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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

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Pulmonary fibrosis bronchiectasis omer alamoudi md frcp fccp facp

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


Histopathologic elements of uip

HISTOPATHOLOGIC ELEMENTS OF UIP


Chronic interstitial inflammation in uip

CHRONIC INTERSTITIAL INFLAMMATION IN UIP


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.


Hrct findings in ipf

HRCT FINDINGS IN IPF


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

    • PO2Reduced (V/Q mismatch)

    • PCO2Normal 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

      • LymphocyteGood prognosis/ respond to therapy

      • Eosinophil &

      • neutrophilsPoor 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%


Pulmonary fibrosis bronchiectasis omer alamoudi md frcp fccp facp

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


Pulmonary fibrosis bronchiectasis omer alamoudi md frcp fccp facp

Bronchiectasis


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


    Quiz 1

    Quiz 1


    Pulmonary fibrosis bronchiectasis omer alamoudi md frcp fccp facp

    Quiz 2: What is the diagnosis


    Pulmonary fibrosis bronchiectasis omer alamoudi md frcp fccp facp

    Thank you


    Pulmonary fibrosis bronchiectasis omer alamoudi md frcp fccp facp

    • Suggested Text book to read

    • Davidson’s principle of internal medicine


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