Case | Features | Histopathological findings | s-IgG4 (g/L) (ref > 1.35 for RCD, > 2.01 for 2019 ACR/EULAR classification criteria) | RCD | 2019 ACR/EULAR classification criteria |
---|---|---|---|---|---|
1 | Abdominal pain. RPF in a typical distribution. No other manifestations | Not performed | 5.08 | Possible IgG4-RD | Fulfils entry criterion. No exclusion criteria Weighted score = 14 • s-IgG4 = 6 • RPF = 8 |
2 | Abdominal pain. RPF in a typical distribution. No other manifestations | Not performed | 2.15 | Possible IgG4-RD | Fulfils entry criterion. No exclusion criteria Weighted score = 12 • s-IgG4 = 4 • RPF = 8 |
3 | Abdominal pain. RPF in a typical distribution. No other manifestations | Inconclusive fine needle aspiration | 0.63 | Not fulfilled | Fulfils entry criterion. No exclusion criteria Weighted score = 8 • RPF = 8 |
4 | Abdominal pain. iAAA and RPF in a typical distribution. No other manifestations | Not performed | 2.14 | Possible IgG4-RD | Fulfils entry criterion. No exclusion criteria Weighted score = 12 • s-IgG4 = 4 • RPF = 8 |
5 | Abdominal pain. RPF in a typical distribution. No other manifestations | Not performed | 0.50 | Not fulfilled | Fulfils entry criterion. No exclusion criteria Weighted score = 8 • RPF = 8 |
6 | Abdominal pain. RPF in a typical distribution. No other manifestations | Not performed | 2.90 | Possible IgG4-RD | Fulfils entry criterion. No exclusion criteria Weighted score = 12 • s-IgG4 = 4 • RPF = 8 |
7 | Renal failure and hydronephrosis. RPF in a typical distribution. No other manifestation | Not performed | 3.20 | Possible IgG4-RD | Fulfils entry criterion. No exclusion criteria Weighted score = 12 • s-IgG4 = 4 • RPF = 8 |
8 | Abdominal pain. RPF in a typical distribution. FDG-uptake in non-enlarged bilateral parotids. No other manifestations | Parotid gland biopsy: inconclusive | 0.80 | Not fulfilled | Fulfils entry criterion. No exclusion criteria Weighted score = 14 • One set glands = 6 • RPF = 8 |
9 | Hydronephrosis. RPF in a typical distribution. No other manifestations | Not performed | 2.87 | Possible IgG4-RD | Fulfils entry criterion. No exclusion criteria Weighted score = 12 • s-IgG4 = 4 • RPF = 8 |
10 | Abdominal pain and renal failure. RPF in a typical distribution. No other manifestations | Not performed | 2.00 | Possible IgG4-RD | Fulfils entry criterion. No exclusion criteria Weighted score = 8 • RPF = 8 |
11 | Long standing, severe asthma, rhinosinusitis and nasal polyposis. 15 years prior to IgG4-RD diagnosis: eosinophilic pneumonia with blood hypereosinophilia. Mild polyneuropathy. No other EGPA-manifestations. Negative ANCA. Nasal polyps demonstrated subacute inflammation with tissue eosinophilia, without evidence of vasculitis or granulomas. Treated with mepolizumab, which facilitated discontinuation of glucocorticoids. Two years prior to IgG4-RD diagnosis: AAA (without evidence of RPF). At time of diagnosis: large right coronary artery aneurysm (CAA). No other organ manifestations. Deemed as coexisting EGPA and IgG4-RD, with the latter accounting for the CAA. The rational was (i) the remaining EGPA manifestations were well controlled; (ii) CAA is an increasingly recognized manifestation of IgG4-RD; and (iii) previous reports of coexistence of IgG4-RD and AAV. However, we acknowledge the diagnostic uncertainty | Resected coronary artery aneurysm: LPC infiltrate and fibrosis (without storiform appearance), 72 IgG4 + PC/hpf and IgG4/IgG-ratio 0.55. Mild tissue eosinophilia | 3.20 | Not fulfilled (Exclusion criterion: EGPA) | Does not fulfil entry criterion (coronary artery not listed as a typical organ). No exclusion criteria Weighted score = 22 • Histology = 4 • IHC = 14 • s-IgG4 = 4 |
12 | Abdominal pain and hydronephrosis. RPF in a typical distribution, in combination with aortitis and iAAA. No other manifestations | Not performed | Not measured prior to glucocorticoids. < 1.35 following treatment initiation | Not fulfilled | Fulfils entry criterion. No exclusion criteria Weighted score = 8 • RPF = 8 |
13 | Abdominal pain and hydronephrosis. RPF in a typical distribution. No other manifestations | Not performed | 0.74 | Not fulfilled | Fulfils entry criterion. No exclusion criteria Weighted score = 8 • RPF = 8 |