Pancreatic mass as an initial presentation of severe Wegener's granulomatosis
A 62-year-old woman was admitted to the hospital with ten-day history of acute epigastric pain, radiating to the back and associated with nausea, persistent fever up to 38°C and headache. She had a history of cough and sinusitis a month ago, complicated with otitis media, with insignificant improvement on antibiotic treatment and antitussive drugs. The patient had no other significant past and family history except laparoscopic cholecystectomy for lithiasis 15 years ago. She denied alcohol use, herbal or over-the-counter medications and allergies.\nExamination revealed epigastric tenderness without rebound and no palpable organomegaly, peristalsis was normal. There was purulent discharge from the left ear, pain in maxillary sinus and left mastoid. No other pathological findings were present.\nThe initial laboratory tests showed slight leukocytosis, trombocytosis, normal ESR, CRP 15.8 mg/dL (normal range <0.6 mg/dL), elevated fibrinogen, ALT and GGT were up to 2xURL (upper reference level) and 3xURL respectively. Serology for HBV and HCV infection was negative. Serum amylase and lipase, cholesterol and triglycerides were within normal range. Albumin was 2.95 g/dL; blood glucose was 148 mg/dL. Serum iron (8.4 μg/dL) and TIBC (38 μmol/L) were low. Hemoglobin was normal at admission to hospital but dropped to 8.8 g/dL within a week without signs of bleeding and prothrombin time also decreased to 53%. Urinalysis showed increased numbers of leukocytes and erythrocytes with proteinuria 0.276 g/L/24h. Blood cultures and urine cultures were sterile. Coombs and Schirmer tests were negative. Serum IgG, IgA, IgM and serum tumor markers (CA 19-9, CEA) were within normal range. RF (rheumatoid factor), ANA (anti-nuclear antibodies) and anti-MPO (anti-myeloperoxidase) were tested negative, while anti-Pr3 (anti-proteinase 3, c-ANCA) were 15xURL (89.63 U/mL; negative <6 U/mL).\nInitial abdominal ultrasound (US) revealed slightly enlarged and hypoechoic pancreatic body and tail with blurry margins, as in acute pancreatitis. Computed tomography of the abdomen also confirmed edema of the pancreatic tail without fluid collections or other abnormal findings. Control US in several days showed blight echoes scattered among the enlarged pancreas, compression of the splenic vein and spleenomegaly. Further magnetic resonance was performed and revealed a 3 cm soft-tissue formation in the pancreas tail without pancreatic duct abnormalities, which compressed the splenic vein, without infiltration or enlarged regional lymph nodes. Imaging studies of the kidneys showed no alterations and chest X-ray was normal.\nThe initial diagnosis was acute pancreatitis but normal levels of serum amylase and lipase in the course of the disease, normal billiary tree imaging and lack of pancreatic disease history or alcohol consumption did not support this diagnosis. Normal immunoglobulin levels and absence of pancreatic duct abnormalities ruled out autoimmune pancreatitis, but positive c-ANCA suggested the possibility of other autoimmune diseases. However, pancreatic neoplasm was also suspected because of the presence of pancreatic tail mass. US-guided fine needle aspiration was performed twice but only detritus without atypical cells was established. However malignancy could not be entirely ruled out.\nDespite treatment with antibiotics, IV fluids, proton pump inhibitors and analgesia for a week, fever and severe abdominal pain still persisted. On the fifth day of hospitalization the patient suffered phlebothrombosis of left lower leg and low-molecular-weight heparin was added to therapy. Immunosuppressive treatment was discussed as an option, but the severe intractable abdominal pain and lack of cytological confirmation along with the possibility of pancreatic carcinoma indicated surgery. A solid tumor-like formation in the pancreatic body and tail with well-defined margins was resected and pancreato-jejunoanastomosis a modo Roux and splenectomy were performed ().\nThe histology from the surgical specimens confirmed WG of the pancreas and spleen – typical vasculitis with fibrinoid necrosis, granulomas and giant cells ().\nThe patient was further treated with steroids and azathioprine and a clinical remission followed. She was doing well at 6 months of follow up.