Granulomatosis with Polyangiitis Presenting as Pancreatic Pseudotumor and Peripancreatic Lymphadenitis: Diagnostic Challenges and Review of 55 Cases

Camille Beniada; Yann Coattrenec; Sahar Mack; Alexis Ricoeur; Volkan Adsay; Giacomo Puppa; Jörg D. Seebach

doi:10.12659/AJCR.945741

31 May 2025 : Case report Switzerland

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

Authors information Article notes Copyright and License information

Introduction Case Report Discussion Conclusions References

Related articles Order reprints Share article Share by email

View HTML version

Table 1 Literature review: characteristics of 55 cases of pancreatic AAV.

Reference	Author	Publication		Sex	Age (y)	Digestive clinical signs and symptoms	Radiological findings	Known diagnosis	Time to Dx	ANCA
Reference	Author	Year	Country	Sex	Age (y)	Digestive clinical signs and symptoms	Radiological findings	Known diagnosis	Time to Dx	Type	Timing*

[]⁵	Abu-Hilal	2008	UK	F	20	Epigastric pain, nausea	Pancreatitis, thrombose splenic vein	No	2m	PR3+ >600 IU/ml	1m after IE
[]⁶	Alesaeidi	2021	IRA	F	38	Epigastric pain, nausea, vomiting, jaundice	Pancreatic mass (head), pancreatic duct dilatation, pancreatitis		IE
[]⁷	Aslam	2024	UK	M	50	Jaundice	Pancreatitis, bile duct dilatation; pseudocyst (1 m later)	No	IE	PR3+ 43IU/mL	IE
[]⁸	Bachmayer	1984	Austria	F	60			No	IE	NA	NA
[]⁹	Balasa	2013	ROM	F	39	Abdominal pain, jaundice, nausea, vomiting	Pancreatic mass	No	2y	Neg	2y after IE
Present case	Beniada	2025	CH	F	55	Epigastric pain, nausea, vomiting	Pancreatic mass (tail), pancreatitis lymphadenopathies	No	3m	PR3+ 35 IU/mL (<5)	After surgery
[]¹⁰	Benmediouni	2020	ALG	M	51	Abdominal pain, nausea, vomiting	Pancreatic mass (tail), peripancreatic infiltration	No	IE	cANCA+	IE
[]¹¹	Çakar	2017	TUR	F	47	Abdominal pain	Pancreatic mass (tail)	No	2m	PR3+ >100 (<4) IU/mL	After surgery
[]¹²	Castillo	2019	USA	M	73		Pancreatic mass (head)	No	3m	cANCA+	IE
[]¹³	Chawla	2011	USA	F	60	Epigastric pain, nausea, vomiting	Pancreatic mass (head), pancreatitis	No	2m	PR3+ 45 IU/ml	2m after IE
[]¹⁴	Christl	2004	GER	F	50	Epigastric pain, nausea, vomiting	Pancreatic mass (tail)	No	1m	PR3+	After surgery
[]¹⁵	De Bie	2015	NL	M	57	Jaundice	Pancreatic mass (head), bile duct dilatation	No	1m	MPO	IE
[]¹⁶	Garbe	2021	GER	F	49	Epigastric pain	Pancreatitis	No		PR3+	IE
[]¹⁷	Hamilton	2011	UK	F	78		Pancreatic mass (tail)			PR3+ >100 IU/ml (<9)
[]¹⁸	Ilo	2021	USA	F	41	Epigastric pain, nausea, vomiting	Pancreatic mass (head and body)	No		PR3+	IE
[]¹⁹	Iqbal	2019	IRE	M	69		Pancreatic mass	No	NA	cANCA+	IE
[]²⁰	Joshipura	2007	IND	M	47	Abdominal pain	Pancreatitis	No	2m	PR3+ 156 IU/ml (<5)	IE
[]²¹	Katikineni	2019	USA	NA	85	Abdominal pain, jaundice	Pancreatic mass	No	5m	PR3+	IE
[]²¹	Katikineni	2019	USA	NA	70	Abdominal pain	Pancreatic mass	No	3m	PR3+	IE
[]²¹	Katikineni	2019	USA	NA	21	Abdominal pain	NA	No	2m	PR3+	IE
[]²²	Kawajiri	2024	JAP	M	62	None	Pancreatitis	No	2m	PR3+ 4.5 IU/ml (<2)	IE
[]²³	Kemp	1990	USA	M	57	Epigastric pain, nausea, vomiting	Pancreatitis, pseudocyst (body)	Yes	3m	NA	NA
[]²⁴	Kontis	2014	UK	M	57	Abdominal pain, nausea	Pancreatic mass (tail)	No	NA		After surgery
[]²⁴	Kontis	2014	UK	F	68	Abdominal pain, jaundice	Pancreatic masses (uncinate process and tail)	No	3m	MPO+ 8.8 IU/mL (<3.4)	After surgery
[]²⁵	Lisboa	2022	UK	F	61	Nausea, vomiting	Pancreatic mass and pseudocyst (body), presacral pelvic mass (2m before)	No	2m	PR3+ 44 IU/mL	IE
[]²⁶	Marroun	2006	FRA	M	68	Jaundice	Pancreatic mass (head)		NA	pANCA+ 1/160, ELISA negative
[]²⁷	Matsubayashi	2001	JAP	M	65	Abdominal pain, constipation	Pancreatitis	No	1m	PR3+ 14 EU/ml (<10)	1m after IE
[]²⁸	McCormick	2017	UK	F	52	Jaundice	Bile duct and pancreatic duct dilatation	No		PR3+ >8 IU/ml	8m after IE
[]²⁹	O’Neil	1992	USA	M	62	Jaundice	Pancreatic mass (head), bile duct dilatation, pancreatitis	No	6m	Pos	IE
[]³⁰	Pezzilli	1991	ITA	M	66			No	NA	pANCA+	NA
[]³¹	Reddy A	2017	USA	F	20	Epigastric pain, jaundice	Pancreatic mass and pseudocyst (head), bile duct dilatation	No		Neg	After surgery
[]³²	Reddy R	2007	USA	F	34	Epigastric pain, dysphagia due to intestinal ulcerations	Pancreatic mass (body), chronic pancreatitis (several months later under treatment)	No	IE	PR3 >100 IU/mL (<3.5)	IE
[]³³	Şahin	2012	TUR	M	37	Abdominal pain	Pancreatitis, pseudocyst		1m	cANCA+
[]³³	Şahin	2012	TUR	F	54	Abdominal pain	Pancreatitis, pseudocyst, lymphadenopathies		NA	NA	NA
[]³⁴	Sowida	2019	UK	F	22		Pancreatic mass, pseudocyst (tail), hepatomegaly	No		PR3+ 21 IU/ml	IE
[]³⁵	Stuckey	1992	AUS	M	45	Epigastric pain, nausea, vomiting, jaundice	Pancreatitis, pseudocyst	No	Months		NA
[]³⁶	Tao	2021	CAN	F	66	Epigastric pain, nausea, vomiting	Focal pancreatitis, multiples lesions (head and tail)	No	2m	PR3+ 262 IU/mL (<20)	IE
[]³⁷	Tinazzi	2007	ITA	F	48	Epigastric pain	Pancreatic mass (head)	No	NA		IE
[]³⁸	Tyagi	2021	IND	M	43	Epigastric pain	Pancreatitis	No		PR3+	IE
[]³⁹	Valerieva	2013	BUL	F	62	Epigastric pain	Pancreatitis, pancreatic mass (tail)	No	IE	PR3+ 90 IU/mL (<6)	IE
[]⁴⁰	Youssef	2023	CAN	F	48	Epigastric pain	Pancreatitis, focal swelling (tail/body)	No		PR3+ 27 IU/ml	3m after IE
[]⁴¹	Zaidi	2024	USA	M	51	Abdominal pain, nausea	Pancreatic mass (tail)		NA	NA	NA

[]⁴²	Berney	1997	CH	M	32	Epigastric pain	Pancreatitis (initially necrotizing/edematous upon relapse)	No	1m	cANCA+	IE
[]⁴³	Cho	2016	KOR	F	59	Epigastric pain, nausea		No	IE	MPO	IE
[]⁴⁴	Haraguchi	2005	JAP	F	84	Abdominal pain?	Pancreatitis		IE	MPO+ 73 IU/ml (<5)
[]⁴⁵	Iida Takeshi	2015	JAP	F	64		Pancreatitis (head)	No	IE	MPO 1625 IU/mL (<10)	IE
[]⁴⁶	Iida Tomoya	2016	JAP	M	72		Pancreatic mass (tail, uncinated process), pancreatitis	No	IE	MPO	9m after IE
[]⁴⁷	Iwasa	2005	JAP	F	84		Normal	No		MPO 12 IU/ml (<10)	IE
[]⁴⁸	Miyawaki	2016	JAP	M	77		Normal	No		MPO 128 IU/ml	IE
[]⁴⁹	Segraves	2017	USA	M	56	Abdominal pain	Pancreatitis, small bowel ischemia	Yes	2m	MPO	Before
[]⁵⁰	Suzuki	2019	JAP	M	71	Abdominal pain	Pancreatic mass (head), pancreatic duct dilatation, pancreatitis	No	IE	MPO 132 IU/ml	IE
[]⁵¹	Yamada	2022	JAP	M	67	None	Pancreatic mass (head), pancreatitis	No	IE	MPO 60IU/ml	IE
[]⁵²	Yokoi	2015	JAP	F	66	None	Pancreatic mass (head), bile duct and pancreatic duct dilatation	No	IE	MPO 473IU/mL (<3.5]	IE
[]⁵³	Zandvakili	2017	USA	F	72	Abdominal pain, nausea, vomiting	Pancreatic mass (tail)	No	3m	MPO	IE

[]⁵⁴	Marvisi	2021	ITA	M	62	Abdominal pain, nausea, vomiting	Pseudocyst			MPO
^* timing of ANCA dosing; ^** onset timing of extra-pancreatic manifestations. m – months; y – years; NA – no details available; Neg – negative; Pos – positive; M – male; F – female; CCS – corticosteroids; CYPH – cyclophosphamide; AZA – azathioprine; MMF – mofetil mycophenolate; RTX – rituximab; PEX – plasma exchange; ENT – ear nose and throat; IVIG – intravenous immunoglobulins; EUS-FNA – Endoscopic Ultrasound-Guided Fine-Needle Aspiration; IE – initial evaluation.
Reference	Elevated amylase, lipase, trypsin	Operation	Pancreatic Histology	Other histology	Extra-pancreatic involvement		Treatment	Diagnosis	Outcome (follow-up)
Reference	Elevated amylase, lipase, trypsin	Operation	Pancreatic Histology	Other histology	Organs	Timing**	Treatment	Diagnosis	Outcome (follow-up)

[]⁵	Normal amylase	Colonectomy	No	Kidney, intestine	Kidneys, lungs, skin, joints, digestive tract	1m after	CCS, CYPH	GPA	Death 13m (multi-organ failure)
[]⁶	NA	Pancreatic duct stenting	EUS-FNA inconclusive	Nose	ENT	1y before	CCS, CYPH, RTX	GPA	Death (sepsis)
[]⁷	Normal amylase	EUS guided cystogastrostomy	No	No	ENT, lungs, gingivitis	Concomitant	CCS, CYPH, Creon	GPA	Favorable (2m)
[]⁸	Lipase 604U/L	No	Granulomatous pancreatitis, multi-nucleated giant cells, fibrosis, necrotizing vasculitis, lymphadenitis		Kidneys, lungs, ENT (sialadenitis), mouth ulcer, intestinal, spleen, liver, lymph nodes, vulvar ulcer	Concomitant	CCS, CYPH	GPA	Death (renal failure)
[]⁹	NA	No	EUS-FNA inconclusive: chronic inflammation, no necrosis	Mastoid	ENT, lungs, strawberry gingivitis, skin, kidneys, cranial neuropathy	2y after		GPA	Death (renal failure)
Present case	Yes, lipase 69 IU/L (<60)		Granulomatous chronic pancreatitis with multi-nucleated giant cells, fibrosis and necrotizing vasculitis, lymphadenitis; 2 EUS-FNA inconclusive	No	ENT(?), kidneys, lungs	Concomitant (lungs, ENT), 8m after (kidneys)	CCS, RTX	GPA	Favorable
[]¹⁰	Yes, amylase normal, lipase 100 IU/L		Granulomatous inflammation, necrotizing vasculitis of pancreas, peripancreatic fat tissue, spleen, colon	No				GPA	Favorable (3m)
[]¹¹	NA	Laparotomy	Acute necrotizing pancreatitis	Skin	Kidneys, lungs, skin	Concomitant	CCS, CYPH, IVIG, RTX, dialysis	GPA	Favorable (4 m)
[]¹²	NA	No	No	Lung	ENT, kidneys, skin	Concomitant	CCS, RTX	GPA	Favorable (5 m)
[]¹³	Yes, lipase 1316 IU/L	No	EUS-FNA inconclusive	Kidney	ENT, kidneys, lungs, heart	2m after	CCS, CYPH, AZA	GPA	Favorable (10 m)
[]¹⁴	Normal		Granulomatous inflammation, necrosis, necrotizing vasculitis	Kidney	Kidneys, nervous system	Concomitant	CCS, CYPH, MMF, dialysis	GPA	Favorable
[]¹⁵	Normal	Biliary stent	No	Kidney, nose	ENT, kidneys	Concomitant	CCS, CYPH, PEX, dialysis	GPA	Dialysis
[]¹⁶	Yes	No	No	Kidney	ENT, lungs, skin, nervous system	Concomitant	CCS, CYPH, AZA, PEX	GPA	Favorable
[]¹⁷	NA	No	No	Lung	ENT, lungs, kidneys, joints, skin	8y before	CCS, CYPH	GPA	Favorable
[]¹⁸	Normal	No	Granulomatous disease (EUS-FNA)	No	ENT, lungs, skin	Concomitant, ENT probably before	CCS	GPA	Favorable (2 m)
[]¹⁹	NA	Laparotomy	Necrotizing vasculitis, CT guided, laparoscopic bx inconclusive	No	Lungs	Concomitant	CCS, CYPH?	GPA	Favorable
[]²⁰	Yes, amylase 874 IU/L (<300); lipase 1294 IU/L (<105)	No	No	Nose	ENT, kidney, joints, episcleritis, mouth ulcers	Concomitant	CCS, CYPH	GPA	Favorable (6m)
[]²¹	Yes	No	No	Kidney	Kidneys	Concomitant	CCS, RTX	GPA	Favorable
[]²¹	Yes	No	No	Kidney	Kidneys	Concomitant	CCS, CYPH, dialysis	GPA	Death (respiratory failure)
[]²¹	Yes	No	No	Kidney	Kidneys, joints	Concomitant	CCS, RTX, PEX	GPA	Favorable
[]²²	NA	No	EUS-FNA inconclusive: mild inflammatory infiltrate, few plasma cells	Lung, lacrimal gland	Lungs, lacrimal gland, ENT	Concomitant	CCS, RTX	GPA	Favorable (18 m)
[]²³	Yes, amylase 1039 IU/L, lipase 6.2 U/ml (<1.5)	No	No	Nose	ENT, kidney, skin, joint, lungs	16y before	CCS, CYPH	GPA	Favorable (2m)
[]²⁴	NA		Necrotic mass, palisading granuloma, vasculitis of the pancreatic adipose tissue, infiltration into pancreas, colon and splenic capsules; no preoperatory bx	No				GPA	NA
[]²⁴	NA		Necrotic masses with granulomatous inflammation and vasculitis, no preoperatory bx	No				GPA	NA
[]²⁵	NA	Nephrostomy	No	Pelvic mass	Skin, pelvic mass with hydronephrosis	2m after	CCS, CYPH, AZA, PEX	GPA	Favorable (7y)
[]²⁶	Yes, lipase 160 IU/L (<60), amylase normal		Granulomatous pancreatitis, necrotizing vasculitis of small and medium vessels, no preoperatory bx	No	ENT, joints, orchitis	7y before	CCS, CYPH, IVIG	GPA	Favorable (6m)
[]²⁷	Yes; trypsin 550, amylase normal	No	Diffuse necrotizing pancreatitis, vasculitis, fibrosis		ENT, kidneys, lungs, spleen	1m after		GPA	Death (pancreatitis, hemorrhagic pneumonia)
[]²⁸	NA	Biliary stent	No	Lung	Lungs, kidneys, episcleritis, joints, skin, neuropathy	8m after	CCS, CYPH	GPA	Favorable
[]²⁹	Normal	Biliary stent	EUS-FNA inconclusive	Kidney	ENT, kidneys, lungs, heart	Concomitant	CCS, CYPH	GPA	Favorable (1y)
[]³⁰	Yes, amylase 277 IU/ml, lipase 256 IU/L	No	No	Nose	ENT, joints, kidneys, skin, lung, liver	Concomitant	CCS, CYPH	GPA	Favorable, exocrine insufficiency
[]³¹	Yes, lipase		Granulomatosis with vasculitis	No	Lungs(?)	Concomitant	NA	GPA	NA
[]³²	Initially normal (mass), later Yes, amylase 273IU/L, lipase 74IU/L	No	No	Skin	Kidneys, lungs, ENT, esophageal, skin, intestinal	NA	CCS, CYPH, AZA, dialysis	GPA	Dialysis
[]³³	Yes, amylase 463 IU/L (<128)	Cyst drainage	No	Kidney?	Kidneys, lungs	7m before		GPA	Favorable
[]³³	Yes, amylase 625 IU/L (<128); lipase 89 ng/dl	EUS guided cystogastrostomy	No	Kidney?	Kidneys, lungs	20m before		GPA	Favorable
[]³⁴	NA	No	No	Lungs	ENT, strawberry gingivitis, lungs	Concomitant	CCS, RTX, AZA	GPA	Favorable
[]³⁵	Yes, amylase 239 IU/ml	No	No	Parotid glands	ENT, lungs	2m after	CCS, CYPH	GPA	Favorable (2m)
[]³⁶	Yes, lipase 338 IU/L (<300); amylase 96 IU/L (<105)	No	ERCP based bx inconclusive: chronic inflammation, some IgG4+ plasmocytes, no fibrosis, eosinophilia, malignancy	No	ENT, strawberry gingivitis, lungs	Concomitant	CCS, CYPH oral	GPA	Favorable (3m)
[]³⁷	NA		Granulomatous inflammation, necrosis, necrotizing vasculitis; no preoperatory bx	No			CCS, CYPH, MTX	GPA	Favorable
[]³⁸	Yes, amylase 362 IU/L, lipase 495 IU/L	No	No	No	ENT, skin, lungs	Concomitant	CCS, CYPH, AZA	GPA	Favorable (9m)
[]³⁹	Normal		Vasculitis with fibrinoid necrosis, granulomas and giant cells; 2 EUS-FNA inconclusive	No	ENT	Concomitant	CCS, AZA	GPA	Favorable (6m)
[]⁴⁰	Yes, lipase 316IU/L	No	No	No	ENT	Concomitant	CCS, MTX	GPA	Favorable
[]⁴¹	Normal lipase	No	Granulomatose inflammation, necrosis (EUS-FNA)	Lung, nose	ENT, lungs, liver	ENT before; lungs and liver concomitant	NA	GPA	NA

[]⁴²	Yes, both elevated	Laparotomy	No	Kidney	Kidneys	Concomitant	CCS, CYPH, dialysis	MPA	Kidney transplantation, relapse 3y later
[]⁴³	Yes, 825 U/L (<100); 927 U/L (<60)	No	No	Kidney	Kidneys, lungs	Concomitant	CCS, CYPH, dialysis, PEX	MPA	Death (sepsis)
[]⁴⁴	Yes, amylase 165 IU/mL (<110)	No	Vasculitis, fibrinoid necrosis, no granulomatosis		Kidneys, lungs	6m before	CCS	MPA	Death (necrotizing pancreatitis, DIC)
[]⁴⁵	Yes, amylase 543 IU/ml, lipase 460 IU/L	No	No	Kidney	Kidneys	Concomitant	CCS	MPA	NA
[]⁴⁶	Yes, amylase normal 59 IU/ml; lipase normal 53 IU/ml; trypsin elevated 723	No	3 EUS-FNA inconclusive	NA	Kidneys, lungs	9m after		MPA	Death (alveolar hemorrhage, renal failure)
[]⁴⁷	Yes, lipase 165 IU/L	No	Necrotizing pancreatitis, vasculitis		Kidneys, lungs, skin	Concomitant	CCS	MPA	Death 6m (necrotizing pancreatitis, DIC)
[]⁴⁸	NA	No	Necrotizing vasculitis		Kidneys, brain, myocardium, adrenal gland, small intestine, gallbladder, bronchus, prostate, testis, liver and spleen	Concomitant	CCS, CYPH	MPA	Death (cerebral bleeding)
[]⁴⁹	NA	60% terminal small intestine removal, ileostomy	No	Kidney	Kidneys, lungs, small intestine	Before	CCS, RTX, PEX, dialysis	MPA	Favorable
[]⁵⁰	Yes, 35 IU/ml; 294 IU/L	No	EUS-FNA inconclusive	No	Kidneys, lungs	Concomitant	CCS, CYPH, dialysis	MPA	Favorable (5m)
[]⁵¹	Normal, 18 IU/L (<127); 15 IU/L (<55)	No	Inflammatory cell infiltration, fibrosis, vasculitis (EUS-FNA)	Kidney	Kidneys, lungs, skin	Concomitant	CCS, AZA, insulin	MPA	Favorable, endocrine pancreatic insufficiency
[]⁵²	Normal amylase		Necrotizing vasculitis of small to medium-sized arteries, fibrosis, inflammatory cell infiltration, no granulomatosis	Kidney	Kidneys, small intestine, gall bladder	Concomitant	CCS, CYPH	MPA/PAN	Favorable (6m)
[]⁵³	Yes, amylase 130 IU/L, lipase 1200 UI/L	No	No	Kidney	Kidneys, lungs	Concomitant	CCS, CYPH, RTX	MPA	Favorable

[]⁵⁴	NA	Laparotomy, cyst removal	Vasculitis	No	Neuropathy, nasal polyps, asthma	Before	CCS, CYPH	EGPA	NA
^* timing of ANCA dosing; ^** onset timing of extra-pancreatic manifestations. m – months; y – years; NA – no details available; Neg – negative; Pos – positive; M – male; F – female; CCS – corticosteroids; CYPH – cyclophosphamide; AZA – azathioprine; MMF – mofetil mycophenolate; RTX – rituximab; PEX – plasma exchange; ENT – ear nose and throat; IVIG – intravenous immunoglobulins; EUS-FNA – Endoscopic Ultrasound-Guided Fine-Needle Aspiration; IE – initial evaluation.

Back to the Article

Granulomatosis with Polyangiitis Presenting as Pancreatic Pseudotumor and Peripancreatic Lymphadenitis: Diagnostic Challenges and Review of 55 Cases

Your Privacy