1. Pericarditis | (A) Clinical judgment and exudation verified by echocardiography, if ultrasound not available (B) Clinical criteria (1 required): Typical pericardial pain, peripheral oedema, dyspnoea/orthopnoea, ascites, dysrythmia (heart rate > 140/min, atrial flutter/fibrillation, 2–3 degrees atrioventricular block, ventricular tachycardia) Objective criteria compatible with pericarditis (1 required) Physical examination Cardiac catheterisation findings Histological examination Other causes improbable, such as tuberculosis or other infection, metastases, primary tumour, postoperative status or other trauma |
2. Pleuritis | Clinical judgment and exudation verified by x-ray examination Other causes improbable, such as tuberculosis or other infection, metastases, primary tumour, postoperative status or other trauma |
3. Felty’s syndrome | Splenomegaly (clinically evident or measured by ultrasound) and neutropenia (< 1.8 × 109/l) on two occasions Other causes improbable, such as drug side effect or infection |
4. Major cutaneous vasculitis | Diagnostic biopsy or clinical judgment by dermatologist |
5. Neuropathy | Clinical judgement by doctor and signs of polyneuropathy/mononeuropathy at electromyography/electroneurography |
6. Scleritis, episcleritis or retinal vasculitis | Clinical judgment by ophthalmologist |
7. Glomerulonephritis | Clinical judgment by nephrologist and positive biopsy |
8. Vasculitis affecting other organs | Clinical judgment by organ specialist and biopsy compatible with vasculitis |
9. Amyloidosis | Clinical judgment and positive biopsy from affected organ |
10. Keratoconjunctivitis sicca | Clinical judgment Positive Rose-Bengal staining or result of Schirmer’s test < 5 mm/min |
11. Xerostomia | Clinical judgment Abnormal sialometry, sialography, salivary scintigraphy or salivary gland biopsy with lymphocytic infiltrate |
12. Secondary Sjögren’s syndrome | Two of three criteria: Keratoconjunctivitis sicca (see above); Xerostomia (see above); Serological evidence: rheumatoid factor, ANA, anti-Ro (SSA), anti-La (SSB) positive, or hypergammaglobulinaemia |
13. Pulmonary fibrosis | Clinical judgment and Decreased vital capacity or carbon dioxide transfer factor by 15% from normal |
14. Bronchiolitis obliterans organising pneumonia | Clinical judgment by pulmonologist |
15. Cervical myelopathy | Clinical judgment Increased atlantoaxial movement — verified by x-ray examination |
16. Subcutaneous rheumatoid nodules | Clinical judgment |
17. Rheumatoid nodules in other locations | Positive biopsy |