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This article presents the evidence for a tentative reconstruction of the theoretical and iconographic programme of Bellori’s Vite. The author seeks to show that Bellori’s biographies are arranged according to a programmatic scheme probably conceived in the late 1630s or early 1640s, during the biographer’s youth, and that this programme is inspired by Lomazzo’s Temple of Painting and the theory of Governors. Published in 1672, the Vite present the lives of twelve artists, following—up to a point—the chronological order of their deaths, as was the rule in that literary genre. However, three of them (Annibale Carracci, Caravaggio, and Domenichino) are out of chronological order, and at the same time divide the whole group into three equal parts. This anomaly is explained by a poem published in 1642 by the young Bellori (as a preface to Baglione’s Vite de’Pittori) in which he classified modern artists according to the scheme of the three Graces. In the poem, Bellori devoted a complete strophe to each of the artists he would choose as “governors” thirty years later, describing his particular genius and relating him to one of the Graces. The same kind of classification was used by the poet Antonio Bruni, who in Le Tre Gratie (1630) divided poetry into three broad categories: lyric, heroic, and moral and sacred. Each of these categories is governed by one of the Graces. Bruni asserts that his aim is to classify poetry with greater clarity and avoid the confusion generated by the mixture of different genres in a single poem. A study of the emblematic etchings that precede the biographies confirms this analysis. Apparently the Idea, which was to become so famous in European academies of art, was added to the work very late, almost at the moment of printing. This late addition was the cause of some confusion at the printer’s desk, with the result that this etching was misplaced in the text. Nevertheless the scheme of the programme appears very clearly. The three governors are associated with three concepts: Theoria, Praxis, and Conceptus-Imaginatio. The other three etchings in each group are related to the main theme and explain it. This figurative complement to the biographies testifies to the importance and vitality of such figurative and theoretical sources as Testa’s Liceo della Pittura, also designed in the late 1630s. The iconographic analysis of the etchings shows that Bellori apparently adhered closely to the three-level educational programme defined by Testa for the perfect painter, seeking at the same time to reconcile schools and styles of the modern period in his Ideal Temple of Painting. Despite what has often been said, it was not his intention to oppose Poussin to Caravaggio, nor did he aim to write mere biographies of individuals.

International audience; Background: The systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a very rare condition characterized by recurrent life-threatening episodes of vascular hyperpermeability in the presence of a monoclonal gammopathy. Extended intravenous immunoglobulin (IVIG) treatment is associated with fewer recurrences and improved survival, but the optimal treatment dosage and duration remain unknown. Objective: We aim to evaluate the safety of IVIG tapering and withdrawal in patients with SCLS. Methods: We conducted a retrospective multicenter study including all adult patients with monoclonal gammopathy–associated SCLS from the EurêClark registry who received at least 1 course of IVIG. The primary end point was overall survival according to IVIG withdrawal. Results: Fifty-nine patients of mean ± SD age 51 ± 13 years were included. Overall cumulative probabilities of 2-, 5-, 10- and 15-year survival were 100%, 85%, 72%, 44%, respectively. The IVIG was withdrawn at least once in 18 patients (31%; W+ group) and never in 41 patients (69%; W– group). Cumulative probabilities of 10-year survival in W+ versus W– groups were 50% and 83% (log rank test, P = .02), respectively. Relapse rate and the median number of relapses in the W+ versus the W– groups were 72% versus 58% (P = 0.3) and 2.5 (0.3–4) versus 1 (0–2) (P = .03), respectively. The IVIG tapering was not statistically associated with increased person-year incidence of attacks using a mixed linear model. Conclusions: The IVIG withdrawal was associated with increased mortality and higher rate of recurrence in SCLS patients. The IVIG tapering might be cautiously considered in stable SCLS patients.

ObjectiveTo report on a large series of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features.MethodsRetrospective nationwide multicenter study of patients diagnosed with both AAV and bronchiectasis.ResultsSixty-one patients were included, among whom 27 (44.25%) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis (GPA), and 7 (11.5%) had eosinophilic GPA. Thirty-nine (64%) had myeloperoxidase (MPO)-ANCA and 13 (21%) had proteinase 3–ANCA. The diagnosis of bronchiectasis either preceded (n = 25; median time between both diagnoses: 16 yrs, IQR 4–54 yrs), was concomitant to (n = 12), or followed (n = 24; median time between both diagnoses: 1, IQR 0–6 yrs) that of AAV. Patients in whom bronchiectasis precedes the onset of AAV (B-AAV group) have more frequent mononeuritis multiplex, MPA, MPO-ANCA, and a 5-fold increase of death. The occurrence of an AAV relapse tended to be protective against bronchiectasis worsening (HR 0.6, 95% CI 0.4–0.99, P = 0.049), while a diagnosis of bronchiectasis before AAV (HR 5.8, 95% CI 1.2–28.7, P = 0.03) or MPA (HR 18.1, 95% CI 2.2–146.3, P = 0.01) were associated with shorter survival during AAV follow-up.ConclusionThe association of bronchiectasis with AAV is likely not accidental and is mostly associated with MPO-ANCA. Patients in whom bronchiectasis precedes the onset of AAV tend to have distinct clinical and biological features and could carry a worse prognosis.

The anti–von Willebrand factor nanobody caplacizumab was licensed for adults with immune-mediated thrombotic thrombocytopenic purpura (iTTP) based on prospective controlled trials. However, few data are available on postmarketing surveillance. We treated 90 iTTP patients with a compassionate frontline triplet regimen associating therapeutic plasma exchange (TPE), immunosuppression with corticosteroids and rituximab, and caplacizumab. Outcomes were compared with 180 historical patients treated with the standard frontline treatment (TPE and corticosteroids, with rituximab as salvage therapy). The primary outcome was a composite of refractoriness and death within 30 days since diagnosis. Key secondary outcomes were exacerbations, time to platelet count recovery, the number of TPE, and the volume of plasma required to achieve durable remission. The percentage of patients in the triplet regimen with the composite primary outcome was 2.2% vs 12.2% in historical patients (P = .01). One elderly patient in the triplet regimen died of pulmonary embolism. Patients from this cohort experienced less exacerbations (3.4% vs 44%, P < .01); they recovered durable platelet count 1.8 times faster than historical patients (95% confidence interval, 1.41-2.36; P < .01), with fewer TPE sessions and lower plasma volumes (P < .01 both). The number of days in hospital was 41% lower in the triplet regimen than in the historical cohort (13 vs 22 days; P < .01). Caplacizumab-related adverse events occurred in 46 patients (51%), including 13 major or clinically relevant nonmajor hemorrhagic events. Associating caplacizumab to TPE and immunosuppression, by addressing the 3 processes of iTTP pathophysiology, prevents unfavorable outcomes and alleviates the burden of care.

Sarcoidosis is a rare disease of unknown cause with wide heterogeneity in clinical features and outcomes. We aimed to explore sarcoidosis phenotypes and their clinical relevance with particular attention to extrapulmonary subgroups.The Epidemiology of Sarcoidosis (EpiSarc) study is a French retrospective multicentre study. Sarcoidosis patients were identified through national hospitalisation records using appropriate codes from 11 hospital centres between 2013 and 2016 according to a standardised protocol. Medical charts were reviewed. The phenotypes of sarcoidosis were defined using a hierarchical cluster analysis.A total of 1237 patients were included (562 men and 675 women). The mean age at sarcoidosis diagnosis was 43.5±13 years. Hierarchical cluster analysis identified five distinct phenotypes according to organ involvement and disease type and symptoms: 1) erythema nodosum, joint involvement and hilar lymph nodes (n=180); 2) eye, neurological, digestive and kidney involvement (n=137); 3) pulmonary involvement with fibrosis and heart involvement (n=630); 4) lupus pernio and a high percentage of severe involvement (n=41); and 5) hepatosplenic, peripheral lymph node and bone involvement (n=249). Phenotype 1 was associated with being European/Caucasian and female and with non-manual work, phenotype 2 with being European/Caucasian, and phenotypes 3 and 5 with being non-European/Caucasian. The labour worker proportion was significantly lower in phenotype 5 than in the other phenotypes.This multicentre study confirms the existence of distinct phenotypes of sarcoidosis, with a non-random distribution of organ involvement. These phenotypes differ according to sex, geographical origin and socioprofessional category.

Introduction La maladie associee aux IgG4 (MAG4) est une pathologie fibro-inflammatoire localisee ou systemique caracterisee par des lesions histologiques typiques. D’un point de vue epidemiologique, elle touche preferentiellement l’homme avec un âge moyen au diagnostic du plus de 60 ans [1] . La survenue d’une MAG4 dans l’enfance ou chez l’adulte jeune est exceptionnelle et rarement rapportee [2] . Nous presentons une etude descriptive ainsi qu’une revue de la litterature de cas debutant avant l’âge de 25 ans. Les donnees ont ensuite ete comparees a une cohorte adulte. Materiels et methodes Les observations de notre cohorte ont ete recueillies par un appel a observations national aupres des societes savantes et du recueil d’observations national de la MAG4. Les cas de la litterature ont ete recueillies par une recherche systematique sur PubMed et GoogleScholar. Les criteres d’inclusion retenus etaient : un âge aux 1e symptomes ≤ 25 ans et un diagnostic de MAG4 retenu selon les criteres CDC [3] ou les criteres diagnostiques specifiques d’organe. Nous avons repris les donnees d’une cohortre adulte francaise respectant les memes criteres d’inclusion CDC et avec âge des 1ers symptomes > 25 ans. Resultats Deux patients etaient exclus de l’analyse en raison de diagnostics genetiques finalement retenus. Parmi les 23 patients inclus, 12 (52 %) etaient de sexe feminin. Un antecedent familial concomittant de MAG4 etait retrouve chez une patiente. Des atypies cliniques ou biologiques etaient retrouvees chez 8 patients. Une histologie etait disponible chez 21 patients (91 %), avec des seuils de plasmocytes IgG4+ eleves chez 17 d’entre eux (81 %). Des atypies histologiques etaient associees chez 4 d’entre eux. Lorsque les criteres de classification ACR/EULAR 2019 etaient appliques, 14 (60 %) avaient un score ≥ 20, mais 3 d’entre eux presentaires au moins un critere d’exclusion. Le traitement de 1ere ligne etait represente par une corticotherapie seule chez 15 patients (65 %), avec l’absence de reponse chez 1 patient. Le recours a d’autres traitements concernait 12 patients (52 %) avec une duree moyenne de suivi de 43 mois. Nous avons recense 74 observations dans la litterature, avec des donnees similaires. Lorsques les donnees etaient comparees aux adultes, on retrouvait de facon significative plus de femmes, plus de presentations febriles, et un nombre de rechute ou de 2nd ligne plus importants dans la cohorte pediatrique et juvenile. Discussion Nous rapportons ici la plus large cohorte de cas jeunes de MAG4 selon les criteres CDC et specifiques d’organes. La predominance masculine semble moins marquee dans cette population. Alors que les atteintes d’organes sont le plus souvent typiques, des atypies cliniques, biologiques ou histologiques sont retrouvees chez une proportion importante des patients. Conclusion Un diagnostic de MAG4 peut etre pose chez des patients jeunes, de moins de 25 ans. Alors qu’une partie d’entre eux semble presenter un tableau typique de la maladie, une proportion significative des patients de diagnostic pediatrique presentent des atypies, qui peuvent evoquer la possibilite de formes “secondaires”, devant faire eliminer l’ensemble des diagnostics differentiels incluant des pathologies monogeniques associees.

Objectives: The nature and impact of food and other external triggers in recurrences of Behcet's disease (BD)-related oral ulcers (OUs) remain unknown. This survey investigated dietary and non-dietary triggers of BD-related OU recurrences. Methods: Patients with BD who were followed in 7 French hospital departments completed a self-administered patient questionnaire. General and specific dietary triggering factors were sought in open questions. The questionnaire also included closed questions, notably to evaluate the effect of 6 general triggering situations and 24 selected foods. The results were expressed as number (%) of positive responses. Results: Among the 101 questionnaires distributed, 81 were usable. Among the 81 patients, 96% fulfilled the International Criteria for Behcet's Disease classification criteria and 53% qualified their OU recurrences during the previous 12 months as “very discomforting” or “discomforting”. For the 6 general situations suggested, 50 patients (62%) declared ≥1 as a “sure” trigger of OU recurrences. In both open and closed questions, the most frequent triggers were fatigue/stress (37–47% of patients) and food (32–35%). Among the 24 suggested foods, nuts (48%), pineapple (42%), peanuts (32%), Emmental cheese (30%), almonds (23%), lemons (22%), and other cheeses (21%) were the most frequently reported. The corresponding open question gave consistent findings but with lower frequencies. Conclusions: Most patients can identify triggers of recurring BD-related OUs, with fatigue/stress and food representing the most frequent triggers. The management of OU must consider such external factors. The histamine-rich or -liberating properties of the commonly cited OU-triggering foods suggest a hyperreactivity mechanism. This article is protected by copyright. All rights reserved.

Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB.Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies.214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7-32.7]) and prematurity (p=0.002; HR 17.1 [95%CI 2.8-103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with child death were in utero DCM (p=0.0157; HR 6.37 [95%CI: 1.25-32.44]), postnatal DCM (p0.0001; HR 227.58[95%CI: 24.33-2128.46]) and pacemaker implantation (p=0.0035; HR 0.11[95%CI: 0.02-0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB.In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids.

Introduction: Although the clinical expression of sarcoidosis in each organ has been well described, there are few data on the associations among the various disorders. Methods: 1237 patients (45% men, mean age=43.5±13 yrs) were recruited through the French Medical Information Systems Program (PMSI) databases from January 2013 to December 2016. Medical records were reviewed by a panel of experts to validate inclusion criteria (sarcoidosis with histological documentation and at least one extra-pulmonary disease, or Lofgren9s syndrome) and determine patient characteristics. Results: Cluster analysis identified 5 phenotypes. Cluster 1 (n=180) associating erythema nodosum and joint involvement, characterized by female sex (F:M ratio=2.4), being more European (51%) and less manual workers (30%). This phenotype was the less treated (corticotherapy (53% of cases), anti-TNF alfa (4%)). Cluster 2 (n=137) associating nervous system involvement and uveitis, characterized by being mainly European (62.5%), men (F:M=1.3) and manual workers (44%). Cluster 3 (n=630) identified a preferential association of pulmonary lesions, sometimes fibrosing, with cardiac lesions associated mainly with a 1.1 F:M ratio, being manual workers (46%) and non-European (58%). Cluster 4 (n=41) marked by the presence of lupus pernio occurring more in non-Europeans (80%). This was the most commonly treated phenotype (corticotherapy (88%), anti-TNF alfa (41%)). Cluster 5 (n=249) associating hepato-splenic involvement with extra-mediastinal lymphadenopathy, characterized by being mainly non-European (62.5%), women (F:M=1.1) and manual workers (35%). Conclusion: We identified 5 clinical phenotypes of sarcoidosis with distinct demographic features and outcomes.

International audience; Background: Episodic Angioedema with eosinophilia (EAE, Gleich's syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia and frequent elevated serum Immunoglobin M.Methods: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France.Results: Thirty patients were included with a median age at diagnosis of 41 years [5-84]. The median duration of each crisis was 5.5 days [1-90] with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%) among which 5 (17%) showed evidence of clonal TCR γ gene rearrangement. Median follow-up duration was 53 months [31-99]. The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio 4.15 [CI 95% 1.18-14.66; p=0.02). At last follow-up, 3 patients (10%) were able to withdraw all treatments and 11 (37%) were in clinical and biological remission with less than 10 mg of daily prednisone.Conclusion: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.

Objective To describe the epidemiology of primary Sjogren's syndrome (SS) in a multiracial/multiethnic population. Methods A cross-sectional study with 5 case-retrieval sources identified adults with primary SS living in the Greater Paris area (population 1,172,482 adults) in 2007. Diagnoses were verified by the American–European Consensus Group (AECG) criteria and study-specific enlarged criteria based on the presence of ≥3 of 4 AECG items among subjective oral or ocular dryness, anti-SSA/SSB positivity, and positive minor salivary gland biopsy results. Prevalence estimates were standardized to those for the world population and a 5-source capture–recapture analysis (CRA) was used. Racial/ethnic differences in primary SS features were evaluated. Results In all, 133 subjects met the AECG criteria and 203 met the enlarged criteria. The 2007 prevalence of primary SS was 1.02 cases per 10,000 adults (95% confidence interval [95% CI] 0.85–1.22) for the AECG criteria and 1.52 cases per 10,000 adults (95% CI 1.30–1.76) for the enlarged criteria. The CRA indicated completeness of case findings of ∼90%. Compared to subjects with European backgrounds, those with non-European backgrounds had 2.1–2.3 times higher primary SS prevalence and were younger (P < 0.0001) and were more likely to have polyclonal hypergammaglobulinemia (P < 0.0001) and anti-SSA/SSB antibodies (P = 0.0005 and P < 0.0001 for the AECG and enlarged criteria, respectively). Conclusion The figure of 1.02–1.52 cases per 10,000 adults we found and estimates from the few other population-based census surveys support that the prevalence of diagnosed primary SS is between 1 and 9 cases per 10,000 people (0.01–0.9%) in the general population. Non-European race/ethnicity may be associated with increased primary SS risk and a distinct disease profile.

International audience; Background: Monoclonal gammopathy-associated systemic capillary-leak syndrome, also known as Clarkson disease, is a rare condition characterized by recurrent life-threatening episodes of capillary hyperpermeability in the context of a monoclonal gammopathy. This study was conducted to better describe the clinical characteristics, natural history, and long-term outcome of monoclonal gammopathy-associated systemic capillary-leak syndrome.Methods: We conducted a cohort analysis of all patients included in the European Clarkson disease (EurêClark) registry between January 1997 and March 2016. From diagnosis to last follow-up, studied outcomes (eg, the frequency and severity of attacks, death, and evolution toward multiple myeloma) and the type of preventive treatments administered were monitored every 6 months.Results: Sixty-nine patients (M/F sex ratio 1:1; mean ± SD age at disease onset 52 ± 12 years) were included in the study. All patients had monoclonal gammopathy of immunoglobulin G type, with kappa light chains in 47 (68%). Median (interquartile range) follow-up duration was 5.1 (2.5-9.7) years. Twenty-four patients (35%) died after 3.3 (0.9-8) years. Fifty-seven (86%) patients received at least one preventive treatment, including intravenous immunoglobulins (IVIg) n = 48 (73.8%), theophylline n = 22 (33.8%), terbutaline n = 22 (33.8%), and thalidomide n = 5 (7.7%). In the 65 patients with follow-up, 5- and 10-year survival rates were 78% (n = 35) and 69% (n = 17), respectively. Multivariate analysis found preventive treatment with IVIg (hazard ratio 0.27; 95% confidence interval, 0.10-0.70; P = .007) and terbutaline (hazard ratio 0.35; 95% confidence interval, 0.13-0.96; P = .041) to be independent predictors of mortality.Conclusions: We describe the largest cohort to date of patients with well-defined monoclonal gammopathy-associated systemic capillary-leak syndrome. Preventive treatment with IVIg was the strongest factor associated with survival, suggesting the use of IVIg as the first line in prevention therapy.

Introduction Des associations sporadiques de dilatations des bronches (DDB) et de vascularite a ANCA (VAA) ont deja ete decrites. Certaines observations permettent de suspecter un lien entre ces deux pathologies. L’objectif de notre etude etait de decrire les caracteristiques cliniques, biologiques et radiologiques de patients atteints de DDB et de VAA, ainsi que leur profil evolutif et leur pronostic. Methodes Etude retrospective des patients ayant une VAA selon les criteres de l’ACR et des DDB confirmees au scanner. Les dossiers etaient issus de la cohorte du Groupe francais d’etude des vascularites (GFEV) ou d’un appel national a observations aupres des societes francaises de pneumologie et de medecine interne (13 centres). Resultats Soixante et un patients ont ete inclus : 27 (44 %) polyangeite microscopique (PAM), 26 (43 %) granulomatose avec polyangeite (GPA), 7 (2 %) granulomatose eosinophilique avec polyangeite (GEPA) et 1 VAA indifferenciee. Quarante-cinq patients (74 %) avaient des symptomes respiratoires au diagnostic de la VAA. Les autres manifestations etaient renales ( n = 20, 33 %), neurologiques ( n = 26, 43 %), rhinosinusiennes ( n = 31, 51) et rhumatologiques ( n = 29, 48 %). Les DDB etaient sans etiologies connues chez 40 (65,5 %) et considerees post-infectieuses chez 15 (24,5 %). La mediane de survie etait de 7 ans [4–13], avec 9 deces (15 %). Trois des deces etaient dus a une insuffisance respiratoire (33 %), et trois a une complication directe de la VAA (33 %). Une exacerbation necessitant un traitement survenait chez 32 patients (52 % ; taux median d’exacerbation = 0,35/an [0,2–1]). Quelles soient diagnostiquees avant ( n = 25, 41 %), concomitamment ou apres le diagnostic de VAA ( n = 36, 59 %), les DDB avaient les memes caracteristiques selon le score radiologique de Bhala. Par contre, les DDB connues avant la VAA etaient plus souvent considerees de cause post-infectieuse (48 % vs 8 %, p p = 0,02) et la mortalite plus importante (32 % vs 3 %, p = 0,02). Conclusion Cette 1 re serie de patients presentant l’association VAA et DDB semble suggerer que les DDB apparaissant au cours du suivi d’une vascularite pourraient etre une manifestation pulmonaire specifique de la VAA. Par ailleurs, l’existence de DDB connues chez un patient a une influence sur la presentation phenotypique des VAA et leur pronostic.

Introduction La maladie de Behcet (MB) est une maladie inflammatoire pouvant affecter les vaisseaux (arteres et veines) de tous calibres. Le tabagisme est un facteur de risque cardiovasculaire majeur et predispose aussi aux thromboses veineuses profondes. Dans la maladie de Behcet, le tabagisme pourrait jouer un role anti-inflammatoire sur la muqueuse buccale. L’objectif de ce travail est d’etudier l’impact du tabagisme sur la presentation clinique et le pronostic dans la MB. Patients et methodes Nous avons etudie retrospectivement des patients atteints de MB, remplissant les criteres internationaux de 2013, provenant de 7 services de medecine interne. Un recueil de donnees standardisees des caracteristiques demographiques, cliniques, evolutives et de la consommation tabagique a ete realise. L’intensite en paquets–annees (PA) et la poursuite du tabagisme ont ete definies aux dernieres nouvelles. Des comparaisons des caracteristiques cliniques et pronostiques selon le tabagisme et son intensite ont ete realisees, ainsi qu’une analyse des courbes de survie de Kaplan–Meier et une regression selon le modele de Cox. Resultats Parmi 496 patients inclus, 44 ont ete exclus pour donnees manquantes sur le tabagisme. Au total 452 patients (âge moyen : 41 ans, sexe ratio (H/F) : 1,1) ont ete analyses. Une consommation tabagique etait trouvee chez 261 patients (46 %) et etait active aux dernieres nouvelles (n = 183, 32 %). Le nombre median (etendue) de PA etait de 16 (1–50). Par rapport aux non-fumeurs, les patients fumeurs avaient, aux dernieres nouvelles, moins d’aphtes genitaux (64 % vs 74 % ; p 20 PA, moins d’atteinte rhumatologique (57 %, 69 %, 81 % ; p 20 PA de 1,92 (1,3–2,8 ; p Conclusion Cette etude suggere un role deletere du tabagisme au cours de la MB, notamment, au-dela d’une consommation superieure a 20 PA, avec une presentation clinique plus severe. Au cours du suivi, le pronostic semble aussi plus defavorable chez les fumeurs.

The nature and impact of food and other external triggers in recurrences of Behçet's disease (BD)-related oral ulcers (OUs) remain unknown. This survey investigated dietary and nondietary triggers of BD-related OU recurrences.Patients with BD who were followed in 7 French hospital departments completed a self-administered patient questionnaire. General and specific dietary triggering factors were sought in open questions. The questionnaire also included closed questions, notably to evaluate the effect of 6 general triggering situations and 24 selected foods. The results were expressed as number (percentage) of positive responses.Among the 101 questionnaires distributed, 81 were usable. Among the 81 patients, 96% fulfilled the International Criteria for Behçet's Disease classification criteria, and 53% qualified their OU recurrences during the previous 12 months as very discomforting or discomforting. For the 6 general situations suggested, 50 patients (62%) declared ≥1 as a "sure" trigger of OU recurrences. In both open and closed questions, the most frequent triggers were fatigue/stress (37-47% of patients) and food (32-35%). Among the 24 suggested foods, nuts (48%), pineapple (42%), peanuts (32%), Emmental cheese (30%), almonds (23%), lemons (22%), and other cheeses (21%) were the most frequently reported. The corresponding open question gave consistent findings but with lower frequencies.Most patients can identify triggers of recurring BD-related OUs, with fatigue/stress and food representing the most frequent triggers. The management of OU must consider such external factors. The histamine-rich or -liberating properties of the commonly cited OU-triggering foods suggest a hyperreactivity mechanism.

The aim of this study was to determine the characteristics, treatment, and outcome according to each etiology of pachymeningitis.We conducted a retrospective multicenter French nationwide study between 2000 and 2016 to describe the characteristics, outcome, and treatment of pachymeningitis.We included 60 patients (median age 55.5 years; interquartile range [IQR] 30-80, female/male ratio 0.43). Neurologic signs were present in 59 patients (98%) and consisted of headache in 43 (72%), cranial nerve palsy in 33 (55%), confusion in 10 (17%), seizures in 7 (12%), and focal neurologic signs in 9 (15%). Fever and weight loss were present in 8 (13%) and 13 cases (22%), respectively. Cerebral venous thrombosis was present in 8 cases (13%). Analysis of cerebrospinal fluid showed moderate hyperproteinorachia (median 0.68 g/L; IQR 0.46-3.2) with or without pleiocytosis. Diagnosis included idiopathic pachymeningitis (n = 18; 30%); granulomatosis with polyangiitis (n = 13; 17%); Erdheim-Chester disease (n = 10; 17%); IgG4-related disease and tuberculosis (n = 3; 5% each); Rosai-Dofman disease, microscopic polyangiitis, and sarcoidosis (n = 2, 3% each); cryptococcal meningitis, Lyme disease, ear-nose-throat infection, postlumbar puncture, low spinal-fluid pressure syndrome, and lymphoma (n = 1 each). We found no difference in demographics and neurologic presentation among idiopathic pachymeningitis, Erdheim-Chester disease, and granulomatosis with polyangiitis. In contrast, frequencies were lower with idiopathic pachymeningitis than Erdheim-Chester disease for general signs (6% and 40%, respectively, P = .041) and complete neurologic response (0% vs 39%, P = .045).The detection of extraneurologic signs and routine screening are needed to classify the pachymeningitis origin. Prospective studies are warranted to determine the best treatment in each case.

Introduction La maladie de Behcet (MB) est une maladie inflammatoire dont le tableau clinique et la severite sont tres variables variant d’une aphtose bipolaire, a une vascularite retinienne ou un anevrisme. De nombreuses manifestations sont possibles, avec certaines associations preferentiellement decrites, comme l’atteinte articulaire, les papulo-pustules, la presence de l’antigene HLA B51 et le sexe masculin. Nous avons mene une etude utilisant la methode d’analyse en « cluster hierarchique » pour etudier l’existence de sous-groupes phenotypiques homogenes de MB. Patients et methodes Les patients etudies atteints de MB, remplissant les criteres internationaux de 2013, provenaient de 7 services de medecine interne. Ils avaient fait l’objet d’un recueil retrospectif de donnees standardisee de leurs caracteristiques demographiques, cliniques et evolutives. Une analyse en cluster hierarchique basee sur la methode de Ward a ete realisee en implementant les 5 variables suivantes presentes au diagnostic ou au cours du suivi : uveite, thrombose veineuse profonde, atteintes arterielle, rhumatologique et neurologique. Une comparaison des caracteristiques cliniques des groupes a ete realisee. Resultats Au total, 496 patients (âge moyen : 41 ans, sexe ratio [H/F] : 1), ont ete inclus. Les principales caracteristiques cliniques sont : aphtose orale (98 %), genitale (71 %), atteinte cutanee (72 %), pseudo folliculites (71 %), erytheme noueux (21 %), atteinte articulaire (62 %), atteinte axiale (19 %), uveite (54 %), uveite anterieure isolee (24 % des yeux atteints), atteinte neurologique centrale (24 %), atteinte psychiatrique (23 %), thrombose veineuse profonde (20 %), atteinte arterielle (12 %), atteinte digestive (11 %) et HLA B5 ou B51 (54 %). Cinq clusters de patients ont ete identifies (clusters 1 a 5) qui se caracterisaient de la facon suivante : cluster 1 (n = 123, 25 %), atteinte ophtalmologique (74 %) sans atteinte rhumatologique, neurologique ou vasculaire ; cluster 2 (n = 169, 34 %), atteinte rhumatologique (100 %) sans atteinte neurologique ou vasculaire ; cluster 3 (n = 69, 14 %), atteinte neurologique centrale (100 %) sans atteinte vasculaire ; cluster 4 (n = 76, 15 %), atteinte vasculaire veineuse (100 %) sans atteinte arterielle ; enfin, cluster 5 (n = 59, 11 %), caracterise par une atteinte arterielle (arterite ou anevrisme). Les caracteristiques demographiques et cliniques etaient differentes selon les clusters : sexe masculin (64 %, 43 %, 48 %, 43 % et 61 %, respectivement ; p Conclusion Cette analyse en « cluster » suggere que la MB se decompose en 5 phenotypes cliniques : ophtalmologique, rhumatologique, neurologique non vasculaire, veineux et arteriel. Bien qu’un patient puisse changer de groupe au cours du suivi, cette categorisation pourra permettre d’apporter des elements utiles pour la prise en charge et le suivi de ces patients. Cette « categorisation » devra etre confirmee avec un plus grand nombre de patients et dans d’autres populations.

Objective To estimate the prevalence of Behcet's disease (BD) in a multiethnic population living in France, with particular focus on disease risk among immigrants. Methods The study was conducted in a county in the Paris metropolitan area that is home to 1,094,412 adults (ages ≥15 years), of whom 26% are of non-European ancestry. Patients with BD living in this area during 2003 were identified using 3 sources (hospitals, community physicians, and the National Health Insurance database), and diagnoses were verified using the International Study Group criteria. Standardized, year-2003 prevalence rates were computed for the overall population and for each ethnic group. Stratified prevalence rates according to age at immigration to France were calculated to investigate the relationship between age at immigration and BD risk. Results Seventy-nine subjects fulfilled our search criteria. The overall prevalence per 100,000 adults was 7.1 (95% confidence interval [95% CI] 3.5–14.4), and the prevalence for populations of European, North African, and Asian ancestry was 2.4 (95% CI 0.6–7.2), 34.6 (95% CI 24.4–47.5), and 17.5 (95% CI 10.7–27.2), respectively. Within the migrant population of either North African or Asian ancestry, BD prevalences were similar for residents born in France, residents

Churg-Strauss syndrome is a systemic necrotizing vasculitis involving small and medium-sized vessels. Classic features include asthma and hypereosinophilia. Antineutrophil cytoplasm antibodies (ANCA) are detected in about 40% of patients. Churg-Strauss syndrome has been reported in patients receiving leukotriene modifiers for asthma, in particular, leukotriene receptor antagonists (LTRA) (montelukast, zafirlukast or pranlukast). Clinical manifestations cases do not differ in these cases from those in Churg-Strauss syndrome without antileukotriene exposure. It is increasingly less likely that LTRA is the direct cause of this syndrome in those patients, although this hypothesis has not been completely ruled out. In many patients, LTRA treatment is prescribed because of worsening asthma, which is an early sign of Churg-Strauss syndrome. LTRA for asthma patients should be prescribed with great care, especially in cases of atypical or rapidly aggravated asthma. The onset of Churg-Strauss syndrome in patients treated with LTRA usually requires that they stop this treatment. Prescription of LTRA In patients with Churg-Strauss syndrome should be discussed with specialists.

A RARE EVENTUALITY: Although parasite infections are frequent, observations of vasculitis related to parasitosis are, however, very rare. REGARDING THE MECHANISM: The simultaneous occurrence of a parasitosis and vasculitis may be the consequence of either the direct implication of a parasite observed in the histological lesions in the onset of alteration in the vascular wall, or of immunopathological phenomena occuring during the anti-parasite immune response, or a fortuitous association. THE HUMAN PARASITOSIS IMPLIED: In most cases, vasculitis associated with parasitosis is an isolated event with varied clinical aspects. Such cases have been reported in toxoplasmosis, trichinosis, strongyliasis, ascaridiosis, sarcocystosis, amibiasis, leishmaniosis and toxocarosis.

The objective of the study was to estimate the prevalence and incidence of interstitial lung diseases (ILDs) in Seine-Saint-Denis, a multi-ethnic county of Greater Paris, France. Patients with ILDs were identified between January and December 2012 by using several sources; all potentially involved medical specialists from public and private hospitals, community-based pulmonologists and general practitioners, and the Social Security system. Diagnoses were validated centrally by an expert multidisciplinary discussion. 1170 ILD cases were reported (crude overall prevalence: 97.9/105 and incidence: 19.4/105/year). In the 848 reviewed cases, the most prevalent diagnoses were sarcoidosis (42.6%), connective tissue diseases associated ILDs (CTDs-ILDs) (16%), idiopathic pulmonary fibrosis (IPF) (11.6%), and occupational ILDs (5.0%), which corresponded to a crude prevalence of 30.2/105 for sarcoidosis, 12.1/105 for CTDs-ILDs and 8.2/105 for IPF. The prevalence of fibrotic idiopathic interstitial pneumonias, merging IPF, nonspecific interstitial pneumonia and cases registered with code J84.1 was 16.34/105. An adjusted multinomial model demonstrated an increased risk of sarcoidosis in North Africans and Afro-Caribbeans and of CTDs-ILDs in Afro-Caribbeans, compared to that in Europeans. This study, with a comprehensive recruitment and stringent diagnostic criteria, emphasises the importance of secondary ILDs, particularly CTDs-ILDs and the relatively low prevalence of IPF, and confirms that sarcoidosis is a rare disease in France., ILD prevalence reaches 97.9/100000, with a fairly low prevalence of IPF and a high contribution of secondary ILDs http://ow.ly/vLXq30bssKR

Auteur(s) : Veronique Verge1, Francois Lhote2, Robert Mary3, Bruno Verdiere4, Francoise Baledent1 1Laboratoire d’hematologie, Centre hospitalier de Saint-Denis, Saint-Denis 2Service de medecine interne, Centre hospitalier de Saint-Denis, Saint-Denis 3Urgences adultes, Centre hospitalier de Saint-Denis, Saint-Denis 4Reanimation, Centre hospitalier de Saint-Denis, Saint-Denis Observation clinico-biologique M. D., 44 ans, d’origine ivoirienne, consulte aux [...]

Objective To retrospectively analyze the clinical symptoms, laboratory findings, and outcomes in patients with microscopic polyangiitis (MPA) who were enrolled in various clinical trials conducted by the French Vasculitis Study Group. Methods A cohort of 85 patients meeting the Chapel Hill criteria for MPA participated in the study. Seventy-one of them were included in prospective therapeutic trials. Eighty-one diagnoses were biopsy proven. In the other patients, diagnosis was based on clinical findings. Results Forty-seven men and 38 women, with a mean ± SD age of 56.8 ± 14.6 years, met the criteria for MPA. Their main clinical symptoms were renal manifestations (78.8%), weight loss (72.9%), skin involvement (62.4%), fever (55.3%), mononeuritis multiplex (57.6%), arthralgias (50.6%), myalgias (48.2%), hypertension (34.1%), lung involvement (24.7%; alveolar hemorrhage 11.8%), and cardiac failure (17.6%). The mean ± SD serum creatinine level before treatment was 2.59 ± 2.96 mg/dl; 47 patients had renal insufficiency (serum creatinine >1.36 mg/dl). Eight patients underwent dialysis at the time of diagnosis, and long-term dialysis was necessary for 10 patients. Antineutrophil cytoplasmic antibodies (ANCA) were present in 38 of 51 patients (74.5%), of whom 33 had a perinuclear staining pattern (pANCA) and 5 had a cytoplasmic pattern. Antibodies to proteinase 3 were present in 4 patients and antibodies to myeloperoxidase were detected in 31, as determined by enzyme-linked immunosorbent assay. Of the 30 patients who underwent renal and celiac angiography, 4 had microaneurysms. Of the 29 patients (34.1%) who had relapses, 8 died during or after the relapse. During followup, 28 of the 85 patients (32.9%) died. The mean ± SD duration of followup of the group was 69.9 ± 60.6 months. Deaths were less frequent when patients had been treated with steroids and immunosuppressive drugs (13 patients [24.1%]) than with steroids alone (15 patients [48.4%]) (P < 0.01). The 5-year survival rate was 74%. Conclusion This study demonstrated that MPA is a multisystemic disease in which renal symptoms are frequent, but the disease is also associated with general symptoms, arthritis, mononeuritis multiplex, and other manifestations that are also seen in various vasculitides. The rarity of abnormal angiogram findings and the high frequency of pANCA are characteristic of MPA. In most cases, the outcome is comparable with those of other systemic vasculitides, but relapses are frequent.

Churg-Strauss syndrome (CSS) is a systemic vasculitis characterized by the presence of asthma, hypereosinophilia, and necrotizing vasculitis with extravascular eosinophil granulomas. In this retrospective study of 96 patients between 1963 and 1995, we analyzed clinical manifestations, identified prognostic factors, and assessed the long-term outcome. CSS was diagnosed when asthma, hypereosinophilia > 1,500/mm3 or > 10%, and clinical manifestations consistent with systemic vasculitis, with or without histologic evidence, were present. Asthma was the most frequently observed manifestation at presentation, with mononeuritis multiplex the second. Other common manifestations were weight loss, fever, myalgia, skin involvement, paranasal sinusitis, arthralgia, pulmonary infiltrate, and gastrointestinal involvement. Mean eosinophilia at presentation was 7.193 +/- 6.706/mm3; ANCA, present in 20 of 42 (47.6%) patients, predominantly gave the perinuclear labeling pattern. All the patients were treated with corticosteroids alone or in combination with cyclophosphamide or plasma exchanges. Clinical remission was obtained in 91.5%; 22 (25.6%) patients relapsed. Twenty-three patients died during follow-up: 11 of these deaths were directly due to vasculitis. The presence of severe gastrointestinal tract or myocardial involvement was significantly associated with a poor clinical outcome. The long-term prognosis of CSS is good and does not differ from that of polyarteritis nodosa, although most patients need low doses of oral corticosteroids for persistent asthma, even many years after clinical recovery from vasculitis.

In this article, three systemic vasculitides--polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome--are reviewed, focusing on advances in the understanding of the pathogenesis, new classification nomenclature, and major clinical features of these vasculitides. Distinguishing criteria between polyarteritis nodosa and microscopic polyangiitis are also presented in detail, and recent advances in the clinical management of these diseases according to appropriate therapeutic strategies are recommended.

Polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS) all have neurologic symptoms and share characteristics and outcomes. Clinical aspects, neurologic manifestations, and treatment of these three diseases are examined in this article.

To describe the epidemiology of primary Sjögren's syndrome (SS) in a multiracial/multiethnic population.A cross-sectional study with 5 case-retrieval sources identified adults with primary SS living in the Greater Paris area (population 1,172,482 adults) in 2007. Diagnoses were verified by the American-European Consensus Group (AECG) criteria and study-specific enlarged criteria based on the presence of ≥3 of 4 AECG items among subjective oral or ocular dryness, anti-SSA/SSB positivity, and positive minor salivary gland biopsy results. Prevalence estimates were standardized to those for the world population and a 5-source capture-recapture analysis (CRA) was used. Racial/ethnic differences in primary SS features were evaluated.In all, 133 subjects met the AECG criteria and 203 met the enlarged criteria. The 2007 prevalence of primary SS was 1.02 cases per 10,000 adults (95% confidence interval [95% CI] 0.85-1.22) for the AECG criteria and 1.52 cases per 10,000 adults (95% CI 1.30-1.76) for the enlarged criteria. The CRA indicated completeness of case findings of ∼90%. Compared to subjects with European backgrounds, those with non-European backgrounds had 2.1-2.3 times higher primary SS prevalence and were younger (P0.0001) and were more likely to have polyclonal hypergammaglobulinemia (P0.0001) and anti-SSA/SSB antibodies (P = 0.0005 and P0.0001 for the AECG and enlarged criteria, respectively).The figure of 1.02–1.52 cases per 10,000 adults we found and estimates from the few other population-based census surveys support that the prevalence of diagnosed primary SS is between 1 and 9 cases per 10,000 (0.01-0.09%) [corrected] in the general population. Non-European race/ethnicity may be associated with increased primary SS risk and a distinct disease profile.

Since 1977, plasma exchanges (PE) have been proposed to treat immune-mediated disease, especially when an immune complex-induced mechanism is suspected, including vasculitis and particularly polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS). The rationale for the use of PE is to remove circulating pathogenic factors, such as immune complexes and their components (antigens and antibodies). The indications of PE in the treatment of PAN without hepatitis B virus (HBV) infection and CSS have recently been clarified by two prospective therapeutic studies. There is presently no argument to support the systematic prescription of PE at the time of diagnosis of PAN without HBV infection and CSS, even for patients with factors of poor prognosis. PE may be useful as a second-line treatment in patients with refractory vasculitis and sometimes allowed, in association with corticosteroids and immunosuppressive drugs, recovery from PAN in some clinically severely compromised patients. HBV-related PAN is the best indication of PE in the treatment of PAN and is, in combination with antiviral therapy, the first-line treatment of this particular form of vasculitis. Among 41 patients with HBV-related PAN treated with PE and antiviral therapy (vidarabine in 35, interferon-alpha 2b in 6), the 7 year survival rate was 83%. HBeAg/HBeAb seroconversion was obtained in 51.2% and total virus clearance (HBeAg/HBeAb and HBsAg/HBsAb seroconversions) was seen in 24.4%. These results are much better than those obtained under corticosteroids ± cyclophosphamide ± PE, with which seroconversion was rare. There are presently no convincing data to support the prescription of PE in Wegener's granulomatosis and microscopic polyangiitis, even in the more severe forms, except in dialysis-dependent patients and further prospective trials are needed to determine the therapeutic value of PE, especially for severe glomerulonephritis in anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. Side effects of PE in the treatment of PAN and CSS became more rare with technological improvements. In our experience of 182 patients with PAN and CSS treated with PE, none died during a session and the side effects and complications due to PE were usually mild and transient.

Hepatitis B virus (HBV)-related polyarteritis nodosa (PAN) is a rare disease whose frequency has been decreasing over the past 10 years. We evaluated 41 patients with HBV-related PAN to determine the circumstances leading to infection, the clinical features of vasculitis, the prognostic factors, and the response to therapy. Most patients were first treated briefly with corticosteroids, and all were included in 2 nonrandomized prospective therapeutic trials of an antiviral agent (35 patients with vidarabine, 6 patients with interferon-alpha 2b) and plasma exchanges. The mean duration of follow-up was 69.6 +/- 44.8 months. At the end of the study, 21 (51.2%) patients had seroconverted to anti-HBeAb and 10 (24.4%) also had seroconverted to anti-HBsAb. In all, 23 (56%) patients no longer expressed serologic evidence of HBV replication. All 33 (80.5%) patients still alive at the end of follow-up recovered from PAN. Nineteen also recovered from HBV infection and were considered to be cured; 13 patients had persistent HBV infection and were considered to be in clinical recovery; and 1 patient was in remission, maintained with steroid therapy. Eight patients died during the study period; 3 deaths were directly attributable to PAN. HBV-related PAN is an acute disease, occurring shortly after infection and sharing the characteristics of classic PAN. It is not an antineutrophil cytoplasm antibodies (ANCA)-mediated vasculitis. The outcome was good for patients treated with short-term steroid therapy, antiviral agents, and plasma exchanges. We propose this protocol as the first treatment for HBV-related PAN, because it surpasses the conventional treatment with corticosteroids and cyclophosphamide, which facilitates viral replication and the development of chronic HBV infection.

Introduction Le syndrome de fuite capillaire idiopathique, ou syndrome de Clarkson, est une maladie rare. Plusieurs traitements preventifs de la recidive des crises ont ete proposes. Les imunoglobulines polyvalentes intraveineuses (IgIV) semblent etre efficaces sans que leur effet n’ait pu etre parfaitement demontre. Objectif Evaluer l’efficacite du traitement preventif par IgIV sur la survie sans crise, la survenue de crise, de crise grave et sur la mortalite au cours du syndrome de fuite capillaire idiopathique. Analyse retrospective des donnees du registre europeen EureClark. Le diagnostic de syndrome de fuite capillaire idiopathique etait retenu devant au moins une crise definie par des manifestations liees a une hypovolemie aigue (malaise, lipothymie, insuffisance renale aigue) et a une fuite capillaire simultanee (œdemes diffus, manifestations digestives, musculaires ou cardiaques) avec une hemoconcentration et une hypoalbuminemie paradoxale associee(s) a une gammapathie monoclonale. Une crise severe etait definie comme la survenue d’une hypotension arterielle (pression arterielle systolique Resultats Entre janvier 1982 et novembre 2015, 67 patients repondant aux criteres diagnostiques et ayant des informations de suivi suffisantes ont ete analysees. Quarante-six malades ont ete traites par IgIV en traitement preventif des crises. Le traitement par IgIV a ete associe a une diminution significative du nombre global de crises 1 [0–4,25] apres IgIV contre 3 [1–6] avant IgIV (p = 0,003) et des crises severes 1 [1–2,5] contre 0 [0–1] (p Conclusion Au cours du syndrome de fuite capillaire idiopathique, un traitement prophylactique mensuel par IgIV diminue significativement le risque de crises et de crises graves.

Introduction Le syndrome de fuite capillaire idiopathique, ou syndrome de Clarkson, est une maladie rare. Il n’existe que peu de donnees concernant la survie des malades et leur evolution au long cours sous traitement preventif. Objectif Connaitre la survie au long cours, les causes de deces et les facteurs associes a la mortalite dans le syndrome de fuite capillaire idiopathique. Analyse retrospective des donnees du registre europeen EureClark. Le diagnostic de syndrome de fuite capillaire idiopathique etait retenu devant au moins une crise definie par des manifestations liees a une hypovolemie aigue (malaise, lipothymie, insuffisance renale aigue.) et a une fuite capillaire simultanee (œdemes diffus, manifestations digestives, musculaires ou cardiaques) avec une hemoconcentration et une hypoalbuminemie paradoxale associee(s) a une gammapathie monoclonale. Une crise severe etait definie comme la survenue d’une hypotension arterielle (pression arterielle systolique Resultats Entre janvier 1982 et novembre 2015, 67 patients repondant aux criteres diagnostiques et ayant des informations de suivi suffisantes ont ete analyses. L’âge au diagnostic etait de 54 ± 12 ans, le sex-ratio de 1 :1 et le delai diagnostique de 238 [4–670] jours. Une gammapathie IgG etait presente chez tous les malades avec une chaine legere kappa (n = 47 ; 70 %) ou lambda (n = 21 ; 31 %). La duree de suivi etait de 5,3 [2,6–10,8] ans pendant lequel les patients ont eu 0,4 [0–1] crises/an dont 0,07 [0–0,4] graves/an. Cinquante-quatre (80 %) malades ont recu un traitement prophylactique au cours du suivi : immunoglobulines polyvalentes intraveineuses (IgIV) n = 46 (68 %), theophylline n = 22 (33 %), terbutaline n = 22 (33 %), thalidomide n = 5 (7 %), pentoxifylline n = 2 (3 %). Dix-huit (27 %) malades ont recu plus d’un traitement et 13 (19 %) aucun traitement. Vingt-trois (34 %) malades sont decedes apres un suivi de 3,4 [0,8–8,7] ans dont 4 lors de leur premiere crise et 4 des suites d’un myelome multiple. La probabilite de survie au cours du syndrome de fuite capillaire idiopathique a 1, 5, 10 et 15 ans etait respectivement de 91 %, 75 %, 67 %, 48 %. En analyse univariee, excluant les malades decedes au diagnostic (n = 4), les facteurs associes a la mortalite etaient la presence d’un traitement preventif specifique de la rechute (HR 0,3 [0,1–0,9] p = 0,03) dont le traitement par IgIV (HR 0,26 [0,1–0,6] p = 0,004) mais aucun des autres traitements preventifs. Le nombre de crise graves apres le diagnostic ou le myelome n’etaient pas associes a la mortalite. En analyse multivariee incluant le type de chaine legere, le myelome, le traitement preventif par IgIV, la presence de crises subintrantes apres le diagnostic et le nombre de crise graves apres le diagnostic, seul restait associe a la mortalite le traitement preventif par IgIV (HR 0,1[0,04–0,5] p = 0,003). Conclusion Dans notre cohorte la probabilite de survie a 10 ans au cours du syndrome de fuite capillaire idiopathique etait de 67 %. Seul le traitement preventif par IgIV etait significativement associe a la survie.

Background Recurrent oral ulcers (ROU) are an almost constant feature of Behcet9s disease (BD) but its pathophysiology is not well understood. It is widely admitted that certain foods or other external factors play a role in BD-related ROU. Thus, the proportion of patients with BD able to identify a specific triggering factor of their ROU and the nature of these factors remain unknown. Objectives To study the role of dietary and non-dietary factors as triggers of BD-related ROU. Methods A 23-item self-report questionnaire was given to consecutive in- and outpatients with BD who attended 7 French hospital departments for internal medicine over a 12-month period. Patients were enrolled if they consented to participate in the study and if they had a history of ROU that had not become definitively extinct to ensure patients9 ability to provide accurate information. The questionnaire consisted of 13 items collecting general information, e.g., demographic characteristics, dietary habits, age at onset and severity of ROU. Six additional open-ended, dichotomous (“Yes” or “No”) or scaled questions (“Yes, I am sure”, “Yes, that9s possible”, “No, that9s highly unlikely”, or “I don9t know”) collected information on potential triggering factors of ROU. In particular, the questionnaire evaluated the influences of 6 general triggering situations (i.e., fatigue/stress, dental care, tooth brushing, menstruation, infection, and food/drinks), 24 selected food and drink items, 8 physicochemical properties of food (i.e., salty, sweet, bitter, acid, astringent, hard, hot, cold), and the effect of fast eating. The results were described as percentages of positive responses; for scaled questions, the combined responses of “Yes, I am sure” or “Yes, that9s possible” were considered positive. Results Of the 100 questionnaires distributed, 87 were returned and 80 could be used for the analyses. Among those 80 patients (mean age: 41 years, men: 63%), 96% fulfilled the International criteria for BD, 75% had non-French origins and 81% consumed European-style food. The mean time since ROU onset was 19 years and the ROU was judged by 53% as “very discomforting” or “discomforting” over the previous 12 months. Among the 6 general situations suggested, 91% recognized at least 1 of them as “surely” or “possibly” related to their ROU: fatigue/stress (75%) and food/drinks (70%) were the most frequently cited triggering factors. Among the 24 food/drinks items suggested, the most frequently reported items were: walnuts (49%), pineapple (43%), peanuts (33%), Emmental cheese (30%), lemon (25%), almonds (24%), and other cheeses (21%). Acidity was the most frequently reported physicochemical food characteristic triggering ROU (58%). Conclusions Fatigue/stress and food are frequently recognized triggering factors of BD-related ROU. This implies the need to take into account such external factors in the management of ROU. The fact that most food items identified as common triggers of ROU are histamine-rich or histamine-releasing could suggest a role of histamine in the pathophysiology of BD-related ROU. Hormonal and traumatic components seemed to be of lesser importance in ROU. Disclosure of Interest None declared

Introduction L’aphtose buccale (AB) recurrente est une manifestation quasi-constante de la maladie de Behcet (MB). Il est largement admis que les recurrences de l’AB peuvent etre declenchees par des facteurs exterieurs, alimentaires ou autres, mais ces facteurs ne sont pas bien etudies dans la MB. L’objectif de ce travail etait d’etudier les facteurs alimentaires et non alimentaires declenchant les poussees d’AB. Patients et methodes Un auto-questionnaire etait distribue a des patients suivis pour MB provenant de 6 services hospitaliers de medecine interne. Le questionnaire comprenait 13 items portant sur les caracteristiques demographiques, le regime alimentaire, l’anciennete et l’importance de l’AB, et 6 questions ouvertes ou a choix multiples portant sur les facteurs declenchant les poussees d’AB. Le questionnaire evaluait l’influence de 6 situations generales (fatigue/stress, soins dentaires, brossage des dents, cycles menstruels, infections, aliments/boissons), de 24 aliments/boissons, de 8 caracteristiques alimentaires physico-chimiques (sale, sucre, amer, acide, astringent, dur, chaud, froid) et de la duree du repas. L’influence d’un facteur declenchant etait evaluee par une echelle ordinale a 4 niveaux de reponses allant de « Oui, j’en suis sur(e) » a « Non, ce n’est surement pas ca ». Les resultats sont donnes en pourcentages de reponses positives (« Oui, j’en suis sur(e) » ou « Oui, c’est possible ») rapportees a toutes les reponses. Resultats Parmi les 98 questionnaires distribues, 81 ont ete retournes et 74 ont ete inclus dans les analyses. Les 74 patients avaient un âge moyen de 42 ans. Il y avait 64 % d’hommes, 35 % d’origine maghrebine et 81 % consommait de la nourriture francaise ou europeenne. L’AB recurrente evoluait depuis 19 ans en moyenne et etait qualifiee par 55 % de « tres genante » ou « genante » dans l’annee precedente. Parmi les 6 situations generales proposees, 91 % en reconnaissaient au moins une comme « surement » ou « possiblement » en cause ; la fatigue/stress (74 %) et les aliments/boissons (68 %) etaient les facteurs declenchant les plus frequemment cites. Parmi les 24 aliments/boissons proposes, les noix (49 %), l’ananas (42 %), les cacahuetes (34 %), le gruyere (30 %), le citron (24 %), les amandes (22 %) et les fromages (hors gruyere) (20 %) etaient les plus frequemment incrimines. La nature acide de l’aliment etait incriminee comme declencheur de poussee d’AB par 57 % des patients. Conclusion La fatigue, le stress et l’alimentation sont les facteurs declenchant de poussees d’AB les plus frequemment cites. Ceci implique l’interet potentiel de prendre en compte les facteurs declenchant exterieurs dans la prise en charge d’une AB recurrente. La plupart des aliments identifies comme responsables de poussees d’AB sont riches en histamine ou histamino-liberateurs ce qui pourrait suggerer un role de l’histamine dans la physiopathologie de la poussee d’AB. La composante hormonale ou mecanique dans les poussees d’AB semble moins importante.

OBJECTIVES--To test the effectiveness of and tolerance to interferon-alpha 2b (INFa2b) in association with plasma exchanges for the treatment of polyarteritis nodosa (PAN) related to hepatitis B virus (HBV). METHODS--A prospective, non blinded, multicentre trial was carried out in which patients with multisystemic HBV-related PAN were included. Each patient received the association of INFa2b and plasma exchanges. The end point of the study was control of the disease (recovery or remission) or death. RESULTS--Six patients were included in the study. Each patient had histopathological or arteriographic evidence of vasculitis and was infected with actively replicating HBV. All patients were alive at the end of the study and no longer presented clinical or laboratory evidence of systemic vasculitis. HBeAg/anti-HBeAb seroconversion was observed in four patients (66.6%) and HBsAg/anti-HBsAb in 3/6 (50%). Two patients are still being treated with INFa2b because of chronic active hepatitis. CONCLUSIONS--It is considered that this new therapeutic approach to HBV-related PAN effectively cured systemic vasculitis and was associated with control of HBV infections. Antiviral therapy may have a role to play as the first line treatment regime of virus-induced vasculitis.

Churg-Strauss Syndrome is a disorder characterized by pulmonary and systemic necrotizing vasculitis, extravascular granulomas, and eosinophilia occurring almost exclusively in patients with asthma or a history of allergy. The clinical manifestations most commonly associated with asthma are mononeuritis multiplex, gastrointestinal, cutaneous, cardiac and renal involvement. ANCA are found in 2/3 of the patients with Churg-Strauss Syndrome and are usually p-ANCA. The treatment of Churg-Strauss Syndrome relies on corticosteroids and cyclophosphamide.

Congenital heart block (CHB) is a severe complication of maternal connective tissue disorders, especially systemic lupus erythematosus (SLE). CHB is often associated with SSA-Ro and/or SSB-La antibodies, present during pregnancy and which may provoke fetal conduction disturbances. When applied at the time of detection of CHB, steroids and plasma exchanges (PE) are ineffective. The discussion evokes the possible prevention of CHB by PE performed during the first 3 months of pregnancy in mothers with SLE or other connective tissue disorders, who have had a previous pregnancy complicated by CHB.

We report the assessment by MRI of a lumbosacral lymphomatous localization of adult T-cell leukemia/lymphoma (ATLL) with human T-cell lymphotropic virus-1 infection in an African patient. Brain MRI detected associated multifocal lesions of increased signal intensity in the subcortical white matter. These MRI abnormalities are compatible with reported necropsy findings in cases of ATLL with neurological complications.

Objective. To define the most effective treatment for polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS). Methods. We conducted a prospective, randomized, multicenter trial in which 78 patients were randomly assigned to receive either prednisone and plasma exchange (group A; n = 36) or prednisone alone (group B; n = 42) as first-line treatment of PAN and CSS. Patients with hepatitis B virus—related PAN were not included in this study. The end point of the study was control of the disease (recovery and remission) or death. Results. Clinical symptoms and laboratory findings did not differ statistically in the 2 groups at study entry. Initial control of the disease was similar in both groups. The assigned treatment was stopped in 16 patients because of lack of efficacy. Oral cyclophosphamide or dapsone therapy reversed the disease evolution in 7 of these 10 group A patients and in 4 of these 6 group B patients. At 7 years of followup, 56 patients had completely recovered (27 in group A, 29 in group B), 7 patients were in clinical remission, and 15 patients had died (19.2%; 6 group A patients and 9 group B patients). The prednisone—plasma exchange combination was no more beneficial than corticosteroids alone in preventing relapses over the long term. There was no significant difference in the 7-year cumulative survival rates of the two groups (83% and 79%, respectively). Conclusion. Based on our data, we conclude that combined treatment with prednisone and plasma exchange is not superior to treatment with prednisone alone and must not be systematically employed for initial treatment of PAN and CSS. In most cases, cyclophosphamide as second-line treatment is effective and well tolerated.

Churg-Strauss syndrome is a systemic and pulmonary vasculitis, defined by its association with severe asthma and with hypereosinophilia of the blood and tissues. The systemic vasculitis is a small-vessel vasculitis frequently associated with purpura, mononeuritis multiplex, and, more rarely, with rapidly progressive glomerulonephritis or diffuse alveolar hemorrhage. Its prevalence of 7 to 13 per million population makes it one of the rarest of the systemic vasculitides. Anti-MPO (antimyeloperoxydase) pANCA (ANCA with a perinuclear fluorescence pattern) are present in 35-40% of cases and appear to determine a subgroup of patients with a higher frequency of renal damage, alveolar hemorrhage, and central nervous system involvement. The diagnosis of Churg-Strauss syndrome is made on the basis of clinical features. In patients with late onset asthma the presence of constitutional symptoms, eosinophilia (1000/mm3), the appearance of a systemic illness characterized by monoreutis multiplex, purpura, cardiomyopathy or pulmonary infiltrates should lead to consider the diagnosis of Churg-Strauss syndrome. Cardiac involvement is an important cause of morbidity and the leading cause of specific mortality in Churg-Strauss syndrome. Treatment is based on corticosteroid therapy and immunosuppressive drugs (cyclophosphamide and azathioprine) and is determined according to validated prognostic criteria (Five Factors Score). Complete remission occurs in almost 90% of cases, and the 10-year survival rate has reached 79.4%. Relapses are frequent (25% of cases) and even after recovery from vasculitis, most patients (90%) still have asthma requiring corticosteroid treatment.

Churg-Strauss syndrome is a systemic and pulmonary vasculitis, defined by its association with severe asthma and with hypereosinophilia of the blood and tissues. The systemic vasculitis is a small-vessel vasculitis frequently associated with purpura, mononeuritis multiplex, and, more rarely, with rapidly progressive glomerulonephritis or diffuse alveolar hemorrhage. Its prevalence of 7 to 13 per million population makes it one of the rarest of the systemic vasculitides. Anti-MPO (antimyeloperoxidase) pANCA (ANCA with a perinuclear fluorescence pattern) is present in 35-40% of cases and appears to determine a subgroup of patients with a higher frequency of renal damage, alveolar hemorrhage, and central nervous system damage. Cardiac involvement is an important cause of morbidity and the leading cause of mortality in Churg-Strauss syndrome. Treatment is based on corticosteroid therapy and immunosuppressive drugs (cyclophosphamide and azathioprine) and is determined according to validated prognostic criteria (Five-Factor Score). Complete remission occurs in almost 90% of cases, and the 10-year survival rate has reached 79.4%. Relapses are frequent (25% of cases) and even after recovery from vasculitis, most patients (90%) still have asthma requiring corticosteroid treatment.

Churg-Strauss syndrome is a systemic necrotizing vasculitis involving small and medium-sized vessels. Classic features include asthma and hypereosinophilia. Antineutrophil cytoplasm antibodies (ANCA) are detected in about 40% of patients. Churg-Strauss syndrome has been reported in patients receiving leukotriene modifiers for asthma, in particular, leukotriene receptor antagonists (LTRA) (montelukast, zafirlukast or pranlukast). Clinical manifestations cases do not differ in these cases from those in Churg-Strauss syndrome without antileukotriene exposure. It is increasingly less likely that LTRA is the direct cause of this syndrome in those patients, although this hypothesis has not been completely ruled out. In many patients, LTRA treatment is prescribed because of worsening asthma, which is an early sign of Churg-Strauss syndrome. LTRA for asthma patients should be prescribed with great care, especially in cases of atypical or rapidly aggravated asthma. The onset of Churg-Strauss syndrome in patients treated with LTRA usually requires that they stop this treatment. Prescription of LTRA In patients with Churg-Strauss syndrome should be discussed with specialists.

Summary Gastrointestinal tuberculosis is a rare form of extrapulmonary tuberculosis and its diagnosis can be difficult. Aims To analyze the diagnostic and therapeutic characteristics of gastrointestinal tuberculosis. Methods Retrospective study from 17 cases collected in 4 hospitals in Seine Saint-Denis between 1987 and 2002. Results Seventeen cases and 19 localizations were collected: small intestine (N = 7), ileocecum (N = 6), colon (N = 4) and gastroduodenum (N = 2). Two patients had two localizations. Mean age was 43.9 years. Subjects from immigrant populations (76.5%) were preferentially affected. Twenty-three percent of patients (13 tested) were infected by human immunodeficiency virus. Weight-loss and general weakness (88%), abdominal pain (88%), fever (59%), nausea/vomiting (53%) were the predominant symptoms. The delay in diagnosis was 82 days (range: 7-180) and time before specific treatment 31.6 days (range: 7-90). Histological evidence of caseating granuloma was found in six patients. Mycobacterium tuberculosis was detected in six. Digestive imaging was abnormal in 15 patients. Mesenteric lymph nodes were the most common associated site of tuberculosis (N = 8, 47%). Mean duration of treatment was 8.2 months (range: 6-12). Thirteen patients were cured, three died and one was lost to follow up. Conclusion Gastrointestinal tuberculosis is not an uncommon diagnosis in the north-eastern Parisian area, especially among immigrant populations and immunodeficient patients. The most frequent localizations are the small intestine and ileocecum. Diagnosis can be made by pathology and/or bacteriology on endoscopic and/or surgical biopsy samples.

A RARE EVENTUALITY: Although parasite infections are frequent, observations of vasculitis related to parasitosis are, however, very rare. REGARDING THE MECHANISM: The simultaneous occurrence of a parasitosis and vasculitis may be the consequence of either the direct implication of a parasite observed in the histological lesions in the onset of alteration in the vascular wall, or of immunopathological phenomena occuring during the anti-parasite immune response, or a fortuitous association. THE HUMAN PARASITOSIS IMPLIED: In most cases, vasculitis associated with parasitosis is an isolated event with varied clinical aspects. Such cases have been reported in toxoplasmosis, trichinosis, strongyliasis, ascaridiosis, sarcocystosis, amibiasis, leishmaniosis and toxocarosis.

Limb restricted polyarteritis nodosa (PAN) and PAN-type diseases such as isolated vasculitis of skeletal muscle are localised vasculitides affecting the skin, muscles, or peripheral nerves, usually of the lower limbs. These diseases often present with non-specific clinical symptoms and normal laboratory values and electromyograms. The usefulness of magnetic resonance imaging (MRI) of skeletal muscle has been poorly investigated to date.To describe the MRI findings in the legs of three patients with limb restricted vasculitides (two PAN, one isolated vasculitis of the skeletal muscle) with histologically established muscle involvement.MRI was carried out on calf muscles and T(2) weighted images, unenhanced T(1) weighted images, and STIR sequences were obtained.Muscle damage resulted in oedema-like changes on MRI characterised by hyperintense signals in T(2) weighted and slow tau inversion recovery (STIR) sequences and normal unenhanced T(1) weighted sequences of one or several leg muscles.MRI should be considered a useful complementary examination that might facilitate the recognition of limb restricted vasculitides, and possibly indicate the site for muscle biopsy. It could also be useful in monitoring the course of the disease. Future studies should also evaluate MRI for systemic PAN or other systemic vasculitides with muscle involvement.