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