Carruthers et al reported a responder index for patients with IgG4-related disease (IgG4-RD). This can help to identify a patient who may benefit from more aggressive management. The authors are from Massachusetts General Hospital.
Patient selection: IgG4-related disease
Organ/site involvement over the past 28 days:
(1) pachymeninges
(2) pituitary gland
(3) orbits and lacrimal glands
(4) salivary glands
(5) thyroid gland
(6) lymph nodes
(7) lungs
(8) aorta and large blood vessels
(9) retroperitoneum, mediastinum and mesentery
(10) pancreas
(11) bile ducts and liver
(12) kidney
(13) skin
(14) other sclerosis or mass formation
|
Status
|
Points
|
|
normal or resolved
|
0
|
|
improved
|
1
|
|
persistent, unchanged
|
2
|
|
new or recurrent
|
3
|
|
worse despite treatment
|
4
|
Each site is evaluated for urgent disease (need for immediate treatment to prevent serious organ damage) as Yes or No. If present then the points for site involvement status are doubled (multiplied by 2). Urgency probably does not apply to sites that are normal, resolved or improved.
Each site is evaluated to see if the patient is symptomatic. These should match all sites of involvement that are not normal or resolved.
Each site is evaluated for damage, which is designated as Yes or No. The total number of damaged organs is tallied.
The serum IgG4 concentration is graded from 0 to 4 similar to the organ involvement.
The final assessment is the cumulative corticosteroid dose in the past 28 days as mg prednisone equivalent.
total activity score =
= SUM(organ activity score for all 14 organs) + (score for serum IgG4)
Interpretation:
• minimum score: 0
• maximum score: 116
• The higher the score the greater the disease activity.
