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Dear Editor,
Granulomatosis with polyangiitis (GPA) previously known as Wegener’s Granulomatosis
is an anti-neutrophilic cytoplasmic autoantibody (ANCA) associated vasculitis (AAV), usually
initially presents with respiratory, renal, and/or ear, nose & throat (ENT) involvement [1].
Recently, rituximab (RTX) is increasingly used for either induction or maintenance phases in
GPA [2, 3]. During the GPA, various organs could be involved. However, pancreas involvement
in GPA is rarely reported. Until now, there are few reported cases with either a pancreas mass or
acute pancreatitis, which in most cases were the first presentation of disease [4-6]. Here we
report a complicated ANCA negative GPA patient with both pancreas mass and acute pancreatitis
presentation, who had been expired weeks after RTX due sepsis occurrence.
A 38-year-old woman with complaining from swelling, pain, and nasal congestion and
discharge admitted to Amir-Alam otolaryngology tertiary referral center, Tehran University of
Medical Sciences in February 2019. Taking biopsy had confirmed Wegener’s granulomatosis. At
the time of admission, assessment severity through the Birmingham Vasculitis Activity Score
(BVAS) reveled score of 24, and the patient fell into a major relapse. Prednisolone at the dose of
1 mg/kg/day and one cycle of cyclophosphamide at the dose of 1000 mg were initiated. While
she showed drug reaction to cyclophosphamide. Ten days later, the patient started to have
epigastric pain, nausea, vomiting, icter and had a progressive rise in liver function tests and
bilirubin. Alkaline phosphatase and total bilirubin levels reached at the peak up 1900 unit/liter
and 16 mg/dl, respectively. In abdominal CT scan and magnetic resonance cholangiography
(MRCP), a mass in pancreas head with the diameter of 26*23 mm in the posterior part of the
pancreatic head dilated common bile duct with a diameter of 14mm has been recorded.
Accordingly, an endosonography (EUS) was done, showing a pancreas mass in pancreas head.
Fine needle biopsy (FNA) pathology showed pancreatitis pattern with no malignant cell. Then an
ERCP (endoscopic retrograde cholangio pancreatography) was done and a stent was placed in
pancreatic duct along with initiation of prednisolone at the dose of 1 mg/kg/day. After 20 days,
the patient’s symptoms resolved, pancreatic mass became smaller, and LFTs became normal.
RTX at the dose of 1000 gr has been initiated and the patient discharged with 60 mg/day
prednisolone. Two weeks later, she received the second dose of RTX at the dose of 1000 gr. One
month after the last dose of RTX, sepsis had occurred and she expired a few days later.
In most of the previously reported cases, pancreas involvement was reported as the first
presentation of the disease, while in our case was not. Indeed, she first was presented with nasal
and respiratory involvement and 1 year later pancreatitis symptoms appeared. In our cases,
although RTX and high dose corticosteroids resolved issue, they led in a serious lethal infection.
Taken together, both acute pancreatitis and non-malignant pancreatic mass could be presented as
the secondary presentations in GPA patients. Although such conditions could be well-managed
with high dose glucocorticoids and probably RTX, risk of serious and lethal infections is warned.
Bilateral Ureteral Stenosis with Hydronephrosis as First Manifestation of Granulomatosis with Polyangiitis (Wegener’s Granulomatosis): A Case Report and Review of the Literature