BILAG-2004 INDEX Centre: Date: Initials/Hosp No:

 Only record manifestations/items due to SLE Disease Activity

 Assessment refers to manifestations occurring in the last 4 weeks (compared with the previous 4 weeks)
 TO BE USED WITH THE GLOSSARY
Record: ND Not Done CARDIORESPIRATORY
0 Not present 44. Myocarditis - mild ( )
1 Improving 45. Myocarditis/Endocarditis + Cardiac failure ( )
2 Same 46. Arrhythmia ( )
3 Worse 47. New valvular dysfunction ( )
4 New 48. Pleurisy/Pericarditis ( )
Yes/No OR Value (where indicated) 49. Cardiac tamponade ( )
Y/N Confirm this is due to SLE activity (Yes/No) 50. Pleural effusion with dyspnoea ( )
51. Pulmonary haemorrhage/vasculitis ( )
CONSTITUTIONAL 52. Interstitial alveolitis/pneumonitis ( )
1. Pyrexia - documented > 37.5ºC ( ) 53. Shrinking lung syndrome ( )
2. Weight loss - unintentional > 5% ( ) 54. Aortitis ( )
3. Lymphadenopathy/splenomegaly ( ) 55. Coronary vasculitis ( )
4. Anorexia ( )
GASTROINTESTINAL
MUCOCUTANEOUS 56. Lupus peritonitis ( )
5. Skin eruption - severe ( ) 57. Abdominal serositis or ascites ( )
6. Skin eruption - mild ( ) 58. Lupus enteritis/colitis ( )
7. Angio-oedema - severe ( ) 59. Malabsorption ( )
8. Angio-oedema - mild ( ) 60. Protein losing enteropathy ( )
9. Mucosal ulceration - severe ( ) 61. Intestinal pseudo-obstruction ( )
10. Mucosal ulceration - mild ( ) 62. Lupus hepatitis ( )
11. Panniculitis/Bullous lupus - severe ( ) 63. Acute lupus cholecystitis ( )
12. Panniculitis/Bullous lupus - mild ( ) 64. Acute lupus pancreatitis ( )
13. Major cutaneous vasculitis/thrombosis ( )
14. Digital infarcts or nodular vasculitis ( ) OPHTHALMIC
15. Alopecia - severe ( ) 65. Orbital inflammation/myositis/proptosis ( )
16. Alopecia - mild ( ) 66. Keratitis - severe ( )
17. Peri-ungual erythema/chilblains ( ) 67. Keratitis - mild ( )
18. Splinter haemorrhages ( ) 68. Anterior uveitis ( )
69. Posterior uveitis/retinal vasculitis - severe ( )
NEUROPSYCHIATRIC 70. Posterior uveitis/retinal vasculitis - mild ( )
19. Aseptic meningitis ( ) 71. Episcleritis ( )
20. Cerebral vasculitis ( ) 72. Scleritis - severe ( )
21. Demyelinating syndrome ( ) 73. Scleritis - mild ( )
22. Myelopathy ( ) 74. Retinal/choroidal vaso-occlusive disease ( )
23. Acute confusional state ( ) 75. Isolated cotton-wool spots (cytoid bodies) ( )
24. Psychosis ( ) 76. Optic neuritis ( )
25. Acute inflammatory demyelinating ( ) 77. Anterior ischaemic optic neuropathy ( )
polyradiculoneuropathy
26. Mononeuropathy (single/multiplex) ( ) RENAL
27. Cranial neuropathy ( ) 78. Systolic blood pressure (mm Hg) value ( ) Y/N
28. Plexopathy ( ) 79. Diastolic blood pressure (mm Hg) value ( ) Y/N
29. Polyneuropathy ( ) 80. Accelerated hypertension Yes/No ( )
30. Seizure disorder ( ) 81. Urine dipstick protein (+=1, ++=2, +++=3) ( ) Y/N
31. Status epilepticus ( ) 82. Urine albumin-creatinine ratio mg/mmol ( ) Y/N
32. Cerebrovascular disease (not due to vasculitis) ( ) 83. Urine protein-creatinine ratio mg/mmol ( ) Y/N
33. Cognitive dysfunction ( ) 84. 24 hour urine protein (g) value ( ) Y/N
34. Movement disorder ( ) 85. Nephrotic syndrome Yes/No ( )
35. Autonomic disorder ( ) 86. Creatinine (plasma/serum) mol/l ( ) Y/N
36. Cerebellar ataxia (isolated) ( )
87. GFR (calculated) ml/min/1.73 m2 ( ) Y/N
37. Lupus headache - severe unremitting ( )
88. Active urinary sediment Yes/No ( )
38. Headache from IC hypertension ( )
89. Active nephritis Yes/No ( )
MUSCULOSKELETAL
HAEMATOLOGICAL
39. Myositis - severe ( )
90. Haemoglobin (g/dl) value ( ) Y/N
40. Myositis - mild ( )
41. Arthritis ( severe) ( ) 91. Total white cell count (x 109/l) value ( ) Y/N
42. Arthritis (moderate)/Tendonitis/Tenosynovitis ( ) 92. Neutrophils (x 109/l) value ( ) Y/N
43. Arthritis (mild)/Arthralgia/Myalgia ( ) 93. Lymphocytes (x 109/l) value ( ) Y/N
94. Platelets (x 109/l) value ( ) Y/N
Weight (kg): Serum urea (mmol/l): 95. TTP ( )
African ancestry: Yes/No Serum albumin (g/l): 96. Evidence of active haemolysis Yes/No ( )
97. Coombs’ test positive (isolated) Yes/No ( )

Revision: 1/Sep/2009
 BILAG-2004 INDEX GLOSSARY

INSTRUCTIONS

 only record features that are attributable to SLE disease activity and not due to
damage, infection, thrombosis (in absence of inflammatory process) or other
conditions

 assessment refers to manifestations occurring in the last 4 weeks compared with the
previous 4 weeks

 activity refers to disease process which is reversible while damage refers to permanent
process/scarring (irreversible)

 damage due to SLE should be considered as a cause of features that are fixed/persistent
(SLICC/ACR damage index uses persistence  6 months to define damage)

 in some manifestations, it may be difficult to differentiate SLE from other conditions as

there may not be any specific test and the decision would then lies with the physician’s
judgement on the balance of probabilities

 ophthalmic manifestations usually need to be assessed by an ophthalmologist and these

items would need to be recorded after receiving the response from the ophthalmologist

 guidance for scoring:

(4) NEW
 manifestations are recorded as new when it is a new episode occurring in the last
4 weeks (compared to the previous 4 weeks) that has not improved and this
includes new episodes (recurrence) of old manifestations

 new episode occurring in the last 4 weeks but also satisfying the criteria for
improvement (below) would be classified as improving instead of new

(3) WORSE
 this refers to manifestations that have deteriorated/worsened significantly in the
last 4 weeks compared to the previous 4 weeks, sufficient for consideration of
increase in therapy

(2) SAME
 this refers to manifestations that have been present for the last 4 weeks and the
previous 4 weeks without significant improvement or deterioration (from the
previous 4 weeks)

 this also applies to manifestations that have improved over the last 4 weeks
compared to the previous 4 weeks but do not meet the criteria for improvement

(1) IMPROVING
 definition of improvement: (a) the amount of improvement is sufficient for

Revision: 1/Sep/2009 1
 consideration of reduction in therapy and
would not justify escalation in therapy

AND

(b) improvement must be present currently and

for at least 2 weeks out of the last 4 weeks

OR

manifestation that has completely resolved and

remained absent over the whole of last 1 week

(0) NOT PRESENT

(ND) NOT DONE

 it is important to indicate if a test has not been performed (particularly laboratory
investigations) so that this will be recorded as such in the database & not as
normal or absent (which is the default)

 INDICATE (TICK) IF NOT DUE TO SLE ACTIVITY

 for descriptors that are based on measurements (in renal and haematology
systems), it is important to indicate if these are not due to lupus disease activity
(for consideration of scoring) as they are usually recorded routinely into a
database

CHANGE IN SEVERITY CATEGORY

 there are several items in the index which have been divided into categories of
mild and severe (depending on definition). It is essential to record mild and
severe items appropriately if the manifestations fulfil both criteria during the last
4 weeks

 if a mild item deteriorated to the extent that it fulfilled the definition of severe
category (ie changed into severe category) within the last 4 weeks:
severe item scored as new (4)
AND mild item scored as worsening (3)

 if a severe item improved (fulfilling the improvement criteria) to the extent that it
no longer fulfilled the definition of severe category (ie changed into mild
category) within the last 4 weeks:
severe item scored as not present (0) if criteria for severe category has not
been met over last 4 weeks
or as improving (1) if criteria for severe category has been
met at some point over last 4 weeks

AND

mild item scored as improving (1) if it is improving over last 4 weeks

or as the same (2) if it has remained stable over last 4 weeks

Revision: 1/Sep/2009 2
CONSTITUTIONAL

1. Pyrexia temperature > 37.5˚C documented

2. Unintentional weight loss > 5%

3. Lymphadenopathy lymph node more than 1 cm diameter

exclude infection

4. Anorexia

MUCOCUTANEOUS

5. Severe eruption > 18% body surface area

any lupus rash except panniculitis, bullous lesion

& angio-oedema

body surface area (BSA) is estimated using the

rules of nines (used to assess extent of burns) as
follows:

palm(excluding fingers) = 1% BSA

each lower limb = 18% BSA
each upper limb = 9% BSA
torso (front) = 18% BSA
torso (back) = 18% BSA
head = 9% BSA
genital (male) = 1% BSA

6. Mild eruption ≤ 18% body surface area

Revision: 1/Sep/2009 3
 any lupus rash except panniculitis, bullous lesion
& angio-oedema

malar rash must have been observed by a

physician and has to be present continuously
(persistent) for at least 1 week to be considered
significant (to be recorded)

7. Severe angio-oedema potentially life-threatening eg: stridor

angio-oedema is a variant form of urticaria

which affects the subcutaneous, submucosal and
deep dermal tissues

8. Mild angio-oedema not life threatening

9. Severe mucosal ulceration disabling (significantly interfering with oral

intake), extensive & deep ulceration

must have been observed by a physician

10. Mild mucosal ulceration localised &/or non-disabling ulceration

11. Severe panniculitis or bullous lupus any one:

> 9% body surface area
facial panniculitis
panniculitis that is beginning to ulcerate
panniculitis that threatens integrity of
subcutaneous tissue (beginning to cause
surface depression) on > 9% body surface
area

panniculitis presents as a palpable and tender

subcutaneous induration/nodule

note that established surface depression and

atrophy alone is likely to be due to damage

12. Mild panniculitis or bullous lupus ≤ 9% body surface area

does not fulfil any criteria for severe panniculitis
(for panniculitis)

13. Major cutaneous vasculitis/thrombosis resulting in extensive gangrene or ulceration or

skin infarction

14. Digital infarct or nodular vasculitis localised single or multiple infarct(s) over
digit(s) or tender erythematous nodule(s)

15. Severe alopecia clinically detectable (diffuse or patchy) hair loss

with scalp inflammation (redness over scalp)

Revision: 1/Sep/2009 4
16. Mild alopecia diffuse or patchy hair loss without scalp
inflammation (clinically detectable or by history)

17. Peri-ungual erythema or chilblains chilblains are localised inflammatory lesions

(may ulcerate) which are precipitated by
exposure to cold

18. Splinter haemorrhages

NEUROPSYCHIATRIC

19. Aseptic meningitis criteria (all): acute/subacute onset

headache
fever
abnormal CSF (raised protein &/or
lymphocyte predominance) but negative
cultures

preferably photophobia, neck stiffness and

meningeal irritation should be present as well but
are not essential for diagnosis

exclude CNS/meningeal infection, intracranial

haemorrhage

20. Cerebral vasculitis should be present with features of vasculitis

in another system

supportive imaging &/or biopsy findings

21. Demyelinating syndrome discrete white matter lesion with associated

neurological deficit not recorded elsewhere

ideally there should have been at least one

previously recorded event

supportive imaging required

exclude multiple sclerosis

22. Myelopathy acute onset of rapidly evolving paraparesis or

quadriparesis and/or sensory level

exclude intramedullary and extramedullary

space occupying lesion

23. Acute confusional state acute disturbance of consciousness or level of

arousal with reduced ability to focus, maintain or
shift attention

Revision: 1/Sep/2009 5
 includes hypo- and hyperaroused states and
encompasses the spectrum from delirium to
coma

24. Psychosis delusion or hallucinations

does not occur exclusively during course of a

delirium

exclude drugs, substance abuse, primary

psychotic disorder

25. Acute inflammatory demyelinating criteria:

polyradiculoneuropathy progressive polyradiculoneuropathy
loss of reflexes
symmetrical involvement
increased CSF protein without pleocytosis
supportive electrophysiology study

26. Mononeuropathy (single/multiplex) supportive electrophysiology study required

27. Cranial neuropathy except optic neuropathy which is classified

under ophthalmic system

28. Plexopathy disorder of brachial or lumbosacral plexus

resulting in neurological deficit not
corresponding to territory of single root or nerve

supportive electrophysiology study required

29. Polyneuropathy acute symmetrical distal sensory and/or motor

deficit

supportive electrophysiology study required

30. Seizure disorder independent description of seizure by reliable

witness

31. Status epilepticus a seizure or series of seizures lasting ≥ 30

minutes without full recovery to baseline

32. Cerebrovascular disease any one with supporting imaging:

(not due to vasculitis) stroke syndrome
transient ischaemic attack
intracranial haemorrhage

exclude hypoglycaemia, cerebral sinus

thrombosis, vascular malformation, tumour,
abscess

cerebral sinus thrombosis not included as

Revision: 1/Sep/2009 6
 definite thrombosis not considered part of lupus
activity

33. Cognitive dysfunction significant deficits in any cognitive functions:

simple attention (ability to register & maintain
information)
complex attention
memory (ability to register, recall & recognise
information eg learning, recall)
visual-spatial processing (ability to analyse,
synthesise & manipulate visual-spatial
information)
language (ability to comprehend, repeat &
produce oral/written material eg verbal
fluency, naming)
reasoning/problem solving (ability to reason &
abstract)
psychomotor speed
executive functions (eg planning, organising,
sequencing)

in absence of disturbance of consciousness or

level of arousal

sufficiently severe to interfere with daily

activities

neuropsychological testing should be done or

corroborating history from third party if possible

exclude substance abuse

34. Movement disorder exclude drugs

35. Autonomic disorder any one:

fall in blood pressure to standing > 30/15 mm
Hg (systolic/diastolic)

increase in heart rate to standing ≥ 30 bpm

loss of heart rate variation with respiration

(max – min < 15 bpm, expiration:inspiration
ratio < 1.2, Valsalva ratio < 1.4)

loss of sweating over body and limbs

(anhidrosis) by sweat test

exclude drugs and diabetes mellitus

36. Cerebellar ataxia cerebellar ataxia in isolation of other CNS

features

Revision: 1/Sep/2009 7
 usually subacute presentation

37. Severe lupus headache (unremitting) disabling headache unresponsive to narcotic

analgesia & lasting ≥ 3 days

exclude intracranial space occupying lesion

and CNS infection

38. Headache from IC hypertension exclude cerebral sinus thrombosis

MUSCULOSKELETAL

39. Severe myositis significantly elevated serum muscle enzymes

with significant muscle weakness

exclude endocrine causes and drug-induced

myopathy

electromyography and muscle biopsy are used

for diagnostic purpose and are not required to
determine level of activity

40. Mild myositis significantly elevated serum muscle enzymes

with myalgia but without significant muscle
weakness

asymptomatic elevated serum muscle enzymes

not included

exclude endocrine causes and drug-induced

myopathy

electromyography and muscle biopsy are used

for diagnostic purpose and are not required to
determine level of activity

41. Severe arthritis observed active synovitis ≥ 2 joints with marked

loss of functional range of movements and
significant impairment of activities of daily
living, that has been present on several days
(cumulatively) over the last 4 weeks

42. Moderate arthritis or Tendonitis tendonitis/tenosynovitis or active synovitis ≥ 1

or Tenosynovitis joint (observed or through history) with some
loss of functional range of movements, that has
been present on several days over the last 4
weeks

43. Mild arthritis or Arthralgia or Myalgia inflammatory type of pain (worse in the morning
with stiffness, usually improves with activity &
not brought on by activity) over joints/muscle

Revision: 1/Sep/2009 8
 inflammatory arthritis which does not fulfil the
above criteria for moderate or severe arthritis

CARDIORESPIRATORY

44. Mild myocarditis inflammation of myocardium with raised

cardiac enzymes &/or ECG changes and without
resulting cardiac failure, arrhythmia or valvular
dysfunction

45. Cardiac failure cardiac failure due to myocarditis or non-

infective inflammation of endocardium or
cardiac valves (endocarditis)

cardiac failure due to myocarditis is defined by

left ventricular ejection fraction ≤ 40% &
pulmonary oedema or peripheral oedema

cardiac failure due to acute valvular regurgitation

(from endocarditis) can be associated with
normal left ventricular ejection fraction

diastolic heart failure is not included

46. Arrhythmia arrhythmia (except sinus tachycardia) due to

myocarditis or non-infective inflammation of
endocardium or cardiac valves (endocarditis)

confirmation by electrocardiogram required

(history of palpitations alone inadequate)

47. New valvular dysfunction new cardiac valvular dysfunction due to

myocarditis or non-infective inflammation of
endocardium or cardiac valves (endocarditis)

supportive imaging required

48. Pleurisy/Pericarditis convincing history &/or physical findings that

you would consider treating

in absence of cardiac tamponade or pleural

effusion with dyspnoea

do not score if you are unsure whether or not it is

pleurisy/pericarditis

49. Cardiac tamponade supportive imaging required

50. Pleural effusion with dyspnoea supportive imaging required
51. Pulmonary haemorrhage/vasculitis inflammation of pulmonary vasculature with

Revision: 1/Sep/2009 9
 haemoptysis &/or dyspnoea &/or pulmonary
hypertension

supportive imaging &/or histological diagnosis

required

52. Interstitial alveolitis/pneumonitis radiological features of alveolar infiltration not

due to infection or haemorrhage required for
diagnosis

corrected gas transfer Kco reduced to < 70%

normal or fall of > 20% if previously abnormal

on-going activity would be determined by

clinical findings and lung function tests, and
repeated imaging may be required in those with
deterioration (clinically or lung function tests) or
failure to respond to therapy

53. Shrinking lung syndrome acute reduction (> 20% if previous measurement
available) in lung volumes (to < 70% predicted)
in the presence of normal corrected gas transfer
(Kco) & dysfunctional diaphragmatic
movements

54. Aortitis inflammation of aorta (with or without

dissection) with supportive imaging
abnormalities

accompanied by > 10 mm Hg difference in BP

between arms &/or claudication of extremities
&/or vascular bruits

repeated imaging would be required to determine

on-going activity in those with clinical
deterioration or failure to respond to therapy

55. Coronary vasculitis inflammation of coronary vessels with

radiographic evidence of non-atheromatous
narrowing, obstruction or aneurysmal changes
GASTROINTESTINAL

56. Lupus peritonitis serositis presenting as acute abdomen with

rebound/guarding

57. Serositis not presenting as acute abdomen

58. Lupus enteritis or colitis vasculitis or inflammation of small or large

bowel with supportive imaging &/or biopsy
findings

59. Malabsorption diarrhoea with abnormal D- xylose absorption

Revision: 1/Sep/2009 10
 test or increased faecal fat excretion after
exclusion of coeliac’s disease (poor response to
gluten-free diet) and gut vasculitis

60. Protein-losing enteropathy diarrhoea with hypoalbuminaemia or increased

faecal excretion of iv radiolabeled albumin after
exclusion of gut vasculitis and malabsorption

61. Intestinal pseudo-obstruction subacute intestinal obstruction due to intestinal

hypomotility

62. Lupus hepatitis raised transaminases

absence of autoantibodies specific to

autoimmune hepatitis (eg: anti-smooth muscle,
anti-liver cytosol 1) &/or biopsy appearance of
chronic active hepatitis

hepatitis typically lobular with no piecemeal

necrosis

exclude drug-induced and viral hepatitis

63. Acute lupus cholecystitis after exclusion of gallstones and infection

64. Acute lupus pancreatitis usually associated multisystem involvement

OPHTHALMIC

65. Orbital inflammation orbital inflammation with myositis &/or extra-

ocular muscle swelling &/or proptosis

supportive imaging required

66. Severe keratitis sight threatening

includes: corneal melt
peripheral ulcerative keratitis

67. Mild keratitis not sight threatening

68. Anterior uveitis

69. Severe posterior uveitis &/or retinal sight-threatening &/or retinal vasculitis
vasculitis not due to vaso-occlusive disease

70. Mild posterior uveitis &/or retinal not sight-threatening

vasculitis
not due to vaso-occlusive disease

71. Episcleritis
72. Severe scleritis necrotising anterior scleritis

Revision: 1/Sep/2009 11
 anterior &/or posterior scleritis requiring
systemic steroids/immunosuppression &/or not
responding to NSAIDs

73. Mild scleritis anterior &/or posterior scleritis not requiring

systemic steroids

excludes necrotising anterior scleritis

74. Retinal/choroidal vaso-occlusive includes: retinal arterial & venous occlusion

disease serous retinal &/or retinal pigment
epithelial detachments secondary to
choroidal vasculopathy

75. Isolated cotton-wool spots also known as cytoid bodies

76. Optic neuritis excludes anterior ischaemic optic neuropathy

77. Anterior ischaemic optic neuropathy visual loss with pale swollen optic disc due to
occlusion of posterior ciliary arteries

RENAL

78. Systolic blood pressure

79. Diastolic blood pressure
80. Accelerated hypertension blood pressure rising to > 170/110 mm Hg
within 1 month with grade 3 or 4 Keith-
Wagener-Barker retinal changes (flame-shaped
haemorrhages or cotton-wool spots or
papilloedema)

81. Urine dipstick

82. Urine albumin-creatinine ratio on freshly voided urine sample

conversion: 1 mg/mg = 113 mg/mmol

it is important to exclude other causes (especially
infection) when proteinuria is present

83. Urine protein-creatinine ratio on freshly voided urine sample

conversion: 1 mg/mg = 113 mg/mmol

it is important to exclude other causes (especially

infection) when proteinuria is present

84. 24 hour urine protein it is important to exclude other causes (especially

infection) when proteinuria is present

85. Nephrotic syndrome criteria:

heavy proteinuria (  3.5 g/day or protein-

Revision: 1/Sep/2009 12
 creatinine ratio  350 mg/mmol or albumin-
creatinine ratio  350 mg/mmol)

hypoalbuminaemia
oedema

86. Plasma/Serum creatinine exclude other causes for increase in creatinine

(especially drugs)

87. GFR MDRD formula:

GFR = 170 x [serum creatinine (mg/dl)]-0.999 x
[age]-0.176 x [serum urea (mg/dl]-0.17 x
[serum albumin (g/dl)]0.318 x [0.762 if
female] x [1.180 if African ancestry]

units = ml/min per 1.73 m2

normal: male = 130 ± 40
female = 120 ± 40

conversion:
serum creatinine - mg/dl = (mol/l)/88.5
serum urea - mg/dl = (mmol/l) x 2.8
serum albumin - g/dl = (g/l)/10

creatinine clearance not recommended as it is not

reliable

exclude other causes for decrease in GFR

(especially drugs)

88. Active urinary sediment pyuria (> 5 WCC/hpf or > 10 WCC/mm3 (l))

OR

haematuria (> 5 RBC/hpf or > 10 RBC/mm3 (l))

OR

red cell casts

OR

white cell casts

exclude other causes (especially infection,

vaginal bleed, calculi)

89. Histology of active nephritis WHO Classification (1995): (any one)

Class III – (a) or (b) subtypes
Class IV – (a), (b) or (c) subtypes
Class V – (a), (b), (c) or (d) subtypes
Vasculitis

Revision: 1/Sep/2009 13
 OR

ISN/RPS Classification (2003): (any one)

Class III – (A) or (A/C) subtypes
Class IV – (A) or (A/C) subtypes
Class V
Vasculitis

within last 3 months

glomerular sclerosis without inflammation not

included

HAEMATOLOGICAL

90. Haemoglobin exclude dietary deficiency & GI blood loss

91. White cell count exclude drug-induced cause
92. Neutrophil count exclude drug-induced cause
93. Lymphocyte count
94. Platelet count exclude drug-induced cause
95. TTP thrombotic thrombocytopaenic purpura

clinical syndrome of micro-angiopathic

haemolytic anaemia and thrombocytopenia in
absence of any other identifiable cause

96. Evidence of active haemolysis positive Coombs’ test & evidence of haemolysis
(raised bilirubin or raised reticulocyte count or
reduced haptoglobulins or fragmented RBC or
microspherocytes)

97. Isolated positive Coombs’ test

ADDITIONAL ITEMS

These items are required mainly for calculation of GFR

i. Weight
ii. African ancestry
iii. Serum urea
iv. Serum albumin

Revision: 1/Sep/2009 14
References:

1) Rule of nines diagram. Burn Center, University of Utah Health Sciences Center
(http://uuhsc.utah.edu/burncenter/emergencycare/extent.html)

2) Levey, A. S., Bosch, J. P., Lewis, J. B., Greene, T., Rogers, N., & Roth, D. A more
accurate method to estimate glomerular filtration rate from serum creatinine: a new prediction
equation. Modification of Diet in Renal Disease Study Group. Ann.Intern.Med. 1999; 130(6):
461-470.

3) Weening, J. J., D'Agati, V. D., Schwartz, M. M., Seshan, S. V., Alpers, C. E., Appel, G. B.,
Balow, J. E., Bruijn, J. A., Cook, T., Ferrario, F., Fogo, A. B., Ginzler, E. M., Hebert, L., Hill,
G., Hill, P., Jennette, J. C., Kong, N. C., Lesavre, P., Lockshin, M., Looi, L. M., Makino, H.,
Moura, L. A., & Nagata, M. The classification of glomerulonephritis in systemic lupus
erythematosus revisited. J.Am.Soc.Nephrol. 2004; 15(2): 241-250.

Revision: 1/Sep/2009 15
 BILAG-2004 INDEX SCORING
 scoring based on the principle of physician’s intention to treat

Category Definition

A Severe disease activity requiring any of the following treatment:

1. systemic high dose oral glucocorticoids (equivalent to prednisolone > 20

mg/day)

2. intravenous pulse glucocorticoids (equivalent to pulse methylprednisolone

≥ 500 mg)

3. systemic immunomodulators (include biologicals, immunoglobulins and

plasmapheresis)

4. therapeutic high dose anticoagulation in the presence of high dose steroids

or immunomodulators
eg: warfarin with target INR 3 - 4

B Moderate disease activity requiring any of the following treatment:

1. systemic low dose oral glucocorticoids (equivalent to prednisolone ≤ 20

mg/day)

2. intramuscular or intra-articular or soft tissue glucocorticoids injection

(equivalent to methylprednisolone < 500mg)

3. topical glucocorticoids
4. topical immunomodulators
5. antimalarials or thalidomide or prasterone or acitretin
6. symptomatic therapy
eg: NSAIDs for inflammatory arthritis

C Mild disease

D Inactive disease but previously affected

E System never involved

Revision: 1/Sep/2009 1
CONSTITUTIONAL

Category A:
Pyrexia recorded as 2 (same), 3 (worse) or 4 (new) AND

Any 2 or more of the following recorded as 2 (same), 3 (worse) or 4 (new):

Weight loss
Lymphadenopathy/splenomegaly
Anorexia

Category B:
Pyrexia recorded as 2 (same), 3 (worse) or 4 (new) OR

Any 2 or more of the following recorded as 2 (same), 3 (worse) or 4 (new):

Weight loss
Lymphadenopathy/splenomegaly
Anorexia

BUT do not fulfil criteria for Category A

Category C
Pyrexia recorded as 1 (improving) OR

One or more of the following recorded as > 0:

Weight loss
Lymphadenopathy/Splenomegaly
Anorexia

BUT does not fulfil criteria for category A or B

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 2
MUCOCUTANEOUS

Category A
Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Skin eruption - severe

Angio-oedema - severe
Mucosal ulceration - severe
Panniculitis/Bullous lupus - severe
Major cutaneous vasculitis/thrombosis

Category B
Any Category A features recorded as 1 (improving) OR

Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Skin eruption - mild

Panniculitis/Bullous lupus - mild
Digital infarcts or nodular vasculitis
Alopecia - severe

Category C
Any Category B features recorded as 1 (improving) OR

Any of the following recorded as > 0:

Angio-oedema - mild
Mucosal ulceration - mild
Alopecia - mild
Periungual erythema/chilblains
Splinter haemorrhages

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 3
NEUROPSYCHIATRIC

Category A
Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Aseptic meningitis
Cerebral vasculitis
Demyelinating syndrome
Myelopathy
Acute confusional state
Psychosis
Acute inflammatory demyelinating polyradiculoneuropathy
Mononeuropathy (single/multiplex)
Cranial neuropathy
Plexopathy
Polyneuropathy
Status epilepticus
Cerebellar ataxia

Category B
Any Category A features recorded as 1 (improving) OR

Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Seizure disorder
Cerebrovascular disease (not due to vasculitis)
Cognitive dysfunction
Movement disorder
Autonomic disorder
Lupus headache - severe unremitting
Headache due to raised intracranial hypertension

Category C
Any Category B features recorded as 1 (improving)

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 4
MUSCULOSKELETAL

Category A
Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Severe Myositis
Severe Arthritis

Category B
Any Category A features recorded as 1 (improving) OR

Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Mild Myositis
Moderate Arthritis/Tendonitis/Tenosynovitis

Category C
Any Category B features recorded as 1 (improving) OR

Any of the following recorded as > 0:

Mild Arthritis/Arthralgia/Myalgia

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 5
CARDIORESPIRATORY

Category A
Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Myocarditis/Endocarditis + Cardiac failure

Arrhythmia
New valvular dysfunction
Cardiac tamponade
Pleural effusion with dyspnoea
Pulmonary haemorrhage/vasculitis
Interstitial alveolitis/pneumonitis
Shrinking lung syndrome
Aortitis
Coronary vasculitis

Category B
Any Category A features recorded as 1 (improving) OR

Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Pleurisy/Pericarditis
Myocarditis - mild

Category C
Any Category B features recorded as 1 (improving)

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 6
GASTROINTESTINAL

Category A
Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Peritonitis
Lupus enteritis/colitis
Intestinal pseudo-obstruction
Acute lupus cholecystitis
Acute lupus pancreatitis

Category B
Any Category A feature recorded as 1 (improving) OR

Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Abdominal serositis and/or ascites

Malabsorption
Protein losing enteropathy
Lupus hepatitis

Category C
Any Category B features recorded as 1 (improving)

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 7
OPHTHALMIC

Category A
Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Orbital inflammation/myositis/proptosis
Keratitis - severe
Posterior uveitis/retinal vasculitis - severe
Scleritis - severe
Retinal/choroidal vaso-occlusive disease
Optic neuritis
Anterior ischaemic optic neuropathy

Category B
Any Category A features recorded as 1 (improving) OR

Any of the following recorded as 2 (same), 3 (worse) or 4 (new):

Keratitis - mild
Anterior uveitis
Posterior uveitis/retinal vasculitis - mild
Scleritis - mild

Category C
Any Category B features recorded as 1 (improving) OR

Any of the following recorded as > 0:

Episcleritis
Isolated cotton-wool spots (cytoid bodies)

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 8
RENAL

Category A
Two or more of the following providing 1, 4 or 5 is included:

1. Deteriorating proteinuria (severe) defined as

(a) urine dipstick increased by ≥ 2 levels (used only if other methods of urine protein estimation not
available); or

(b) 24 hour urine protein > 1 g that has not decreased (improved) by  25%; or
(c) urine protein-creatinine ratio > 100 mg/mmol that has not decreased (improved) by  25%; or
(d) urine albumin-creatinine ratio > 100 mg/mmol that has not decreased (improved) by  25%

2. Accelerated hypertension
3. Deteriorating renal function (severe) defined as

(a) plasma creatinine > 130 mol/l and having risen to > 130% of previous value; or
(b) GFR < 80 ml/min per 1.73 m2 and having fallen to < 67% of previous value; or
(c) GFR < 50 ml/min per 1.73 m2, and last time was > 50 ml/min per 1.73 m2 or was not measured.

4. Active urinary sediment

5. Histological evidence of active nephritis within last 3 months
6. Nephrotic syndrome

Category B
One of the following:

1. One of the Category A feature

2. Proteinuria (that has not fulfilled Category A criteria)

(a) urine dipstick which has risen by 1 level to at least 2+ (used only if other methods of urine
protein estimation not available); or

(b) 24 hour urine protein ≥ 0.5 g that has not decreased (improved) by  25%; or
(c) urine protein-creatinine ratio ≥ 50 mg/mmol that has not decreased (improved) by  25%; or
(d) urine albumin-creatinine ratio ≥ 50 mg/mmol that has not decreased (improved) by  25%

3. Plasma creatinine > 130 mol/l and having risen to ≥ 115% but ≤ 130% of previous value

Category C
One of the following:

1. Mild/Stable proteinuria defined as

(a) urine dipstick ≥ 1+ but has not fulfilled criteria for Category A & B (used only if other methods
of urine protein estimation not available); or
Revision: 1/Sep/2009 9
 (b) 24 hour urine protein > 0.25 g but has not fulfilled criteria for Category A & B ; or
(c) urine protein-creatinine ratio > 25 mg/mmol but has not fulfilled criteria for Category A & B; or
(d) urine albumin-creatinine ratio > 25 mg/mmol but has not fulfilled criteria for Category A & B

2. Rising blood pressure (providing the recorded values are > 140/90 mm Hg) which has not fulfilled
criteria for Category A & B, defined as

(a) systolic rise of ≥ 30 mm Hg; and

(b) diastolic rise of ≥ 15mm Hg

Category D
Previous involvement

Category E
No previous involvement

Note: although albumin-creatinine ratio and protein-creatinine ratio are different, we use the same cut-
off values for this index

Revision: 1/Sep/2009 10
HAEMATOLOGICAL

Category A
TTP recorded as 2 (same), 3 (worse) or 4 (new) OR

Any of the following:

Evidence of haemolysis and Haemoglobin < 8 g/dl

Platelet count < 25 x 109/l

Category B
TTP recorded as 1 (improving) OR

Any of the following:

Evidence of haemolysis and Haemoglobin 8 - 9.9 g/dl

Haemoglobin < 8 g/dl (without haemolysis)
White cell count < 1.0 x 109/l
Neutrophil count < 0.5 x 109/l
Platelet count 25 - 49 x 109/l

Category C
Any of the following:

Evidence of haemolysis and Haemoglobin ≥ 10g/dl

Haemoglobin 8 - 10.9 g/dl (without haemolysis)
White cell count 1 - 3.9 x 109/l
Neutrophil count 0.5 - 1.9 x 109/l
Lymphocyte count < 1.0 x 109/L
Platelet count 50 - 149 x 109/l
Isolated Coombs’ test positive

Category D
Previous involvement

Category E
No previous involvement

Revision: 1/Sep/2009 11