnosis

  • Management


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


HISTOPATHOLOGIC ELEMENTS OF UIP


CHRONIC INTERSTITIAL INFLAMMATION IN UIP


Pulmonary fibrosis, Idiopathic (IPF)

  • Definition

  • Causes

  • Clinical presentation

  • Pathology

  • Diagnosis

  • Management


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


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.


Chest radiograph

Reduction of the lung volume

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

Diagnosis IPF


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 IPFHRCT

  • Distortion of the pulmonary architecture

  • Thickening of pulmonary interstitium

  • Ground-glass attenuation.

  • No obvious honeycombing is present


Diagnosis IPFHRCT

  • Advanced stage of pulmonary fibrosis

  • Reticular opacities

  • Honeycombing, predominantly subpleural distribution.


HRCT FINDINGS IN IPF


Restrictive pattern

↓ FVC

↓ FEV1

↑FEV1/FVC

Diagnosis IPFPulmonary function tests


Diagnosis IPFPulmonary function tests

  • Restrictive pattern

  • Lung volumes ( TLC, RV, FRC ↓), DLCO ↓


Diagnosis IPF

  • ABG

    • PO2Reduced (V/Q mismatch)

    • PCO2Normal or Reduced

      Increased (End stage)


Diagnosis IPF

  • Bronchoscopy & Bronchoalveolar lavage (BAL)

    • Assess progression of disease

    • Assess response to therapy

    • Assess prognosis of disease

      • LymphocyteGood prognosis/ respond to therapy

      • Eosinophil &

      • neutrophilsPoor prognosis/ no response to steroid


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 (IPF)

  • Definition

  • Causes

  • Clinical presentation

  • Pathology

  • Diagnosis

  • Management


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


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


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)


IPFManagement

  • Cyclophosphamide

    • Dose 2 mg/kg/day (max 200 mg/day)

    • Leucopenia (WBC < 3000)

    • Opportunistic infection

    • Ca bladder

    • Hemorrhagic cystitis


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


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

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%


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


Definition

Acquired disorder affecting the major bronchi and bronchioles

Permanent dilatation and destruction of bronchial wall

Bronchiectasis


Bronchiectasis

  • Definition

  • Etiology

  • Pathophysiology

  • Clinical findings

  • Diagnosis

  • Management


Bronchiectasis

  • Factors trigger bronchiectasis

    • Infection

    • Impairment of drainage

    • Airway obstruction

    • Defect of host defense


Causes of Bronchiectasis

  • Etiology

  • Pulmonary infections

    • Tuberculosis

    • Viral, Mycoplasma Pneumonia

    • Pertussis (whooping cough)

    • Mycobacterium Aviumintracellulare (MAI)


Causes of Bronchiectasis

  • Airway obstruction

    • FB aspiration

      • Rt lung, lower lobes

      • Obstructive pneumonia, focal bronchiectasis

    • LN enlargement

      • Rt middle lobe syndrome


Causes of Bronchiectasis

  • Defective host defenses

    • Local

      • Ciliary Dyskinesia

    • Systemic

      • Hypogammaglobulinemia

      • AIDS


Causes of Bronchiectasis

  • Rheumatic diseases

    • Rheumatoid arthritis

    • Sjogren’s syndrome

  • Inflammatory bowel diseases

    • Ulcerative colitis

    • Crohns disease


  • Causes of Bronchiectasis

    • Kartagener’s syndrome

      • Immotile cilia

        • (Dextrocardia, Sinusitis, Bronchiectasis)

    • Young’s Syndrome

      • (Sinusitis, Obstructive azoospermia, Bronchiectasis)

  • Cystic fibrosis


  • Causes of Bronchiectasis

    • Allergic bronchopulmonary aspergillosis (ABPA)

      • Central bronchiectasis

      • High IgE level

      • Precipitating, specific antibodies to Aspergillus

      • Long standing asthma

  • Cigarette smoking

  • Idiopathic (50%)


  • Bronchiectasis

    • Definition

    • Etiology

    • Clinical findings

    • Diagnosis

    • Management


    Bronchiectasis

    • Clinical findings

      • Symptoms

        • Cough

        • Daily sputum production

        • Dyspnea

        • Hemoptysis

        • Recurrent pleurisy


    Bronchiectasis

    • Clinical findings

      • Signs

        • Coarse crackles

        • Finger Clubbing(50%)

        • Rhonchi


    Bronchiectasis

    • Definition

    • Etiology

    • Clinical findings

    • Diagnosis

    • Management


    Bronchiectasis

    • Diagnostic evaluation

      • Complete blood count, differential

      • Immunoglobulin levels (IgG, IgM, IgA)

      • Sputum culture, smear (TB, Fungi)


    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)


    Bronchiectasis

    • Diagnostic evaluation

      • High resolution CT scanning (HRCT)

        • Airway dilatation

        • Bronchial wall thickening

        • Cystic changes


    Bronchiectasis

    • Definition

    • Etiology

    • Clinical findings

    • Diagnosis

    • Management


    Bronchiectasis

    • Treatment of infection

      • Acute exacerbation

        • Streptococcus pn., H. influenzae

        • Amoxycillin-clavulinic acid

        • Clarithromycin

        • Cefuroxime

        • Duration 10-14 days


    Bronchiectasis

    • Pseudomonas

      • Extensive bronchiectasis

      • Difficult to eradicate

      • Poor quality of life

      • Increased no of hospitalization

        • Ciprofloxacin orally or IV, aerosolized

        • Bronchodilator


    Bronchiectasis

    • Aspergillus

      • Itraconazole 400mg/day


    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


    Bronchiectasis

    • Bronchodilators

      • Airway reactivity

        • Nebulized Salbutamol

    • Anti-inflammatory medications

      • Inhaled corticosteroids

      • Beclomethasone, budesonide


    Bronchiectasis

    • Surgery

      • Indications

        • Removal destroyed lung

        • Reduction in overwhelming purulent sputum production

        • Uncontrolled hemorrhage

        • Removal of area harboring resistant organism


    Bronchiectasis

    • Bronchial artery embolization

      • Intractable bleeding

        • Preserve lung tissue

        • Avoid thoracotomy

    • Lung transplantation

      • Controversial


    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


    Quiz 1


    Quiz 2: What is the diagnosis


    Thank you


    • Suggested Text book to read

    • Davidson’s principle of internal medicine


  • Login