Background-Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic fibro-inflammatory condition characterized by tumefactive lesions, dense lymphoplasmacytic infiltrate, storiform fibrosis and obliterative phlebitis. Serum IgG4 levels are usually elevated in 70–90% of cases. The mean age at diagnosis is 60 years with a striking male preponderance. (Male:Female – 8:3)ObjectivesTo present a single center experience with IgG4-RD and the diagnostic and therapeutic challenges encountered.MethodsA retrospective case review at an urban teaching hospital was done over a year after the availability of quantitative assessment of IgG4 levels and tissue immuno-histological staining techniques. This revealed four cases of IgG4-RD involving the pancreas, retroperitoneum, kidney and meninges demonstrating its multi-systemic nature.ResultsWe encountered a M:F ratio of 3:1 with mean age at presentation being 66 yearCase 1: A 76-year-old male with significant weight loss, erroneously diagnosed with pancreatic tail malignancy, underwent distal pancreatectomy and was subsequently diagnosed with AIP on biopsy.Case 2: A 71-year-old male s/p left partial nephrectomy 4 years ago with an incidental finding of right kidney mass (2x2x2cm). After undergoing right partial nephrectomy, he was diagnosed with IgG4-related kidney disease.Case 3: A 56-year-old male with abdominal pain and worsening renal function had MRI findings of a heterogeneous retroperitoneal mass attached to the right kidney. Biopsy revealed a diagnosis of IgG4-retroperitoneal fibrosis.Case 4: A 57-year-old female presented with headache, bilateral lower extremity weakness and gait abnormality. Elevated serum IgG4, leptomeningeal enhancement on MRI and dural biopsy all suggested IgG4-related pacchymeningitis.ConclusionsIgG4-RD often presents as a diagnostic conundrum. There is a need to increase clinician awareness of IgG4-RD, since early identification and subsequent treatment with immunomodulators may prevent disease progression and prevent complications and/or unnecessary surgeries. PET/CT may prove to be a useful adjunctive imaging technique to investigate for multi-system involvement. Over reliance on serum IgG4 levels for screening may result in missed diagnosis in a significant percentage of patients.Disclosure of InterestNone declared
Journal of Cardiovascular Magnetic Resonance 2009, 11(Suppl 1):O1Introduction: Prophylactic implantation of a cardioverter/ defibrillator (ICD) has been shown to reduce mortality in patients with chronic myocardial infarction (CMI) and an increased risk for life threatening ventricular arrhythmia (VA). The use of ICDs in this large patient population is still limited by high costs and possible adverse events including inappropriate discharges and progression of heart failure. VA is related to infarct size and seems to be related to infarct morphology. Contrast enhanced cardiovascular magnetic resonance imaging (ceCMR) can detect and quantify myocardial fibrosis in the setting of CMI and might therefore be a valuable tool for a more accurate risk stratification in this setting. Hypothesis: ceCMR can identify the subgroup developing VA in patients with prophylactic ICD implantation following MADIT criteria. Methods: We prospectively enrolled 52 patients (49 males, age 69 ± 10 years) with CMI and clinical indication for ICD therapy following MADIT criteria. Prior to implantation (36 ± 78 days) patients were investigated on a 1.5 T clinical scanner (Siemens Avanto © , Germany) to assess left ventricular function (LVEF), LV end-diastolic volume (LVEDV) and LV mass (sequence parameters: GRE SSFP, matrix 256 × 192, short axis stack; full LV coverage, no gap; slice thickness 6 mm). For quantitative assessment of infarct morphology late gadolinium enhancement (LGE) was performed including measurement of total and relative infarct mass (related to LV mass) and the degree of transmurality (DT) as defined by the percentage of transmurality in each scar. (sequence parameters: inversion recovery gradient echo; matrix 256 × 148, imaging 10 min after 0.2 μg/kg gadolinium DTPA; slice orientation equal to SSFP). MRI images were analysed using dedicated software (MASS © , Medis,
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