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 4 Key takeaway points of pancreatic involvement in ANCA-associated vasculitis.
| Key point | Details |
|---|---|
| Pancreatitis & imaging clues | Hypodense lesions, pancreatic masses, and pseudocysts on CT may suggest AAV; GPA is 3.5 times more likely than MPA |
| Diagnostic limitations | Rare causes of pancreatic masses remain hard to identify; even endoscopic ultrasound-guided biopsies may be inconclusive |
| ANCA testing | Positive in 84% of pancreatic AAV cases; should be routinely performed in unexplained pancreatic disease |
| Extra-pancreatic involvement | Present in 75% of patients at diagnosis; should be actively investigated |
| Treatment response | Pancreatic AAV generally responds well to glucocorticoids; immunosuppressants like cyclophosphamide or rituximab recommended for severe cases |
| Importance of early diagnosis | Prompt recognition and treatment are essential to reduce the high morbidity and mortality associated with pancreatic AAV, avoids unnecessary pancreatectomy |