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Respiratory Complication Of Rheumatic Disease
- 1. Respiratory Complications of Rheumatic Diseases Peter Luce Consultant in General and Chest Medicine University Hospital Lewisham
- 2. Relevant Issues Clinical Diseases • Imaging • Rheumatoid Arthritis • Lung Function • Scleroderma • BAL • SLE • Biopsy • Sjögren’s syndrome • Treatment • MCTD • Dermatomyositis
- 3. Causes of diffuse parenchymal lung disease (DPLD) >400 Acute • Infection, Allergy, Toxins, Vasculitis, ARDS Episodic • Eosinophilic pneumonia, Churg-Strauss, • Vasculitis, Extrinsic allergic alveolitis, • Cryptogenic organising pneumonia Chronic secondary to environment • Dust, Fungi • Drugs - Antibiotics - Anti-rheumatics: gold, penicillamine - Chemotherapy: bleomycin, methotrexate
- 4. Causes of diffuse parenchymal lung disease (DPLD) >400 Chronic with systemic disease • Connective tissue diseases RA, AS, SLE, Scleroderma, Sjögren’s syndrome, MCTD, Polymyositis, Behçet’s • Neoplastic • Vasculitis • Sarcoid • Inherited
- 5. Clinical Evaluation - I Clinical Assessment • Detailed timescale • Get all old chest X-rays • Environmental, occupation, pets, travel • Smoking and drug history • Cardiac disease • HIV risk • Family history
- 6. Clinical Evaluation - II Respiratory symptoms and signs • Dyspnoea • Cough, especially in lymphangitis, sarcoid, CFA and COP • Pleurisy (50% in SLE, 25% in RA) • Chest pain - pneumothorax • Haemoptysis
- 7. Clinical Evaluation - III Clinical Findings • Fine end respiratory crackles (up to 90%) • Clubbing (50% in CFA, 75% in RA-associated lung disease) • Pulmonary hypertension • Cor pulmonale
- 8. Laboratory Investigations • full blood count and eosinophils • urea and electrolytes • liver function tests • rheumatoid factor • anti-nuclear antibodies • (ANCA, ABMA, ACE)
- 9. Imaging I Diagnosis • Chest X-ray – may be normal at presentation – very non-specific • HRCT – 94% sensitive, 85% sensitive – radiation dose 7 times chest X-ray dose
- 10. Imaging II Disease Activity and Diagnosis • HRCT very specific for CFA and FA in relation to pre-existing rheumatic disease • Can delineate fibrosis • Traction bronchiectasis • Ground glass appearance of alveolitis • Peripheral fibrosis is better survival predictor than central fibrosis
- 11. Imaging III Benefits of HRCT • Increased likelihood of diagnosis from an extensive disease and potential biopsy site • Clinical and HRCT should give 80% of correct diagnoses in DPLD • Biopsy may not be needed • Valuable in determining activity and prognosis
- 12. Imaging IV Other imaging • Gallium scanning • DTPA • PET • (MRI)
- 13. Lung function testing Diagnosis • Restrictive pattern commonest - often combined with airflow obstruction • Spirometry and gas transfer best measure • Exercise testing not helpful • Cannot distinguish inflammation and fibrosis Monitoring • Vital capacity and TLCO most appropriate measures • Inadequate data as serial predictors
- 14. Bronchoalveolar lavage • Increased granulocytes in fibrosing alveolitis with/without rheumatic disease • Increased lymphocytes in granulomatous or drug-induced toxicity • Helpful in diagnosis of infection or malignancy • Role in monitoring unclear
- 15. Lung biopsy • Needle biopsy useful for focal lesions only • Transbronchial biopsy good for sarcoid, malignancy or COP • Not useful for staging fibrosing alveolitis • Open lung biopsy or VATS
- 16. Rheumatoid Arthritis - Diffuse parenchymal lung disease • Risk high in men, smokers, nodular RA, family history, high RF or ANA • Severity of joint disease not predictor of DPLD • Differential diagnosis - infections, gold, MTX Treatment • Steroids alone (1 study) improved exercise tolerance; COP markedly improved • Immunosuppressants, especially azathioprine, improved exercise tolerance • Treat as CFA
- 17. Rheumatoid Arthritis - Other pulmonary manifestations Pleural disease • pleural effusion 5%, pleuritic pain 20% • commoner in males • exudate with low glucose • rheumatoid factor usually in high titres (careful with empyema)
- 18. Rheumatoid Arthritis - Other pulmonary manifestations Pulmonary nodules • 1-2cms; may cavitate, cause haemoptysis, cause pneumothorax or become colonised with aspergillus Obliterative bronchiolitis • Progressive airflow obstruction • Primary or secondary to gold, sulphasalazine, penicillamine or NSAID
- 19. Rheumatoid Arthritis - Other pulmonary manifestations • Cricoarytenoid arthritis Especially females in association with temporomandibular disease • Vocal cord nodules • Pulmonary and pleural infections Worse with steroids • Pulmonary arteritis • Lung cancer • Bullae and Pneumothorax
- 20. SLE - I Pleural disease • Pleuritis in up to 50%, may be recurrent • Pleural effusion in 20%, usually exudate • Also associated with pericarditis • NSAIDs, rarely steroids and immunosuppressives DPLD • Infection most common cause • Acute lupus pneumonitis 0.9% + alveolar haemorrhage (17%) worse post partum • Chronic pneumonitis 13% • Treat as for CFS, results unclear
- 21. SLE - II Other respiratory complications • Pulmonary hypertension • Pulmonary thromboembolism • Respiratory muscle involvement and shrinking lung syndrome
- 22. Scleroderma - Interstitial fibrosis • Interstitial fibrosis in 80% • CXR abnormal in 13-78% but 44% with normal CXR at presentation have abnormal HRCT • DLCO sensitive, associated with anti Scl-70; anti-centromere Ab often with normal DLCO • DLCO<40% normal ⇒ 9% 5 year survival DLCO>40% normal ⇒75% 5 year survival • Treatment - some evidence for steroids and cyclophosphamide
- 23. Scleroderma - Pulmonary Hypertension • Pulmonary hypertension in 50% • Pulmonary artery pressure > 20mmHg at rest • Primary pulmonary hypertension or secondary to cardiac or interstitial lung disease • 9% limited scleroderma have clinical pulmonary hypertension • 65% limited scleroderma have pulmonary hypertension on biopsy or PM • Isolated pulmonary hypertension, 40% 2yr survival
- 24. Scleroderma - Pulmonary hypertension • Vasodilators • Calcium antagonists • Anticoagulants • Prostacyclin • Transplantation
- 25. Other rheumatic conditions • Sjögren’s syndrome • Dermatopolymyositis • Ankylosing Spondylitis • Vascular disease – Wegener’s granulomatosis – Churg-Strauss syndrome
- 26. Drugs and Interstitial Lung Disease Pneumonitis Fibrosis • Methotrexate • Methotrexate • Gold • Gold • Penicillamine • Cyclophosphamide • Cyclophosphamide • Chlorambucil • Chlorambucil • Azathioprine • Azathioprine • Sulphasalazine • Sulphasalazine • NSAID
- 27. Drugs and Interstitial Lung Disease Bronchospasm Bronchiolitis Obliterans • Methotrexate • Methotrexate • Salicylates • Cyclophosphamide • NSAID • Salicylates • NSAID • Colchicine
- 28. The Future • Increased understanding of relationship between – intracellular matrix – signalling proteins – fibroblast proliferation • Possible roles for interferons and perfenidone