Wegener's Granulomatosis Disease Activity Index - Medical Algorithm

Description

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.

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Description

Specialty

Objective

ICD-10

Disease Activity Index for Wegener's Granulomatosis

Purpose: To use the disease activity index of Kallenberg et al to evaluate a patient with Wegener's granulomatosis.

Description

Specialty

Objective

ICD-10