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Primary immunodeficiencies (PIDs) in adults are mainly revealed by recurrent and/or severe bacterial infections. The objective of this study was to evaluate a systematic research strategy of PIDs in adults with unexplained bacterial infections, with a special focus on specific polysaccharide antibody deficiency (SPAD).In this prospective multicenter study, inclusion criteria were recurrent benign upper and lower respiratory tract infections (RTIs) for at least two years (group 1), at least one upper or lower RTI requiring hospitalization (group 2), and/or at least one invasive infection documented with encapsulated bacteria (group 3). Main exclusion criteria were all local and general conditions that could explain infections. If no PID diagnosis was made, response to polysaccharide antigens was assessed using a pneumococcal polysaccharide vaccine.From March 2015 to March 2020, 118 patients were included (37 males, median age of 41 years): 73, 17, and 28 in groups 1, 2, and 3, respectively. Forty-seven PIDs were diagnosed, giving an estimated frequency of 39.8% (95% IC [30.4; 48.8]). SPAD was the most frequent diagnosis by far (n = 37/47, 78.7%), and was made in 23, 5, and 9 patients from groups 1 to 3, respectively. All SPAD patients received conjugate vaccines, and according to their infectious history, were on surveillance or treated with preventive antibiotics (n = 6) and/or with immunoglobulins replacement therapy (n = 10), the latter being dramatically efficient in all cases.Considering its high prevalence among adults with unexplained recurrent and/or severe bacterial infections, SPAD should be screened in those patients.

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.

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.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.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 (I(2) = 93%, P0.0001). 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.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.

Introduction Les anticorps anti-ARN polymerase de type III (anti-RNAPIII) sont parmi les anticorps anti-nucleaires les plus frequemment identifies chez les patients avec sclerodermie systemique (ScS), avec une prevalence estimee a 11 % (IC 95 % : 8–14) [1] . Les anticorps anti-RNAPIII sont associes avec des caracteristiques cliniques responsables d’un pronostic plus severe telles que la forme cutanee diffuse et la crise renale sclerodermique. Plus recemment il a ete suggere leur role potentiel dans la reponse immunologique au cancer [2] . Cette etude visait a : – confirmer le phenotype clinique des patients avec anti-RNAPIII dans une nouvelle cohorte Francaise puis dans le cadre d’une revue systematique avec meta-analyse ; – mettre en evidence des associations cliniques non connues. Patients et methodes Les anticorps anti-RNAPIII ont ete recherches chez cent trente-trois patients sclerodermiques consecutifs et non selectionnes. Nous avons recherche dans PubMed et EMBase toutes les etudes presentant des donnees sur les caracteristiques cliniques de patients avec anti-RNAPIII puis realise une meta-analyse en utilisant le nombre de patients positifs (cas) et negatifs pour la recherche d’anti-RNAPIII (controles), leurs caracteristiques cliniques et les atteintes d’organes. Resultats Douze patients presentaient des anticorps anti-RNAPIII. Ces anticorps etaient associes avec la forme cutanee diffuse (p = 0,02), l’atteinte musculaire, la crise renale et la survenue de cancer (p = 0,01). Pour la revue systematique, 2003 resumes etaient analyses et 112 references etaient selectionnees pour une lecture complete. Quarante-cinq etudes etaient finalement inclues dans la meta-analyse. Le nombre d’etudes comprenant des donnees pour chaque association clinique etait compris entre 4 et 26 soit 256 a 1098 patients avec anti-RNAPIII et 2088 a 6612 controles. Les anti-RNAPIII etaient positivement associes avec la forme cutanee diffuse, l’atteinte articulaire, la crise renale, l’atteinte cardiaque et la survenue de cancer ; negativement avec le sexe feminin. Il n’y avait pas d’association significative avec l’atteinte œsophagienne, l’hypertension pulmonaire, la fibrose pulmonaire, les ulcerations digitales et l’atteinte musculaire (Tableau 1). Conclusion Cette meta-analyse confirme que les patients sclerodermiques avec anticorps anti-RNAPIII ont un risque superieur d’atteinte cutanee diffuse, de crise renale et de cancer. L’analyse de donnees sur un grand nombre d’etudes permet de mettre en evidence des associations moins connues telles que l’atteinte articulaire et cardiaque. Ces patients devraient beneficier d’un depistage approprie en raison des complications potentiellement severes auxquelles ils sont exposes.