Autoimmunity associated with Erdheim-Chester disease improves with BRAF/MEK inhibitors

Roeser, Anaïs; Cohen‐Aubart, Fleur; Breillat, Paul; Miyara, Makoto; Émile, Jean-François; Charlotte, Frédéric; Donadieu, Jean; Amoura, Zahir; Haroche, Julien

doi:10.3324/haematol.2018.214007

Cited by 16 publications

(41 citation statements)

References 18 publications

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“…Participants in this study were enrolled in a prospective, IRB-approved ECD registry study (NCT03329274), and retrospective analysis of imaging data is performed by way of a paired retrospective analysis protocol. The registry was probed for participants with 18 F-FDG PET/CT scans performed prior to the initiation of any kinase inhibitor therapy, in order to examine scans reflective of active disease. Demographic and clinical data collected for the registry protocol include sex, race, ethnicity, age at ECD diagnosis, presenting ECD symptoms, and ECD driver mutation (e.g., BRAFV600E)).…”

Section: Patients and Clinical Datamentioning

confidence: 99%

“…Subsequently, patients rested for a scheduled 60-min uptake period. A total of 15 patients had their 18 F-FDG PET/CT examinations performed at outside institutions prior to the initiation of any ECD-specific treatment and submitted to MSKCC. 18 F-FDG PET/CT images were reconstructed with iterative reconstruction and displayed on a PET/CT workstation (PET VCAR; GE Healthcare) as multiplanar reconstructions with multiplanar PET, CT, and PET/CT fusion images.…”

Section: [18]f-fdg Pet/ct Imaging and Interpretationmentioning

confidence: 99%

“…A total of 15 patients had their 18 F-FDG PET/CT examinations performed at outside institutions prior to the initiation of any ECD-specific treatment and submitted to MSKCC. 18 F-FDG PET/CT images were reconstructed with iterative reconstruction and displayed on a PET/CT workstation (PET VCAR; GE Healthcare) as multiplanar reconstructions with multiplanar PET, CT, and PET/CT fusion images. All 18 F-FDG PET/CT images were reviewed by a dual nuclear medicine and diagnostic radiology board certified physician (G.A.U.)…”

Section: [18]f-fdg Pet/ct Imaging and Interpretationmentioning

confidence: 99%

“…There is limited data about optimal imaging to characterize extent of disease in untreated ECD. Studies of small cohorts suggest that 18 F-fluordeoxyglucose ( 18 F-FDG) positron emission tomography/computed tomography (PET/CT) is informative to evaluate the extent of ECD burden [20][21][22]. In addition, Arnaud et al have shown that 18 F-FDG PET/CT has great contribution in follow-up of ECD patients receiving treatment [23].…”

Section: Introductionmentioning

confidence: 99%

“…The purpose of this study was two-fold. First, we sought to describe 18 F-FDG PET/CT findings in 50 ECD patients enrolled in a prospective registry study undergoing metabolic imaging in the setting of either treatment-naive ECD or ECD that had progressed on prior chemotherapeutic or immunosuppressive therapy. Second, we sought to determine the utility of metabolic imaging versus anatomic imaging in evaluating ECD patients for measurable disease by formal response criteria, allowing for enrollment in therapeutic clinical trials.…”

Section: Introductionmentioning

confidence: 99%

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18F-FDG PET/CT versus anatomic imaging for evaluating disease extent and clinical trial eligibility in Erdheim-Chester disease: results from 50 patients in a registry study

Kirchner

Hatzoglou

Buthorn

et al. 2020

Eur J Nucl Med Mol Imaging

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Objectives The aim of this study was to [1] characterize distribution of Erdheim-Chester Disease (ECD) by 18F-FDG PET/CT and [2] determine the utility of metabolic (18F-FDG PET/CT) imaging versus anatomic imaging (CT or MRI) in evaluating ECD patients for clinical trial eligibility. Methods 18F-FDG PET/CT and corresponding CT or MRI studies for ECD patients enrolled in a prospective registry study were reviewed. Sites of disease were classified as [1] detectable by 18F-FDG PET only, CT/MRI only, or both and as [2] measurable by modified PERCIST (mPERCIST) only, RECIST only, or both. Descriptive analysis was performed and paired t test for between-group comparisons. Results Fifty patients were included (mean age 51.5 years; range 18–70 years). Three hundred thirty-three disease sites were detected among all imaging modalities, 188 (56%) by both 18F-FDG PET and CT/MRI, 67 (20%) by 18F-FDG PET only, 75 (23%) by MRI brain only, and 3 (1%) by CT only. Of 178 disease sites measurable by mPERCIST or RECIST, 40 (22%) were measurable by both criteria, 136 (76%) by mPERCIST only, and 2 (1%) by RECIST only. On the patient level, 17 (34%) had mPERCIST and RECIST measurable disease, 30 (60%) had mPERCIST measurable disease only, and 0 had RECIST measurable disease only (p < 0.0001). Conclusion Compared with anatomic imaging, 18F-FDG PET/CT augments evaluation of disease extent in ECD and increases identification of disease sites measurable by formal response criteria and therefore eligibility for clinical trials. Complementary organ-specific anatomic imaging offers the capacity to characterize sites of disease in greater anatomic detail. Trial registration ClinicalTrials.gov Identifier: NCT03329274

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Section: Patients and Clinical Datamentioning

confidence: 99%

Section: [18]f-fdg Pet/ct Imaging and Interpretationmentioning

confidence: 99%