Metachronous extrapancreatic lesions in autoimmune pancreatitis.
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OBJECTIVE: Autoimmune pancreatitis (AIP) is frequently associated with various extrapancreatic lesions. The distribution and frequency of extrapancreatic lesions preceding or subsequent to AIP are unknown. The aim of this study was to investigate metachronous extrapancreatic lesions of AIP. PATIENTS AND METHODS: Extrapancreatic lesions were examined clinically, radiologically, and histologically in 56 AIP patients. RESULTS: Extrapancreatic lesions were associated in 25 (45%) of 56 AIP patients. Twenty-nine extrapancreatic lesions were detected synchronously with AIP in 18 patients, and 18 lesions were detected metachronously in 11 AIP patients. Fourteen patients had more than 2 extrapancreatic lesions. There was no significant difference in serum IgG4 levels between AIP patients with preceding extrapancreatic lesions and synchronous extrapancreatic lesions. Extrapancreatic lesions preceding AIP were sclerosing sialadenitis (n=8), cervical lymphadenopathy (n=4), swelling of the lacrimal glands (n=2), retroperitoneal fibrosis (n=1), and hilar lymphadenopathy (n=1). Retrospective histopathological examination confirmed that these lesions were compatible with IgG4-related sclerosing disease. Steroid therapy was not given for these initial lesions, and AIP occurred 3 to 48 months after these initial lesions. Swelling of the preceding extrapancreatic lesions persisted when AIP occurred. Extrapancreatic lesions subsequent to AIP were retroperitoneal fibrosis (n=1) and systemic lymphadenopathy (n=1), both of which occurred during follow-up of AIP without steroid therapy. All extrapancreatic lesions improved after steroid therapy. CONCLUSION: Swelling of salivary or lacrimal glands, lymphadenopathy, and retroperitoneal fibrosis can precede AIP. Lymphadenopathy and retroperitoneal fibrosis can occur subsequent to AIP. Recognition of these findings will aid in the correct diagnosis of AIP. (+info)
Prognostic factors in retroperitoneal fibrosis.
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The aim of this study is to evaluate effective prognostic factors in the evolution of patients with retroperitoneal fibrosis and to establish the validity of fractal analysis in determining the disease severity in these patients. MATERIAL AND METHODS: This study included 19 patients (M/F: 5/14) treated for idiopathic retroperitoneal fibrosis and bilateral obstructive renal failure between Jan 2004-Dec 2008. Patients were identified retrospectively, searching for patients diagnosed with IRF, after retroperitoneal biopsy or, in most cases the diagnosis rested on radiological findings, especially CT, with identification of a retroperitoneal mass, the absence of other demonstrable renal or ureteric disease or any other pathology that could explain the findings. CT was very useful in describing the retroperitoneal mass around the aorta and inferior vena cava, the extent of the lesion and for monitoring the response to surgical treatment during the follow-up. The data were evaluated about medical history, physical examination findings, laboratory tests (serum urea and creatinine, blood sugar, sodium, potassium, bicarbonate levels, serum pH, uric acid, haematocrit, white blood cell count), imaging methods (renal ultrasound, abdominal CT-scan, MRI). At admission all patients had active disease with obstructive renal failure and underwent bilateral ureteric stenting in order to normalize the BUN levels. After normalizing of BUN levels, ureterolysis and omental wrapping was performed. Postoperatively, ureteric stents were removed after 1 month and remission of renal disfunction was obtained in approximately 5 months (range 2-10 months). All patients were followed for at least 1 year. Patients were regularly checked every 3 months. RESULTS: Of the 19 patients, there were 5 men and 14 women. The median age at diagnosis of RF was 50 years (range 42-64 years). The most frequent presenting symptoms were back or abdominal pain, weakness, weight loss, oligoanuria, arterial hypertension and mild fever. The duration of symptoms before diagnosis ranged from 6 to 18 months. At presentation all patients had active disease, presenting renal dysfunction with a median serum creatinine of 5.18 mg/dl (range 1-15.4 mg/dl). Most of the patients had moderate bilateral hydronephrosis (2nd degree hydronephrosis). In our study, all patients had excellent prognosis, with full recovery of renal function in 78% of cases (15 patients). The fractal dimension of the fibrosis mass contour correlates with level of renal function impairment. Even more, the fractal dimension seems to slightly variate between CT evaluations (1.30 +/- 0.1), suggesting a non aggressive pattern of extension of the fibrotic mass characteristic for benign lesions. CONCLUSIONS: The imaging parameters did not predict the disease severity, except the increase in fractal dimension of fibrosis surface area. Efficacy of bilateral ureteric stenting in improving renal function is limited in most of the cases. Dispite the level of renal function impairment at admission, full recovery can be achieved after bilateral ureteric stenting/nephrostomy and ureterolisis. (+info)
Percutaneous transvenous stent implantation to external iliac vein stenosis in a patient with retroperitoneal fibrosis.
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A 59-year-old man visited our hospital due to right leg edema and right leg pain. Computed tomography revealed that the circumferential enhancement of bilateral external iliac arteries by soft tissue that had similar density as the adjacent psoas muscle and that the right external iliac vein that was constricted by those tissues. The patient was diagnosed as retroperitoneal fibrosis. He underwent stent implantation to the right external iliac vein stenosis and steroid therapy. His right leg edema and pain was immediately improved after the stent implantation and he achieved remission. (+info)
Pseudocyst formation in retroperitoneal fibrosis relapse.
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Chronic periaortitis presenting as common bile duct obstruction.
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The case of a 67 year old woman is reported who presented with cholestatic jaundice and was found to have, in addition, an inflammatory abdominal aortic aneurysm. Only at necropsy did histopathology show chronic periaortitis as the aetiology of a pancreatic head mass which, during life, mimicked a pancreatic neoplasm obstructing the bile and pancreatic ducts. (+info)
Pancreatic actinomycosis as a cause of retroperitoneal fibrosis in a patient with chronic pancreatitis. Case report and literature review.
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CONTEXT: Chronic alcoholic pancreatitis is a debilitating disease that is often complicated by pseudotumoral changes of the pancreas, retroperitoneal fibrosis, and pancreatic cancer. Actinomycosis is an uncommon intra-abdominal infection and its association with chronic pancreatitis has been rarely reported. CASE REPORT: We present a case of a patient with progressive long standing chronic pancreatitis who develops pseudo-tumoural changes and retroperitoneal fibrosis associated with actinomycosis. CONCLUSION: This is a rare presentation of actinomycosis, posing a diagnostic challenge to the clinician, with important therapeutic implications. (+info)