Kallenberg et al proposed a Disease Activity Index for patients with Wegener's granulomatosis. The authors are from The Netherlands.
Symptom groups:
(1) ENT
(2) Pulmonary
(3) Renal
(4) Central Nervous System and Eyes
(5) Cardiovascular
(6) Gastrointestinal
(7) Musculoskeletal
(8) Skin
(9) Miscellaneous
NOTE: The description of the index gives point assignments, but this is open to interpretation:
(1) No mention of a maximum value for a symptom group is given. I have assumed that it is the sum of the subgroupings.
(2) Any clustering of findings with only one value returned is not specified. The tables below reflect what I think makes sense if some clustering is assumed.
ENT Symptoms |
Finding |
Points |
---|---|---|
sinusitis and/or sanguinous nasal discharge |
ulceration and/or proliferative mass on endoscopy and histologic pattern of granulomatous inflammation on biopsy |
5 |
|
ulceration and/or proliferative mass on endoscopy and histologic pattern of necrotizing inflammation without granulomas on biopsy |
3 |
|
absent |
0 |
ear |
serous otitis and/or otorrhea |
2 |
|
absent |
0 |
buccal ulceration |
present |
1 |
|
absent |
0 |
trachea |
tracheal stenosis with severe dyspnea |
8 |
|
tracheal stenosis without severe dyspnea |
3 |
|
absent |
0 |
ENT symptom subscore =
= (points for nasal or sinus findings) + (points for ear findings) + (points for buccal ulceration) + (points for trachea)
maximum subscore: 16
Pulmonary Symptoms |
Finding |
Points |
---|---|---|
respiratory change |
pulmonary infiltrates on chest X-ray with impending respiratory insufficiency with or without hemoptysis |
10 |
|
pulmonary infiltrates on chest X-ray |
4 |
|
absent |
0 |
pulmonary symptom subscore =
= (points for respiratory change)
maximum subscore: 10
Renal Symptoms |
Finding |
Points |
---|---|---|
creatinine clearance and red cells in urine sediment |
decrease in creatinine clearance > 30% within a period of 3 months with erythrocyturia |
10 |
|
decrease in creatinine clearance <= 30% within a period of 3 months with erythrocyturia |
4 |
|
no change in creatinine clearance with > 15 erythrocytes per high power field in urinary sediment on more than 1 occasion |
2 |
|
absent |
0 |
cellular casts in urine sediment |
present on more than 1 occasion |
3 |
|
absent or present on 1 occasion |
0 |
renal symptom subscore =
= (points for creatinine clearance and erythrocyturia) + (points for cellular casts)
maximum subscore: 13
Nervous System and Eye |
Finding |
Points |
---|---|---|
vasculitis |
cerebral vasculitis |
10 |
|
absent |
0 |
mononeuritis multiplex |
with paresis and sensory loss |
8 |
|
without paresis |
3 |
|
absent |
0 |
cranial nerve palsy |
present |
8 |
|
absent |
0 |
ocular change |
corneal ulceration |
2 |
|
episcleritis |
2 |
|
necrotizing scleritis |
8 |
|
absent |
0 |
orbital pseudotumor |
present |
8 |
|
absent |
0 |
recurrent dacrocystitis |
present |
1 |
|
absent |
0 |
nervous system and eye subscore =
= (points for vasculitis) + (points for mononeuritis multiplex) + (points for cranial nerve palsy) + (points for ocular change) + (points for orbital pseudotumor) + (points for recurrent dacrocystitis)
maximum subscore: 43
Cardiovascular |
Finding |
Points |
---|---|---|
heart |
myocardial infarction due to vasculitis |
10 |
|
myocardial infarction negative or due to other cause |
0 |
extremities |
peripheral gangrene |
10 |
|
absent |
0 |
cardiovascular subscore =
= (points for heart) + (points for extremities)
maximum subscore: 20
Gastrointestinal |
Finding |
Points |
---|---|---|
GI change |
acute abdomen with intestinal perforation due to vasculitis |
10 |
|
massive hemorrhage due to vasculitis |
10 |
|
neither |
0 |
gastrointestinal subscore =
= (points for GI change)
maximum subscore: 10 (this could be 20 if assume separate)
Musculoskeletal |
Finding |
Points |
---|---|---|
joint findings |
arthritis of 1 or more joints |
3 |
|
arthralgia of 1 or more joints |
2 |
|
neither |
0 |
musculoskeletal subscore =
= (points for joint findings)
maximum subscore: 3
Skin |
Finding |
Points |
---|---|---|
skin |
ulceration and/or purpura with histologically granulomatous inflammation and/or necrotizing vasculitis |
3 |
|
ulceration |
2 |
|
purpura |
1 |
|
neither |
0 |
skin subscore =
= (points for skin finding)
maximum subscore: 3
Miscellaneous |
Finding |
Points |
---|---|---|
biopsy histology |
any manifestation with evidence of granulomatous inflammation and/or necrotizing vasculitis |
3 |
|
no evidence of granulomatous inflammation and/or necrotizing vasculitis |
0 |
fever of unknown origin for > 1 week |
> 38.5°C rectally |
3 |
|
> 38.0 – 38.5°C |
1 |
|
none or <= 38.0°C rectally |
0 |
C-reactive protein |
> 100 mg/L increase |
3 |
|
50.1 – 100 mg/L increase |
2 |
|
20.1 – 50 mg/L increase |
1 |
|
<= 20 mg/L increase |
0 |
miscellaneous subscore =
= (points for biopsy) + (points for FUO) + (points for C-reactive protein increase)
maximum subscore: 9
disease activity index =
= SUM(points for each of the 9 subscores)
Interpretation:
• minimum disease activity index: 0
• maximum disease index (with above groupings): 127
• A single condition scoring 5 or more points requires treatment with intermediate doses of corticosteroids (30 mg/day prednisolone) and cyclosphosphamide (75 mg/day).
• A minor exacerbation is defined as (a) an increase in the total index >= 5 points within a 3 month period and (b) not meeting criteria for a major exacerbation, as described in the previous section.
• Other diseases causing the findings need to be excluded before inferring Wegener's disease activity.
Purpose: To use the disease activity index of Kallenberg et al to evaluate a patient with Wegener's granulomatosis.
Specialty: Immunology/Rheumatology
Objective: severity, prognosis, stage, disease progression
ICD-10: M31.3,