31 May 2025
: Case report 
Granulomatosis with Polyangiitis Presenting as Pancreatic Pseudotumor and Peripancreatic Lymphadenitis: Diagnostic Challenges and Review of 55 Cases
Mistake in diagnosis, Unusual or unexpected effect of treatment, Diagnostic / therapeutic accidents, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)
Camille Beniada ABCEF 1, Yann Coattrenec AE 1, Sahar Mack
ADEF 2, Alexis Ricoeur CD 3, Volkan Adsay CD 4,5, Giacomo Puppa DE 6, Jörg D. Seebach ACDEF 1*
DOI: 10.12659/AJCR.945741
Am J Case Rep 2025; 26:e945741
Table 2 Comparison of pancreatic disease associated with GPA and MPA.
| GPA (n=42) | MPA (n=12) | ||
|---|---|---|---|
| Age (range) | 53 y (20–85) | 67 y (32–84) | |
| Sex ratio | 22F/17M/3? | 6F/6M | |
| Geographical distribution | Europe/Northern America | 35 (83%) | 3 (25%) |
| Asia | 5 (12%) | 9 (75%) | |
| Digestive symptoms | 35 (83%) | 6 (50%) | |
| Radiological features | Mass | 25 (60%) | 5 (42%) |
| Pseudocyst (3 with mass) | 8 (19%) | 0 | |
| Pancreatitis (5 with mas, 5 with pseudocyst) | 18 (43%) | 7 (58%) | |
| Normal | 1 (2.3%) | 3 (25%) | |
| ANCA testing | cANCA/PR3 | 28 (67%) | 1 (8.3%) |
| pANCA/MPO | 4 (9.5%) | 11 (92%) | |
| ANCA+ | 1 (2.4%) | ||
| ANCA-negative | 6 (14%) | ||
| Pancreatectomy | 9 (21%) | 1 (8.3%) | |
| Extra-pancreatic manifestations | ENT | 25 (60%) | 0 |
| Lungs | 26 (62%) | 8 (67%) | |
| Kidneys | 22 (52%) | 12 (100%) | |
| Skin | 13 (33%) | 2 (17%) | |
| Pancreatic histology (available in 21) | 16 (38%) | 5 (42%) | |
| Granulomatous inflammation | 12 | 0 | |
| Necrotizing vasculitis | 12 | 5 | |
| Outcome (2–18 months) (available in 49) | Favorable | 30 (79%) | 5 (45%) |
| Dialysis/renal transplant | 2 | 1 | |
| Death | 6 (16%) | 5 (45%) | |
| Not available | 4 | 1 |