BILAG (British Isles Lupus Assessment Group index) Patient_______ Date_________

All features must be attributable to SLE and refer the last four weeks compared with the prior visits disease activity. Indicate and score which features are present: 0 = Not Present, 1 = Improving, 2 = Same, 3 = Worse, 4 = New or Recurrence.

General MUST BE SLE RELATED 1. Pyrexia (documented) 2. Weight Loss unintentional >5% 3. Lymphadenopathy/Splenomegaly 4. Fatigue/Malaise/Lethargy 5. Anorexia/nausea/vomiting Mucocutaneous - MUST BE SLE RELATED 6. Maculopapular eruption severe, active, (bullous) 7. Maculopapular eruption mild 8. Active discoid lesions generalized / extensive 9. Active discoid lesions localized including lupus profundus 10. Alopecia (severe, active) 11. Alopecia (mild) 12. Panniculitis (severe) 13. Angioedema 14. Extensive mucosal ulceration 15. Small mucosal ulcers 16. Malar erythema 17. Subcutaneous nodules 18. Perniotic Skin Lesions 19. Periungal erythema 20. Swollen fingers 21. Sclerodactyly 22. Calcinosis 23. Telangiectasia 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 0 0 0 1 1 1 1 2 2 2 2 3 3 3 3 4 4 4 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4

Neurological - MUST BE SLE RELATED 24. Deteriorating level of consciousness 25. Acute psychosis, delirium, confusion 26. Seizures 27. Stroke or stroke syndrome 28. Aseptic Meningitis 29. Mononeuritis multiplex 30. Ascending or transverse myelitis 31. Peripheral or cranial neuropathy 32. Disc swelling/cytoid bodies 33. Chorea 34. 35. 36. 37. Cerebellar ataxia Headache severe, unremitting Organic depressive illness Organic brain syndrome including Pseudotumor cerebri 38. Episodic migranous headaches Musculoskeletal - MUST BE SLE RELATED 39. Definite myositis (Bohan & Peter) 40. Severe Polyarthritis with loss of function 41. Arthritis 42. Tendonitis 43. Mild chronic myositis 44. Athralgia Yes Yes No No 45. Myalgia 46. Tendon contractures and fixed deformity 47. Aseptic necrosis 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 Yes Yes No No 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 0 0 0 1 1 1 1 2 2 2 2 3 3 3 3 4 4 4 4

0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 Yes No Yes No

0 1 2 3 4 0 1 2 3 4

Cardiovascular & Respiratory - MUST BE SLE RELATED 48. Pleuropericardial pain 49. Dyspnea 50. Cardiac Failure 51. Friction Rub 52. Effusion (pericardial or pleural) 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4

Renal - MUST BE SLE RELATED 68. Systolic Blood Pressure (Enter value) 69. Diastolic Blood Pressure (Enter value) 70. Accelerated Hypertension 71. Urine dipstick (Enter value) ( - = 0) (+ = 1) (++ = 2) (+++ = 3) 72. Urinary protein (Record a or b) a. 24 hr urinary protein b. Urine protein-creatine ratio 73. Proteinuria (Record a or b) a. Newly documented proteinuria of > 1g/24 hours b. Newly documented protein-creatine ratio of >100mg/mmol 74. Nephrotic Syndrome

() if SLE Relate d __________mm-Hg __________mm-Hg Yes No a _________ g b _______mm/mmol a. b. Yes Yes No No

53. Mild or intermittent chest pain

0 1 2 3 4 Yes OR Circle: No / Not Done Yes OR Circle No / Not Done Yes OR Circle No / Not Done Yes OR Circle No / Not Done Yes OR Circle No / Not Done Yes OR Circle No / Not Done 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 0 1 2 3 4 Yes No

54. Progressive CXR changes lung fields *If Not Done, NO on EDC BILAG

Yes

No

55. Progressive CXR changes heart size *If Not Done, NO on EDC BILAG

75. Creatinine (serum) (Enter value) ___________mg/dl

56. ECG evidence of pericarditis or Myocarditis *If Not Done, NO on EDC BILAG 57. Cardiac dysrhythmias including tachycardia >100 in the absence of fever *If Not Done, NO on EDC BILAG

76. Creatinine clearance/GFR (Enter value) __________ml/min

77. Active urinary sediment 78. Histological evidence of active Nephritis - within 3 months

Yes

No

58. Pulmonary function fall by 20% *If Not Done, NO on EDC BILAG

Yes 86. Evidence of circulating anticoagulant Yes Hematology - MUST BE SLE RELATED 79. Hemoglobin (g/dl) (Enter value) 9 80. Total white cell count (x 10 /L) (Enter value) 9 81. Neutrophils (x 10 /L) (Enter value) 9 82. Lymphocytes (x 10 /L) (Enter value) 9 83. Platelets (x 10 /L) (Enter value) 84. Evidence of active hemolysis 85. Coombs test positive 86. Evidence of circulating anticoagulant

No

59. Cytohistological evidence of inflammatory lung disease *If Not Done, NO on EDC BILAG Vascular - MUST BE SLE RELATED 60. Major cutaneous vasculitis incl. ulcers 61. Major abdominal crisis due to vasculitis 62. Recurrent thromboembolism excluding strokes 63. Raynauds 64. Livido reticularis 65. Superficial phlebitis 66. Minor cutaneous vasculitis (nailfold vascultitis, digital vasculitis) 67. Thromboembolism (excl. stroke) (first episode)

No

__________g/dl __________x 10 /L __________ x 10 /L __________ x 10 /L 9 __________ x 10 /L Yes No Yes Yes No No

9 9 9