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Although pulmonary events are considered to be frequently associated with malignant haemopathies, they have been sparsely studied in the specific context of myelodysplastic syndromes (MDS). We aimed to describe their different types, their relative proportions and their relative effects on overall survival (OS). We conducted a multicentre retrospective cohort study. Patients with MDS (diagnosed according to the 2016 WHO classification) and pulmonary events were included. The inclusion period was 1 January 2007 to 31 December 2017 and patients were monitored until August 2019. Fifty-five hospitalized patients were included in the analysis. They had 113 separate pulmonary events. Thirteen patients (23.6%) had a systemic autoimmune disease associated with MDS. Median age at diagnosis of MDS was 77 years. Median time to onset of pulmonary events was 13 months. Pulmonary events comprised: 70 infectious diseases (62%), 27 interstitial lung diseases (23.9%), including 13 non-specific interstitial pneumonias and seven secondary organizing pneumonias or respiratory bronchiolitis–interstitial lung diseases, 10 pleural effusions (8.8%), including four cases of chronic organizing pleuritis with exudative effusion, and six pulmonary hypertensions (5.3%). The median OS of the cohort was 29 months after MDS diagnosis but OS was only 10 months after a pulmonary event. The OS was similar to that of the general myelodysplastic population. However, the occurrence of a pulmonary event appeared to be either an accelerating factor of death or an indicator for the worsening of the underlying MDS in our study. More than a third of pulmonary events were non-infectious and could be systemic manifestations of MDS.

Objective Studies assessing the prevalence of anti–RNA polymerase III (anti–RNAP III) antibodies in systemic sclerosis (SSc) have yielded a wide range of results. The aim of the present study was to describe a new SSc cohort tested for presence of anti–RNAP III and perform a systematic review and meta-analysis to assess the prevalence of anti–RNAP III in patients worldwide and the potential factors of variability. Methods Seropositivity for anti–RNAP III was evaluated in a French cohort of SSc patients. A systematic review of the literature was carried out in PubMed and EMBase. Meta-analysis was performed using available data on prevalence, clinical characteristics of SSc patients, and the types of assays used for anti–RNAP III testing. Results One hundred thirty-three French SSc patients were tested for anti–RNAP III, and a prevalence of 6–9% was found in these patients. Thirty studies representing a total population of 8,437 SSc patients were included in the meta-analysis. Prevalence of anti–RNAP III in this population was highly variable (range 0–41%). The overall pooled prevalence of anti–RNAP III was 11% (95% confidence interval 8–14), but heterogeneity was high among studies (I2 = 93%, P < 0.0001). Geographic factors such as continent or country of study origin partially explained this heterogeneity and correlated with the prevalence. No other baseline SSc characteristics were significantly correlated with the prevalence of anti–RNAP III. Conclusion Data on our new cohort and our meta-analysis of the literature confirmed that anti–RNAP III prevalence in SSc varies among centers. Geographic factors were significantly associated with prevalence, which underscores the probable implication that genetic background and environmental factors play a role. Heterogeneity among studies remained largely unexplained.