In situ analysis by SEM-EDX spectroscopy of 10 sarcoidosis cases from MINASARC study

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Sarcoidosis, inorganic dust exposure and content of bronchoalveolar lavage fluid: the MINASARC pilot study

Inhalation of mineral dust was suggested to contribute to sarcoidosis. We compared the mineral exposome of 20 sarcoidosis and 20 matched healthy subjects. Bronchoalveolar lavage (BAL) samples were treated by digestion-filtration and analyzed by transmission electron microscopy. The chemical composition of inorganic particles was determined by energy-dispersive X-ray (EDX) spectroscopy. Dust exposure was also assessed by a specific questionnaire. Eight sarcoidosis patients and five healthy volunteers had a high dust load in their BAL. No significant difference was observed between the overall inorganic particle load of each group while a significant higher load for steel was observed in sarcoidosis patients (p=0.029). Moreover, the building activity sub-score was significantly higher in sarcoidosis patients (p=0.018). These results suggest that building work could be a risk factor for sarcoidosis which could be considered at least in some cases as a granulomatosis caused by airborne inorganic dust. The questionnaire should be validated in larger studies

LATE-BREAKING ABSTRACT: Sarcoidosis and inorganic dust exposition in the MINASARC (MIneralo-NAno-SARCoidosis) study

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The Minasarc study: A case-control study measuring mineral exposome in sarcoidosis

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The Minasarc study: A case-control study measuring mineral exposome in sarcoidosis

It has been suggested that sarcoidosis could be associated with exposure to inorganic particles (Newman LS Curr Opin All Clin Immunol 2012; 12:145-50, Vincent M et al Am J Ind Med 2015; 58:S31-8). In order to test this hypothesis the Minasarc study was designed to evaluate the mineral exposome by a specific questionnaire (SQ) and a mineralogical analysis performed on BALs by optical and electron microscopy in patients and healthy volunteers (HV). We present here the results obtained by the SQ which can be considered as a tool for global assessment of the “whole life” exposure to inorganic particles in occupational and environmental contexts.The study was performed on 20 patients with sarcoidosis and 20 HV. Every HV was matched to a patient by sex, age and smoking habit. The SQ was calibrated with a representative sample of the French population (n=825) in the ELIPSSilice survey (ANR-10-Eqpx-19-01) and the result was expressed as a “dust score”. Scores were compared by a Wilcoxon signed-rank test. [Résumé éditeur

The Minasarc study: A case-control study measuring mineral exposome in sarcoidosis

International audienceIntroduction: it has been suggested that sarcoidosis could be associated with exposure to inorganic particles (Newman LS Curr Opin All Clin Immunol 2012; 12:145-50, Vincent M et al Am J Ind Med 2015; 58:S31-8).Objectives: in order to test this hypothesis the Minasarc study was designed to evaluate the mineral exposome by a specific questionnaire (SQ) and a mineralogical analysis performed on BALs by optical and electron microscopy in patients and healthy volunteers (HV). We present here the results obtained by the SQ which can be considered as a tool for global assessment of the “whole life” exposure to inorganic particles in occupational and environmental contexts.Methods: The study was performed on 20 patients with sarcoidosis and 20 HV. Every HV was matched to a patient by sex, age and smoking habit. The SQ was calibrated with a representative sample of the French population (n=825) in the ELIPSSilice survey (ANR-10-Eqpx-19-01) and the result was expressed as a “dust score”. Scores were compared by a Wilcoxon signed-rank test.Results: The “dust score” was found significantly higher in patients with sarcoidosis than in HV (p=0,036; Wilcoxon signed-rank test). Moreover we found a significant overrepresentation of people exposed to building activities among the cases. However this remains to be assessed on a larger series.Conclusion: The SQ demonstrated a significantly higher level of exposure to inorganic dusts in patients with sarcoidosis compared to HV. Such preliminary results encourage 1) to study the association between sarcoidosis and inorganic dust exposure and 2) to submit routinely this exposure questionnaire to every patient with a granulomatous disease

Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial

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How should we diagnose and treat blastic plasmacytoid dendritic cell neoplasm patients?

Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive leukemia for which we developed a nationwide network to collect data from new cases diagnosed in France. In a retrospective, observational study of 86 patients (2000-2013), we described clinical and biological data focusing on morphologies and immunophenotype. We found expression of markers associated with plasmacytoid dendritic cell origin (HLA-DRhigh, CD303+, CD304+, and cTCL1+) plus CD4 and CD56 and frequent expression of isolated markers from the myeloid, B-, and T-lymphoid lineages, whereas specific markers (myeloperoxidase, CD14, cCD3, CD19, and cCD22) were not expressed. Fifty-one percent of cytogenetic abnormalities impact chromosomes 13, 12, 9, and 15. Myelemia was associated with an adverse prognosis. We categorized chemotherapeutic regimens into 5 groups: acute myeloid leukemia (AML)–like, acute lymphoid leukemia (ALL)–like, lymphoma (cyclophosphamide, doxorubicin, vincristine, and prednisone [CHOP])–like, high-dose methotrexate with asparaginase (Aspa-MTX) chemotherapies, and not otherwise specified (NOS) treatments. Thirty patients received allogeneic hematopoietic cell transplantation (allo-HCT), and 4 patients received autologous hematopoietic cell transplantation. There was no difference in survival between patients receiving AML-like, ALL-like, or Aspa-MTX regimens; survival was longer in patients who received AML-like, ALL-like, or Aspa-MTX regimens than in those who received CHOP-like regimens or NOS. Eleven patients are in persistent complete remission after allo-HCT with a median survival of 49 months vs 8 for other patients. Our series confirms a high response rate with a lower toxicity profile with the Aspa-MTX regimen, offering the best chance of access to hematopoietic cell transplantation and a possible cure.</jats:p