Patterns of Relapse and Complications of Immunoglobulin G4-Related Disease.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic fibroinflammatory condition potentially resulting in organ dysfunction. We aimed to evaluate imaging characteristics of disease relapse and complications in this cohort of patients. METHODS: This was a cohort study of IgG4-RD patients imaged between 2010 and 2020. Radiological manifestations of disease activity (remission/stability vs. relapse and complications) were correlated with clinical symptoms. Univariate analyses were performed with χ2 , Fisher exact, and Mann-Whitney U tests. Times to relapse and organ atrophy were studied with Kaplan-Meier analyses. RESULTS: A total of 69 patients had imaging surveillance over a median duration of 47 months. Radiological relapse occurred in 50.7% (35/69) with median time to relapse at 74 months (95% confidence interval, 45-122 months); 42.8% (15/35) of this cohort had different-site relapse with the following recognized primary-secondary patterns: pancreas-hepatobiliary ( p = 0.005), hepatobiliary-pancreas ( p = 0.013), and periaortitis-mesenteric ( p = 0.006). Clinical symptoms were significantly associated with imaging characteristics ( p < 0.001). Abdominal complications were detected in 52.2% (36/69) of patients, mostly solid organ atrophy (97.2% [35/36]). New-onset diabetes was more likely in pancreatic IgG4-RD (n = 51) when accompanied by gland atrophy (4/21 vs. 0/30 nonatrophy, p = 0.024). CONCLUSION: Radiological relapse of IgG4-RD is common over prolonged imaging surveillance and is significantly associated with symptomatic relapse. A multisystem review to detect new/different sites of disease and abdominal complications may help predict future organ dysfunction.

A Timely Oral Option: Single-Agent Vinorelbine in Desmoid Tumors.

INTRODUCTION: Desmoid tumors (DT) are rare collagen-forming tumors that can exhibit locally aggressive patterns of behavior. The aim of this study was to evaluate the efficacy and safety of treatment of DT with single-agent oral vinorelbine. MATERIALS AND METHODS: A retrospective review of patients treated with vinorelbine 90 mg orally on days 1, 8, and 15 of a 28-day cycle from January 2004 to July 2019 was performed. Response was assessed using RECIST version 1.1. Descriptive statistics were employed. RESULTS: A total of 29 patients were included. Response rate was 20.7% (6/29), and clinical benefit rate (response by RECIST 1.1 and/or clinical symptom improvement) was 65.5% (19/29). No patient experienced grade 3 or above toxicity. Common toxicities were grade 1-2 nausea (14/26, 48.3%), fatigue (9/26, 31.0%), and diarrhea (4/26, 13.8%). CONCLUSION: Single-agent oral vinorelbine is an effective, safe, and well-tolerated treatment for DT. It represents a new oral alternative for management of DT.

Radiology domain in the diagnosis of IgG4-RD according to the 2019 American College of Rheumatology and European League Against Rheumatism classification.

OBJECTIVES: To evaluate the performance of radiology-related inclusion criteria of the 2019 ACR-EULAR classification system in the diagnosis of IgG4-related disease (IgG4-RD). METHODS: This retrospective single-institution study included patients who received a diagnosis of IgG4-RD between January 2010 and December 2020. Two abdominal radiologists independently reviewed baseline imaging studies and scored radiology findings according to the 2019 ACR-EULAR classification criteria. Additional scores were assigned based on serological, histopathological, and immunostaining features. RESULTS: Seventy-four patients (58 males and 16 females) with a mean age of 59.3 ± 13.9 years diagnosed with IgG4-RD were included. 51/74 (68.9%) were classified as having IgG4-RD according to the 2019 ACR-EULAR classification criteria. To reach a score ≥ 20 in these 51 patients, the radiology domain was sufficient in 20/51 (39.2%) and adding the serology domain was required for another 20/51 (39.2%). The remaining 11/51 patients (21.6%) required the histopathology and immunostaining domains. Radiological involvement of two or more organs at presentation was significantly associated with a score of ≥ 20 and seen in 43/51 (84.3%) compared to 5/23 (21.7%) of the non-classified group (p < 0.001). The group classified as having IgG4-RD showed a significantly higher proportion of elevated IgG4 levels (39/51, 76.5%) than the non-classified group (8/23, 34.8%) (< 0.001). CONCLUSION: The study findings support the effectiveness of the radiology-related inclusion criteria of the 2019 ACR-EULAR classification system in diagnosing IgG4-RD. Combining radiology and serology domains achieved the cut-off in 80% of IgG-RD patients, enabling non-invasive diagnosis. The classification of IgG4-RD was significantly associated with multi-organ involvement, particularly affecting the pancreas and biliary system. CRITICAL RELEVANCE STATEMENT: This study is the first to evaluate the diagnostic performance of the radiology domain in the 2019 ACR-EULAR classification criteria. The study results confirm its utility and potential to enable non-invasive diagnosis when combined with serological testing in a significant proportion of patients. KEY POINTS: • A significant proportion of patients can be diagnosed with IgG4-RD using the radiology and serology domains exclusively. • Multi-organ involvement is significantly associated with classifying patients as IgG4-RD, with the pancreas and biliary system most frequently affected. • A high level of inter-reader agreement in the scoring of the radiology domain supports its reliability.

Detection and staging of radio-recurrent prostate cancer using multiparametric MRI.

OBJECTIVE: We determined the sensitivity and specificity of multiparametric magnetic resonance imaging (MP-MRI) in detection of locally recurrent prostate cancer and extra prostatic extension in the post-radical radiotherapy setting. Histopathological reference standard was whole-mount prostatectomy specimens. We also assessed for any added value of the dynamic contrast enhancement (DCE) sequence in detection and staging of local recurrence. METHODS: This was a single centre retrospective study. Participants were selected from a database of males treated with salvage prostatectomy for locally recurrent prostate cancer following radiotherapy. All underwent pre-operative prostate-specific antigen assay, positron emission tomography CT, MP-MRI and transperineal template prostate mapping biopsy prior to salvage prostatectomy. MP-MRI performance was assessed using both Prostate Imaging-Reporting and Data System v. 2 and a modified scoring system for the post-treatment setting. RESULTS: 24 patients were enrolled. Using Prostate Imaging-Reporting and Data System v. 2, sensitivity, specificity, positive predictive value and negative predictive value was 64%, 94%, 98% and 36%. MP-MRI under staged recurrent cancer in 63%. A modified scoring system in which DCE was used as a co-dominant sequence resulted in improved diagnostic sensitivity (61%-76%) following subgroup analysis. CONCLUSION: Our results show MP-MRI has moderate sensitivity (64%) and high specificity (94%) in detecting radio-recurrent intraprostatic disease, though disease tends to be under quantified and under staged. Greater emphasis on dynamic contrast images in overall scoring can improve diagnostic sensitivity. ADVANCES IN KNOWLEDGE: MP-MRI tends to under quantify and under stage radio-recurrent prostate cancer. DCE has a potentially augmented role in detecting recurrent tumour compared with the de novo setting. This has relevance in the event of any future modified MP-MRI scoring system for the irradiated gland.