The American College of Rheumatology (ACR) and the European Union Against Rheumatism (EULAR) published classification criteria for IgG4-related disease. These included both inclusion and exclusion criteria.
Parameters:
(1) histopathology
(2) serum IgG4 concentration as multiple of upper limit of normal (ULN)
(3) bilateral glands (lacrimal, parotid, sublingual, submandibular)
(4) chest
(5) pancreas and biliary tract
(6) kidney
(7) retroperitoneum
|
Parameter |
Finding |
Points |
|
histopathology |
uninformative biopsy |
0 |
|
|
dense lymphocytic infiltrate |
4 |
|
|
dense lymphocytic infiltrate and obliterative phlebitis |
6 |
|
|
dense lymphocytic infiltrate with storiform fibrosis |
13 |
|
|
IgG4+ cells 0 to 9 per hpf; IgG4+ to IgG ratio <= 40% |
0 |
|
|
IgG4+ cells 0 to 9 per hpf; IgG4+ to IgG ratio > 40% |
7 |
|
|
IgG4+ cells >= 10 per hpf; IgG4+ to IgG ratio <= 40% |
7 |
|
|
IgG4+ cells >= 10 per hpf; IgG4+ to IgG ratio 41 to 70% |
14 |
|
|
IgG4+ cells 10 to 50 per hpf; IgG4+ to IgG ratio > 70% |
14 |
|
|
IgG4+ cells > 50 per hpf; IgG4+ to IgG ratio > 70% |
16 |
|
serum IgG4 |
not checked or normal |
0 |
|
|
1.01 to 1.99 times ULN |
4 |
|
|
2 to 5 times ULN |
6 |
|
|
> 5 times ULN |
11 |
|
bilateral glands |
no set involved |
0 |
|
|
one set of glands involved |
6 |
|
|
2 or more sets involved |
14 |
|
chest |
not checked or neither of the items below present |
0 |
|
|
peribronchiovascular and septal thickening |
4 |
|
|
paravertebral band-like soft tissue in the thorax |
10 |
|
pancreas or biliary tract |
not checked or none of items present |
0 |
|
|
diffuse pancreatic enlargement with loss of lobulation |
8 |
|
|
diffuse pancreatic enlargement with capsule-like rim with decreased enhancement |
11 |
|
|
pancreas and biliary tree involved |
19 |
|
kidney |
not checked or none of items present |
0 |
|
|
hypocomplementemia |
6 |
|
|
renal pelvis thickening, soft tissue |
8 |
|
|
bilateral renal cortex low-density areas |
10 |
|
retroperitoneum |
not checked or none of items present |
0 |
|
|
diffuse thickening of abdominal aortic wall |
4 |
|
|
circumferential or anterolateral soft tissue around the infrarenal aorta or iliac arteries |
8 |
total score =
= SUM(MAX(each parameter))
Interpretation:
• minimum score: 0
• maximum score: 88
• A score >= 20 is associated with an IgG4-related disorder.
