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Interstitial lung disease

Interstitial lung disease






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    Interstitial lung disease Interstitial lung disease Document Transcript

    • Interstitial Lung Disease Presentation Sir, this patient has interstitial lung disease affecting both lower lobes (upper lobes) as evidenced by fine velcro-like late inspiratory crepitations heard best posteriorly(anteriorly) in the lower one third bilaterally. This is associated with clubbing(50%) and a non-productive cough. Chest excursion was reduced bilaterally with a normal percussion note and vocal resonance. Trachea is central and apex beat is not displaced. There are no signs of pulmonary hypertension or cor pulmonale. There are also no features of polycythemia. Patient respiratory rate is 14 breaths per minute and there are no signs of respiratory distress. There are also no signs of respiratory failure. There is also no nicotine staining of the fingers and I note that the patient is cachexic looking with wasting of the temporalis muscles. In terms of aetiology, there is no symmetrical deforming polyarthropathy of the hands to suggest RA, or cutaneous signs to suggest presence of SLE, dermatomyositis or scleroderma as these conditions may be complicated by pulmonary fibrosis. With regards to treatment, patient is not Cushingoid and does not have papery thin skin or steroid purpura to suggest chronic steroid usage. On inspection there are no surgical scars to suggest open lung biopsy. I would like to complete the examination by asking for a detailed drug history as well as an occupational history. In summary, this patient has got pulmonary fibrosis affecting bilateral lower lobes. There are no complications of pulmonary hypertension, cor pulmonale and polycythemia. He is clinically not in respiratory failure and has no features of chronic steroid usage. The differential diagnoses include collagen vascular disease, drugs, occupational causes and idiopathic pulmonary fibrosis. Questions What are the differential diagnoses for clubbing and crepitations? Pulmonary fibrosis Bronchiectasis Lung abscess Mitotic lung conditions What are the characteristic auscultatory findings? Late, fine inspiratory crepitations Velcro-like Disappears or quietens with the patient leaning forwards
    • What are the causes of fibrosis? Upper Lobes S – Silicosis, sarcoidosis C- coal worker pnemoconiosis H- histiocytosis A- Ankylosing spondylitis, ABPA R – radiation T – TB Lower lobes R- RA A-Asbestosis S- Scleroderma I – Idiopathic pulmonary fibrosis O- others ie drugs Cytotoxics – MTX, Aza, bleomycin, bulsulphan, cyclo, chlorambucil CNS - Amitryptyline, phenytoin and carbamazepine CVS - Amiodarone, hydralazine, procainamide Antibiotics - Nitrofurantoin, isoniazid Antirheumatics – Gold, sulphasalazine Both N – Neurofibromatosis, Tuberous sclerosis E – Extrinsic allergic alveolitis (acute symptoms within 6 hrs of inhaled allergens eg farmer’s lungs) P – pulmonary haemorrhage syndromes A – alveolar proteinosis Primary Secondary – Inhaled organic dusts(Silica, Al), chronic infection, malignancy Lymphangiomyomatosis How would you classify interstitial lung disease? (ATS/ERS 2001) Diffuse parenchymal lung disease(DPLD) of known cause Collagen Vascular disease RA, SLE, Dermatomyositis, Systemic sclerosis Occupational/Environmental Asbestosis, silicosis, extrinsic allergic alveolitis Drug related Cytotoxic, CNS, CVS, Antibiotics and antirheumatic Idiopathic IPF Other idiopathic interstitial pneumonias DIP AIP LIP NSIP Cryptogenic organising pneumonia Respiratory bronchiolitis Granulomatous Sarcoidosis Others - LAMs, histiocytosis
    • How would you diagnose idiopathic pulmonary fibrosis? Clinical-radiological-pathological diagnosis Clinical o Exclusion of other causes of ILD o >50 yrs, insidious onset of dyspnea, > 3months, non-productive cough o Typical physical findings Radiological (see below) Pathological (see below) How would you investigate? The diagnostic Ix of choice is a HRCT of the thorax but simple IX such as CXR and LFT are useful: CXR Diagnostic bilateral basal reticulonodular shadows, peripheries, which advances upwards honeycombing in advanced cases (gps of closely set ring shadows) loss of lung volume Extent and distribution Complications Lung function Restrictive pattern (reduced TLC or VC with increased FEV1/FVC ratio) Severity of restriction based on TLC Reduced transfer factor (impaired gas exchange) HRCT scan Dx – patchy reticular abnormalities, focal ground glass, architectural distortion, volume loss, subpleural cyst, honeycombing (no consolidation or nodules) Extent and severity – basal, peripheral, subpleural Complications NB: Similar to that of collagen vascular disease and asbestosis Others Bronchoscopy – lavage Predominantly lymphocyte responds to steroids and better Px= not UIP Predominantly neutrophils and eosinophils means poor Px= UIP (if >20% of eosinophils to consider eosinophilic lung disease) Lung biopsy IPF – Usual interstitial pneumonia Bloods ABGs To rule out causes How would you manage? Education and counselling Stop smoking Regular follow up and vaccinations Treat underlying cause Pharmacological Trial of steroids
    • If responding continue steroids If not responding, cyclophosphamide or azathioprine Antifibrotic agents Eg penicillamine which has not been proven to be useful Surgical Lung transplant (single lung transplantation) Manage complications Cor pulmonale - diuresis for heart failure Polycythemia - venesection if Hct >55% Respiratory failure – Oxygen therapy Monitor for lung cancer What are the good prognosticating factors? Young age Female Short duration Ground glass appearance on the CXR Minimal fibrosis on lung biopsy What is the clinical course of patients with IPF? Gradual onset Progressive Median survival from time of dx about 3 years What are the causes of death? Cor pulmonale Respiratory failure Pneumonia Lung carcinoma What is Hamman-Rich syndrome? Rapidly progressive and fatal variant of interstitial lung disease