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Interstitial Lung Disease

Updated: Sep 14, 2024


Physical Examination

General examination:

  • Tachypnoea (advanced disease)

  • Central cyanosis

Chest inspection:

  • Flattened chest (affected side)

Palpation:

  • Reduced chest expansion

  • Increased tactile vocal fremitus and resonance (difficult to appreciate)

Percussion:

  • Dullness to percussion (affected zones)

Auscultation:

  • Fine end-inspiratory crepitations (affected zones)

  • Unaffected by coughing

Other signs:

  • Evidence of pulmonary hypertension

  • Signs of treatment, such as steroid purpura and oxygen supplementation

If associated with underlying causes:

  • Slate grey appearance from amiodarone

  • Peripheral symmetrical deforming polyarthropathy (swan neck deformity, Z thumb, ulnar deviation at wrist, may have nodules) from rheumatoid arthritis

  • Cutaneous signs of systemic sclerosis, lupus, etc. from connective tissue disease

  • Question mark posture, protuberant abdomen from ankylosing spondylitis

  • May have radiation tattoo on chest wall, lymphadenopathy from radiation

  • Cutaneous signs of sarcoidosis

Investigations

Laboratory investigations:

  • Full blood count, erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP): to assess for evidence of inflammation or infection

  • Renal and liver function tests: to assess for organ dysfunction which may impact treatment options or require dose adjustments

  • Serum autoantibodies: to screen for autoimmune diseases that can be associated with interstitial lung disease (e.g. anti-nuclear antibody, anti-SSA/SSB, anti-Jo-1, anti-Scl-70)

  • Serum immunoglobulins: to assess for hypergammaglobulinemia which may suggest sarcoidosis or connective tissue disease

Imaging investigations:

  • Chest X-ray: to evaluate for evidence of interstitial lung disease, assess the extent and distribution of disease, and exclude other lung pathologies

  • High-resolution computed tomography (HRCT) of the chest: to confirm the presence of interstitial lung disease, assess the pattern and extent of disease, and guide further management

Invasive investigations:

  • Bronchoalveolar lavage (BAL): to obtain fluid and cells from the lungs for microbiological and cytological analysis, and exclude infection

  • Transbronchial lung biopsy (TBLB): to obtain tissue samples for histological analysis and confirm the diagnosis of interstitial lung disease

  • Video-assisted thoracoscopic surgery (VATS) lung biopsy: for cases where TBLB is inconclusive or not possible, to obtain larger tissue samples for diagnosis

Other investigations:

  • Pulmonary function tests (PFTs): Restrictive pattern

  • Exercise testing: to assess exercise tolerance and determine the need for oxygen therapy

  • Cardiac evaluation: to assess for evidence of pulmonary hypertension or cor pulmonale which may require specific treatment

Management

General management:

  • Education and advice on smoking cessation

  • Pulmonary rehabilitation

  • Annual influenza and pneumococcal vaccination

  • Airway clearance techniques (e.g., chest physiotherapy, positive expiratory pressure devices)

  • Regular follow-up with a respiratory specialist or multidisciplinary team

  • Psychological support for patients with significant symptoms or impact on quality of life


Medical management:

  • Antibiotics for exacerbations

  • Mucolytic agents (e.g., carbocisteine, dornase alfa) to help clear mucus

  • Bronchodilators (e.g., salbutamol) to help with airflow obstruction

  • Inhaled corticosteroids to reduce inflammation

  • Oxygen therapy for hypoxemia


Surgical management:

  • Surgery may be considered for localized disease, particularly if the bronchiectasis is causing significant symptoms or hemoptysis

  • Lobectomy, segmentectomy, or pneumonectomy may be performed depending on the extent of the disease

Causes of Interstitial Lung Disease

  • Idiopathic Interstitial Pneumonias (IIPs)

  • Connective Tissue Disease-associated ILD (CTD-ILD): Rheumatoid arthritis (RA), Systemic sclerosis (SSc), Systemic lupus erythematosus (SLE)

  • Hypersensitivity Pneumonitis (HP): Extrinsic allergic alveolitis

  • Occupational and Environmental ILD: Asbestosis, Silicosis

  • Drug-induced ILD: Amiodarone, Bleomycin, Methotrexate, Nitrofurantoin, Sulfasalazine

  • Sarcoidosis

  • Vasculitis : Granulomatosis with polyangiitis)

  • Miscellaneous causes: Radiation pneumonitis


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