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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.

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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 tests

Restrictive pattern

↓ FVC

↓ FEV1

↑FEV1/FVC

Diagnosis IPFPulmonary function tests


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%


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






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