Vasculitis manifested with multiple mass lesions in kidneys, lungs and soft tissue, mimicking malignant tumors

We report a case of granulomatosis with polyangiitis (GPA) (Wegner's granulomatosis) who presented with multiple mass lesions in kidneys and lung lobes, as well as neck soft tissue, mimicking malignancies. This 71-year-old woman initially presented with sudden right foot drop, left calf pain and right eye vision loss. She was treated with corticosteroid for the diagnosis of possible temporal arteritis. Months after steroid was tapered to 2 mg per day, she developed increasing fatigue, weight loss, and shortness of breath. CT scan showed lung mass lesions in left upper lobe (3.8 × 2.4 cm), right mid lung with pleural extension (3.4 × 3.3 cm), and right lower lobe (1.1 × 1.0 cm); right neck (3.3 × 2.6 cm), right kidney (2.3 × 1.8 cm) and left kidney (2.0 × 1.6 cm). Right quadriceps muscle biopsy shows focal granulomatous inflammation. Lung biopsy showed necrotizing and poorly formed granulomatous inflammation. Biopsies of kidney mass lesions showed necrotizing and non-necrotizing granulomatous inflammation. No crescentic or necrotizing glomerular lesions were observed in the total 40 sampled glomeruli. No malignancy was identified in any of the biopsies. Her c-ANCA was found to be positive and PR3-ANCA antibody was 6.88 U/ml (normal 0–0.90 U/ml). She was diagnosed with granulomatosis with polyangiitis and treated with high dose corticosteroid and rituximab. Eight months later, follow-up showed resolved mass lesions by chest X-ray and CT and stable renal function. The case highlights the atypical clinical presentation of vasculitis and the significance of considering this possibility in differential diagnosis when confronting mass lesions present in multiple organ systems. Biopsy is critical for the correct diagnosis to initiate timely and appropriate treatment, and also important to avoid unnecessary surgical resection

Wirsungocele: evaluation by MRCP and clinical significance

Purpose: Wirsungocele is a rare cystic dilatation of the main pancreatic duct seen at the terminal portion of the duct of Wirsung. The purpose of our study is to evaluate the diagnostic value of MRCP in detection of Wirsungocele and the association between the MRCP-determined size of Wirsungocele and the MRCP-clinical findings of pancreatitis. Methods: Thirty-four patients with reported 'Wirsungocele' were analyzed in the study. Two radiologists reviewed MRCP/S-MRCP images for the presence and diameter of Wirsungocele (WD), main pancreatic duct dilatation (MPDD), side branch ectasia (SBE), acinarization, and duodenal filling grade. Electronic medical record review included symptoms (abdominal pain), signs (recurrent acute/chronic pancreatitis), and select laboratory testing (serum amylase and lipase). Inter-reader agreement values were calculated by ICC. Pearson correlation analysis was performed to evaluate the association of WD with radiological and clinical findings. The comparison of WD on MRCP versus S-MRCP was calculated by Wilcoxon test. Mann-Whitney U test was used for two independent variable comparisons. Results: The sensitivity of MRCP for the detection of Wirsungocele calculated using the S-MRCP and ERCP as the reference method was 76.9% and 100%, respectively. There was a significant difference in the diameter of Wirsungocele measured by MRCP vs S-MRCP (p < 0.001). There was good inter-reader agreement for the detection of Wirsungocele on MRCP and measurement of WD on MRCP and S-MRCP (ICC: 0.79, 0.89, and 0.80, respectively, p < 0.001). There was a significant difference in WD between the patients with and without MPDD (p < 0.05). There was a significant positive correlation between WD and MPDD (r = 0.66, p < 0.05). WD was significantly associated with recurrent acute pancreatitis (p < 0.05). Conclusion: MRCP is a highly sensitive and non-invasive imaging tool for detection of Wirsungocele. Greater Wirsungocele diameter is associated with MPDD and recurrent acute pancreatitis