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Pulmonary Fibrosis & Bronchiectasis
Omer Alamoudi, MD, FRCP, FCCP, FACP Professor, consultant Pulmonologist Department of Medicine, KAUH
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Pulmonary fibrosis, Idiopathic (IPF)
Definition Causes Clinical presentation pathology Diagnosis Management
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Idiopathic Pulmonary fibrosis (IPF) Definition
Chronic progressive disease of unknown etiology Characterized by inflammation and fibrosis of the lung parenchyma Lung interstitium and alveoli Slide 3 IPF: Definition and Diagnosis
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Pulmonary fibrosis, Idiopathic (IPF)
Definition Causes Clinical presentation Pathology Diagnosis Management
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IPF Causes PCP Radiation pneumonitis Unknown (90%)
Recurrent Intraalveolar hemorrhages Chronic aspiration pneumonia Smoking, wood, metal dust exposure Granulomatosis (Sarcoidosis, histoplasmosis) Unknown (90%) Familial (AD) Viral (Epstein Barr virus) Collagen vascular disorder (RA, scleroderma, SLE, dermatomyositis) Asbestosis Drugs (Amiodarone, Busulphan, Bleomycin)
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Pulmonary fibrosis, Idiopathic (IPF)
Definition Causes Clinical presentation pathology Diagnosis Management
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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
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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)
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Pulmonary fibrosis, Idiopathic (IPF)
Definition Classification pathogenesis Causes Clinical presentation Pathology Diagnosis Management
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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
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HISTOPATHOLOGIC ELEMENTS OF UIP
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CHRONIC INTERSTITIAL INFLAMMATION IN UIP
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Pulmonary fibrosis, Idiopathic (IPF)
Definition Causes Clinical presentation Pathology Diagnosis Management
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IPF Diagnosis 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
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Diagnosis IPF Chest radiograph Early: ground glass appearance
Late: reticular, reticulonodular, honeycombing at the periphery Deviation of trachea to the right Bilateral lower lobe opacities and possible mild decrease in lung volumes.
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Diagnosis IPF Chest radiograph Reduction of the lung volume
Pleural involvement, adenopathy, localized parenchymal densities (very rare)
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Diagnosis IPF High Resolution CT (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
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Diagnosis IPF HRCT Distortion of the pulmonary architecture
Thickening of pulmonary interstitium Ground-glass attenuation. No obvious honeycombing is present
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Diagnosis IPF HRCT Advanced stage of pulmonary fibrosis
Reticular opacities Honeycombing, predominantly subpleural distribution.
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HRCT FINDINGS IN IPF
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Diagnosis IPF Pulmonary function tests
Restrictive pattern ↓ FVC ↓ FEV1 ↑FEV1/FVC
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Diagnosis IPF Pulmonary function tests
Restrictive pattern Lung volumes ( TLC, RV, FRC ↓), DLCO ↓
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Diagnosis IPF ABG PO2 Reduced (V/Q mismatch) PCO2 Normal or Reduced
Increased (End stage)
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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
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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
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Pulmonary fibrosis, Idiopathic (IPF)
Definition Causes Clinical presentation Pathology Diagnosis Management
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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
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IPF Management 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
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How to assess the response to therapy
IPF Management How to assess the response to therapy Reduction of symptoms Improvement of lung function tests Improvement of DLCO Improvement of chest x ray (alveolitis)
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IPF Management Cyclophosphamide Dose 2 mg/kg/day (max 200 mg/day)
Leucopenia (WBC < 3000) Opportunistic infection Ca bladder Hemorrhagic cystitis
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IPF Management 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
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IPF Management Oxygen supply (rest and exercise) if PO2< 55 mmHg
Lung transplantation Age <60 Progressive disease Lack of response to therapy Psychosocial support Influenza vaccine
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CAUSE OF DEATH IPF [N=543] 1-7 year FU 60% Died [N=326] Respiratory
failure 39% Lung cancer 10% Pulmonary embolism 3% Pulmonary infection 3% Cardiovascular disease 27% Other 18%
![CAUSE OF DEATH IPF [N=543] 1-7 year FU 60% Died [N=326] Respiratory](http://slideplayer.com/slide/5681390/18/images/34/CAUSE+OF+DEATH+IPF+%5BN%3D543%5D+1-7+year+FU+60%25+Died+%5BN%3D326%5D+Respiratory.jpg "failure. 39% Lung. cancer. 10% Pulmonary. embolism. 3% Pulmonary. infection. 3% Cardiovascular. disease. 27% Other. 18%")
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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
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Bronchiectasis
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Bronchiectasis Definition Etiology Clinical findings Diagnosis
Management
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Bronchiectasis Definition
Acquired disorder affecting the major bronchi and bronchioles Permanent dilatation and destruction of bronchial wall
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Bronchiectasis Definition Etiology Pathophysiology Clinical findings
Diagnosis Management
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Bronchiectasis Factors trigger bronchiectasis Infection
Impairment of drainage Airway obstruction Defect of host defense
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Causes of Bronchiectasis
Etiology Pulmonary infections Tuberculosis Viral, Mycoplasma Pneumonia Pertussis (whooping cough) Mycobacterium Avium intracellulare (MAI)
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Causes of Bronchiectasis
Airway obstruction FB aspiration Rt lung, lower lobes Obstructive pneumonia, focal bronchiectasis LN enlargement Rt middle lobe syndrome
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Causes of Bronchiectasis
Defective host defenses Local Ciliary Dyskinesia Systemic Hypogammaglobulinemia AIDS
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Causes of Bronchiectasis
Rheumatic diseases Rheumatoid arthritis Sjogren’s syndrome Inflammatory bowel diseases Ulcerative colitis Crohns disease
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Causes of Bronchiectasis
Kartagener’s syndrome Immotile cilia (Dextrocardia, Sinusitis, Bronchiectasis) Young’s Syndrome (Sinusitis, Obstructive azoospermia, Bronchiectasis) Cystic fibrosis
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Causes of Bronchiectasis
Allergic bronchopulmonary aspergillosis (ABPA) Central bronchiectasis High IgE level Precipitating, specific antibodies to Aspergillus Long standing asthma Cigarette smoking Idiopathic (50%)
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Bronchiectasis Definition Etiology Clinical findings Diagnosis
Management
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Bronchiectasis Clinical findings Symptoms Cough
Daily sputum production Dyspnea Hemoptysis Recurrent pleurisy
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Bronchiectasis Clinical findings Signs Coarse crackles
Finger Clubbing (50%) Rhonchi
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Bronchiectasis Definition Etiology Clinical findings Diagnosis
Management
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Bronchiectasis Diagnostic evaluation
Complete blood count, differential Immunoglobulin levels (IgG, IgM, IgA) Sputum culture, smear (TB, Fungi)
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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)
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Bronchiectasis Diagnostic evaluation
High resolution CT scanning (HRCT) Airway dilatation Bronchial wall thickening Cystic changes
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Bronchiectasis Definition Etiology Clinical findings Diagnosis
Management
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Bronchiectasis Treatment of infection Acute exacerbation
Streptococcus pn., H. influenzae Amoxycillin-clavulinic acid Clarithromycin Cefuroxime Duration days
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Bronchiectasis Pseudomonas Extensive bronchiectasis
Difficult to eradicate Poor quality of life Increased no of hospitalization Ciprofloxacin orally or IV, aerosolized Bronchodilator
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Bronchiectasis Aspergillus Itraconazole 400mg/day
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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
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Bronchiectasis Bronchodilators Airway reactivity Nebulized Salbutamol
Anti-inflammatory medications Inhaled corticosteroids Beclomethasone, budesonide
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Bronchiectasis Surgery Indications Removal destroyed lung
Reduction in overwhelming purulent sputum production Uncontrolled hemorrhage Removal of area harboring resistant organism
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Bronchiectasis Bronchial artery embolization Intractable bleeding
Preserve lung tissue Avoid thoracotomy Lung transplantation Controversial
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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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Quiz 1
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Quiz 2: What is the diagnosis
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Thank you
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Suggested Text book to read
Davidson’s principle of internal medicine
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