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• MDS/CMML-associated vasculitis display a highly wide clinical specter without any correlation with hematological disease status. • MDS/CMML-associated vasculitis have no significant impact on overall survival, but time to progression to acute myeloid leukemia was significantly longer in patients with vasculitis. • The high rates of relapse and GCs dependence raise the question of combined therapies and azacytidine therapy for even low-risk MDS/CMML vasculitis. Our objective was to evaluate characteristics, treatment and outcome of vasculitis associated with myelodysplastic syndrome (MDS) and chronic myelomonicytic leukemia (CMML) Retrospective descriptive analysis of MDS/CMML-related vasculitis and comparison with MDS/CMML patients without dysimmune features. Seventy patients with vasculitis and MDS/CMML were included, with median age of 71.5 [21–90] years and male/female ratio of 2.3. Vasculitis was diagnosed prior to MDS/CMML in 31 patients (44%), and after in 20 patients. In comparison with MDS/CMML without autoimmune/inflammatory features, vasculitis with MDS/MPN showed no difference in MDS/CMML subtypes distribution nor International Prognostic Scoring System and CMML-specific prognostic (IPSS/CPSS) scores. Vasculitis subtypes included Giant cell arteritis in 24 patients (34%), Behçet's-like syndrome in 11 patients (20%) and polyarteritis nodosa in 6 patients (9%). Glucocorticoids (GCs) were used as first-line therapy for MDS/CMML vasculitis in 64/70 patients (91%) and 41 (59%) received combined immunosuppressive therapies during the follow-up. After a median follow-up of 33.2 months [1–162], 31 patients (44%) achieved sustained remission. At least one relapse occurred in 43 patients (61%). Relapse rates were higher in patients treated with conventional Disease Modifying Anti-Rheumatic Drug (DMARDs) (odds ratio 4.86 [95% CI 1.38 - 17.10]), but did not differ for biologics (odds ratio 0.59 [95% CI 0.11–3.20]) and azacytidine (odds ratio 1.44 [95% CI 0.21–9.76]) than under glucocorticoids. Overall survival in MDS/CMML vasculitis was not significantly different from MDS/CMML patients without autoimmune/inflammatory features (p = 0.5), but acute leukemia progression rates were decreased (log rank <0.05). This study shows no correlation of vasculitis diagnoses with subtypes and severity of MDS/CMML, and no significant impact of vasculitis on overall survival. Whereas conventional DMARDs seem to be less effective, biologics or azacytidine therapy could be considered for even low-risk MDS/CMML vasculitis. [ABSTRACT FROM AUTHOR]

Pachymeningitis is rare, either idiopathic or secondary to inflammatory disorders, after tumoral, surgical or infectious causes have been excluded. The fibroinflammatory IgG4-related disease is one of the etiologies of pachymeningitis with only few cases reported yet. From a single referral regional center, we evaluated the frequency of IgG4-related disease as the cause of inflammatory pachymeningitis in 10% of cases. From a National case registry of IgG4-related disease the pachymeningitis frequency was 4.1%. We report eight new cases with cranial, spinal or both involvements and a literature review of 46 pathological proven cases. We observed that IgG4-related pachymeningitis is in most cases not associated to extra-neurological manifestations of the disease. Only 27% of spinal and 40% of cranial IgG4-related pachymeningitis are associated with other disease localizations. First line treatment strategies included surgery and steroids. The use of immunosuppressants or rituximab was necessary in 18% of spinal and 54% of cranial localizations. Some patients remained with sequellae and clinical and/or radiological improvement can be difficult to obtain. [ABSTRACT FROM AUTHOR]

Context Amyloidosis(A) prognosis is determined by cardiac involvement.The main types of A are immunoglobulin light chain(AL) and transthyretin-related(TTR), which can be mutated(TTRm) or senile(TTRwt). Specific treatments can’t be administrated unless A has been typed histologically.Literature suggests echocardiography, 99m Tc DPD scintigraphy and cardiac MRI could help typing A. We described these imaging modalities to assess these potential tools for an uninvasive typing. Material and methods We analysed these imaging modalities in patients examined at Cardiomyopathies Competence Center(CCC) of La Timone Hospital in Marseille, with an histologically proven diagnosis of cardiac A(CA). Results We included 75patients examined between September 2006 and March 2014 at CCC, with a strongly suspected diagnosis of CA.CA could be histologically confirmed and typed in 45 patients(10 TTRm, 4 TTRwt, 6 TTR undeterminated; 19 AL;6 of other type).In 11 patients, CA was confirmed but untyped.No statistically significant difference was found between TTR and AL patients for the various imaging modalities.We observed 71% of men, aged 66, NYHA stage 2,4 on average.In all patients, cardiac biomarkers rates were increased. Myocardic mass and interventricular septal thickness were increased (199g/m² and 19mm), restrictive filling pattern was observed in 83% of patients.Despite a relatively preserved left ventricular ejection fraction, Global Longitudinal Strain was impaired at – 11%, with an apical sparing. Scintigraphy showed a frequent myocardic fixation (69%), slightly more intense in TTR patients.Cardiac MRI showed a constant late gadolinium enhancement, more extended in AL patients. Conclusion We didn’t observe the differences described between CA types, probably because of a lack of statistical power.This encouraged us to develop a protocol for multidisciplinary evaluation of CA, to improve the management of this disease, and to keep on evaluating the diagnostic accuracy of these imaging modalities. [ABSTRACT FROM AUTHOR]

It is unclear whether hereditary hemorrhagic telangiectasia (HHT) patients can tolerate antithrombotic therapies (AT) including antiplatelet (AP) and/or anticoagulant (AC) agents. Primary endpoint was tolerance to AT in HHT. Secondary endpoints were to identify factors associated with major bleeding events (MBE) and premature discontinuation of AT. Retrospective multicenter study in French national HHT Registry patients exposed to AT. We included 126 patients with 180 courses of AT. Median follow-up was 24 [11–52] months. Mean age was 65.6 ± 13.1 years. The first 3 months of AT exposure had an increased risk of hospitalization for hemorrhage (p < 0.001) and transfusions (p < 0.001). MBE (n = 63) occurred more frequently in the first 3 months of AT exposure (p < 0.001). Premature discontinuation of AT occurred in 61 cases. Rate of premature discontinuation was 29 % under both AP and AT therapy but significantly higher under dual AP therapy (n = 4/7, 57 % p = 0.008). Risk factors for MBE were: age ≥ 60 years (HR 2.34 [1.12;4.87], p = 0.023), prior hospitalization in the 3 months before starting AT for hemorrhage (HR 3.59 [1.93;6.66], p < 0.001) or transfusion (HR 3.15 [1.61;6.18], p = 0.001), previous history of gastro-intestinal bleeding (HR 2.71 [1.57;4.65], p < 0.001) or MBE (HR 4.62 [2.68;7.98], p < 0.001). Frequency of MBE did not differ between groups except for a higher risk in the dual AP group (HR 3.92 [1.37;11.22], p = 0.011). Tolerance of AC or AP therapy was similar in HHT population but not dual AP therapy. We identified risk factors for MBE occurrence or premature discontinuation under AT. • Similar tolerability of antiplatelet and anticoagulant therapies in HHT • Dual antiplatelet therapy is not tolerated in HHT and should be avoided • Risk factors in HHT patients for major bleeding events (MBE) occurrence or premature discontinuation under antithrombotics are ≥60 years old, previous history of MBE or gastrointestinal bleeding, hospitalizations for bleeding in the last 3 months to help guide clinicians prescribing antithrombotic medication • Direct oral anticoagulants, notably apixaban, appear well tolerated by HHT patients [ABSTRACT FROM AUTHOR]