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Introduction Une prise en charge specialisee pour les patients avec MRC stades 4-5 avant suppleance renale et pour les IRA severes (IRA2-3) selon les recommandations HAS est un enjeu de sante Publique. Nous nous sommes interesses a la description de la frequence et du service d’hospitalisation des patients adultes avec un DFG inferieur a 30 ml/min/1,73m2 ou avec une IRA2-3 decouverte en cours d’hospitalisation dans 3 hopitaux d’un departement de 650000 habitants pour discuter l’interet d’un depistage cible. Description et methodes A partir du fichier de dosage de creatininemie realise dans les laboratoires de biologie de trois hopitaux d’un departement (1 CHU(centre A) et 2 CHG(centre B-C)) en 2017 et 2018, nous avons identifie pour chaque annee, chez les adultes, le nombre de patients et le service d’hospitalisation avec survenue d’un premier signal d’IRA2-3 ou DFG Resultats En 2017/2018, nous avons identifie 65 754/67 223patients hospitalises avec 225 194/233 992 dosages de creatininemie repartis pour le centre A : 44 116/45 343 patients avec 164 282/172 664 dosages, centre B : 13 780/14 262 patients avec 44 250/44 941 dosages et centre C : 7 858/7 618 patients avec 16 662/16 387 dosages. En 2017/2018, nous avons identifie respectivement dans les centres A,B,C : 1,8 %, 2,2 %, 2 %/1,7 %, 1,8 %, 2,2 % de patients avec IRA2-3 et 5,3 %, 5,3 %, 4,7 %/5,3 %, 5,3 %, 5 % de patients avec un DFG Conclusion Les limites de l’etude sont le delai de suivi et le dosage unique de creatininemie chez certains patients. Cette etude nous a permis d’identifier la frequence et les services a risques de signaux d’IRA et de MRC pour proposer un depistage et une intervention cibles.

Vascular endothelial growth factor (VEGF) is important for angiogenesis and inflammation, both of which are codependent and contribute to the pathophysiology of Behcet’s disease (BD). In this report, we sought to investigate whether the selected VEGF polymorphisms [−634 C/G, +936 C/T and an 18 bp insertion/deletion (I/D) at −2549 of the VEGF promoter region] are associated with susceptibility and severity of BD in the Tunisian population. One hundred and thirty-five Tunisian BD patients and 157 healthy controls were recruited. The VEGF gene was genotyped by polymerase chain reaction followed by digestion with restriction endonucleases. VEGF serum levels of BD patients and healthy controls were measured by enzyme-linked immunosorbent assay. We found no association between the VEGF polymorphisms and the susceptibility to BD. However, when data were analysed according to the presence of each symptom, we found a positive association between VEGF 18 bp I/D polymorphism and ocular involvement as well as BD severity. Indeed, among the BD patients, the frequency of the 18 bp I/I genotype was less in patients with ocular inflammation (6.1% vs 24.6%, P = 0.007) and in patients with severe BD (9.2% vs 21.4%, P = 0.0014). Moreover, the mean serum VEGF level was considerably higher in BD patients (P

Improved understanding of the pathogenic mechanisms of Behcet's disease (BD), and especially of the molecular basis involved in its pathogenesis, has sparked a new generation of potential BD treatments with improved side effect profiles and/or more specific targeting of the immune system. These therapies include new immunosuppressants, biologic medications, tolerizing agents, and immunoablation techniques, a number of which are currently in use in clinical practice (interferons). While some of these new therapies target specific inflammatory mechanisms in SLE (tumor necrosis factor-α inhibitors), others work by non-specific inhibition of the immune system (immunoablation). Each of these approaches will be discussed in this review.

Resume Introduction Les maladies de Behcet (MB) et de Takayasu (MT) font partie des vascularites des gros vaisseaux. Etat des connaissances La prevalence de la MB est superieure a celle de la MT. Leur pathogenie implique une auto-immunite cellulaire activee par la proteine HSP60. Les anevrysmes pulmonaires dominent le pronostic de la MB par le risque majeur d’hemoptysies cataclysmiques. Les thromboses in situ provoquent des infarctus. Les thromboses de la veine cave superieure sont d’evolution lente. L’inflammation vasculaire diffuse au mediastin, a la plevre et au parenchyme. Le diagnostic des lesions pulmonaires est au mieux realise par la tomodensitometrie helicoidale. La MT touche plus frequemment l’aorte que les arteres pulmonaires. Les stenoses arterielles se compliquent d’hypertension arterielle pulmonaire et d’infarctus. L’association de signes generaux evoque le diagnostic. L’imagerie par tomodensitometrie ou IRM, est essentielle en objectivant l’epaississement parietal vasculaire. Le traitement est base sur l’association de corticoides et d’immunosuppresseurs. Les anevrysmes, les stenoses et les thromboses caves beneficient de traitements endo-vasculaires. Perspectives La persistance de formes refractaires requiert de nouvelles voies therapeutiques : inhibiteurs du TNF, interferon alpha, vasodilatateurs. Conclusion Des protocoles prospectifs sont indispensables pour evaluer l’interet du depistage et du traitement des lesions pulmonaires latentes.

Introduction L’asthme est une maladie dont le mecanisme etiopathogenique reste encore peu connu. L’IL-37, membre de la famille de l’IL-1 (cytokine majeure de l’immunite innee), est une cytokine anti-inflammatoire dont les effets ont ete demontres dans plusieurs maladies auto-immunes. L’objectif de notre travail etait d’etudier le taux de l’IL-37 dans le sang et l’expectoration induite (EI) dans l’asthme de l’enfant et son effet sur les cytokines pro-inflammatoires (IL-17, IL-6, IL-1β et TNF-α). Methodes Quarante enfants avec un asthme bien controle (20 asthmes legers et 20 moderes) ont ete inclus et compares a 22 controles sains. Le dosage de l’IL-37 a ete fait par Elisa dans le sang et l’EI. L’expression de l’ARN messager (ARNm) de l’IL-37 a ete etudiee dans l’EI par PCR en temps reel. La production de IL-17, IL-6, IL-1β et TNF-α chez 10 asthmes moderes et 10 temoins sains a ete etudiee dans l’EI avant et apres adjonction d’IL-37 recombinante (rIL-37). Resultats Compares aux sujets sains, le taux de l’IL-37 etait plus bas chez les sujets asthmatiques dans le sang et l’EI independamment de la severite (p = 0,0001). De meme pour l’expression de l’ARNm de l’IL-37 dans l’EI qui etait basse chez les enfants asthmatiques (p = 0,0001), mais cette expression etait plus basse dans l’asthme modere par rapport a l’asthme leger (p = 0,0001). Les cellules de l’EI de patients asthmatiques produisaient plus d’IL-6, TNF-α et IL-1β compares aux sujets sains (p Conclusion Nos resultats montrent qu’il y a un defaut de suppression des cytokines pro-inflammatoires dans l’asthme de l’enfant caracterise par des taux bas d’IL-37. La severite de l’asthme semble etroitement liee au degre du deficit de l’IL-37. Restaurer l’expression de l’IL-37 pourrait etre une option therapeutique interessante dans l’asthme severe.

Introduction L’asthme severe est une entite qui represente 10 % des tous les asthmes mais qui est une source importante de depenses de soins de sante. Le clustering dans l’asthme severe ou approche phenotypique permet de classer les patients en groupes homogenes susceptibles d’evoluer de la meme facon et d’avoir des traitements personnalises. Methodes Il s’agit d’une etude prospective descriptive de 51 patientes asthmatiques severes. Nous avons utilise l’analyse de cluster selon la methode de Ward's pour identifier des groupes cliniques en se basant sur 9 variables cliniques et sur des donnees de l’expectoration induite. Resultats L’analyse de cluster a permis d’identifier 3 groupes. Le cluster 1 (33,3 %) comportait les asthmes severes a debut peri-menopausique peu symptomatiques eosinophiliques. C’est un asthme a debut tardif dans 100 % des cas, dont 70,6 % debutant en peri-menopause. Ces patientes etaient les moins atopiques (41,2 %) et presentaient le moins d’exacerbations frequentes (17,6 %) que les 2 autres groupes. Le cluster 2 (49 %) comportait les asthmes severes tardifs avec obstruction fixee (debut tardif dans 76 % des cas) neutrophiliques. Ces patientes etaient les plus severement obstructives (VEMS moyen 49,68 %, avec 100 % d’obstruction fixee) avec le taux de cortico-dependance (chez le tiers des patientes) le plus important parmi les trois groupes. Le cluster 3 (17,64 %) comportait les asthmes severes atopiques a debut precoce (debut precoce dans 66,7 %) exacerbateurs neutrophiliques. Ces patientes avaient significativement la meilleure fonction respiratoire (VEMS moyen a 87,33 %, et une obstruction fixee dans 22 % des cas). Ces patientes presentaient une maladie active (symptomes en intercritique 66,7 %, exacerbations frequentes et AAG chacun dans 55,6 %). Conclusion L’analyse de cluster offre une approche multidimentionnelle des phenotypes clinique et inflammatoires de l’asthme severe permettant ainsi une prise personnalisee de chaque phenotype.

Introduction Vitamin D plays key roles in innate and adaptive immunity through the stimulation of Toll-like receptors, increasing proinflammatory cytokine production, and possibly skewing T helper (Th) responses. Data from human vitamin D supplementation studies have shown beneficial effects of vitamin D on immune function, in particular in the context of autoimmunity. Low levels of this hormone were observed in several autoimmune diseases including Behcet’s disease. The data relating vitamin D to autoimmune and inflammatory diseases are equivocal, with studies linking low vitamin D levels to dysregulation of Th1/Th2 and Th17/ Treg ratios. We summarised the effects of vitamin D on the immune system in Behcet’s disease. Conclusion Vitamin D research has confirmed important interactions between vitamin D and cells from the innate as well as from the adaptive immune system. All the data reported in this review show that vitamin D in Behcet’s disease inhibits Th17 cytokine production, enhances Treg cells, suppresses Th1 and promotes Th2 cytokine production and thus skews T cells towards Th2 polarisation.

The manifestations of BD are considered to have developed as a result of immunological dysfunction, which is suggested to be induced by microbial pathogens. The Toll-like receptor (TLR) genes were known to be associated with a variety of infectious diseases due to their central role in both innate and adaptive immunity. In this report, we investigated the possible association between BD patients and genetic variations within the TLR 2, 4 and 9 genes in a Tunisian population.135 Tunisian BD patients and 159 healthy blood donors from the same geographical area were genotyped by polymerase chain reaction for the TLR polymorphisms.Among the TLR polymorphisms, only the distribution of TLR9 1486 T/C genotype (p=0.07; chi2=3.30; OR=1.54; 95% CI=0.94-2.51) and allele (p=0.08; chi2=2.91; OR=1.34; 95% CI=0.94-1.92) frequencies was different between BD patients and healthy controls, but did not reach statistical significance. For the TLR9 1237 T/C, the distribution of genotypes and alleles were not significantly different comparing total patients with controls. There were no associations between the studied polymorphisms and the main clinical manifestations of BD. The G, T and A allele of the TLR4 1896 A/G, TLR4 11196 C/T and TLR2 12408 G/A polymorphisms were uncommon and absent in the Tunisian population.Our results showed that SNPs in the TLR2, 4 and 9 genes were not significantly associated with susceptibility to BD.

Serum levels of the B-cell activating factor in the tumor necrosis factor family (BAFF), a potent contributor to B-cell survival, are elevated in patients with systemic autoimmune diseases. The objective of this study was to determine serum BAFF levels and to link the results to the clinical features in patients with skin manifestations.Serum BAFF levels were examined by an enzyme-linked immunosorbent assay (ELISA) in 42 patients with BD (16 with active disease), 20 healthy controls, and in 20 patients with systemic lupus erythematosus (SLE) and 15 patients with multiple sclerosis (MS), who served as the disease control groups. Expression of BAFF messenger RNA (mRNA) in the skin was quantified by a real-time reverse transcription-polymerase chain reaction; the expression of BAFF receptor (BAFF-R) on CD19+ B cells was assessed by flow cytometry; and ELISA was used to evaluate the production of IgG, interleukin-6 (IL-6) and IL-10 by isolated B cells.Serum BAFF levels were elevated in patients with active BD compared to the healthy controls, and correlated positively with the extent of skin lesions. Disease remission was accompanied by decreased BAFF levels. SLE patients had the highest serum BAFF levels. Skin biopsies showed BAFF mRNA expression to be up-regulated in active BD patients. BAFF-R expression on B cells was increased in BD patients with vasculitis. Furthermore, in BD patients the ability to produce IgG and IL-6 (but not IL-10) was enhanced in BAFF-stimulated B lymphocytes.These results suggest that BAFF and its signalling in B cells contribute to B cell abnormalities and the development of skin disease in patients with BD.

Several lines of evidence point to a relevant role of IL-18 in the process of asthma. Some studies suggest that the polymorphism in the gene of IL-18 can be involved in many inflammatory and atopic diseases such as asthma. The aim of our study is to estimate the frequency of the IL-18-607 C/A (rs 1946518) promoter polymorphism in Tunisian children with asthma. We investigated whether the presence of this polymorphism -607 C/A was associated with asthma or atopy and whether this polymorphism influenced the severity of asthma in affected children. We examined also the relationship between the IL-18 gene polymorphism and the serum total IgE level. The IL-18/-607 C/A polymorphism was analysed by polymerase chain reaction and restriction fragment-length polymorphism (PCR-RFLP) analysis. A total of 105 asthma patients and 112 controls as part of the whole children population were studied in a case-control study. Among the 105 children with asthma, 40 were also studied for linkage analyses with their respective parents. We noted that the A allele was associated with statistically significant increases in the risk of asthma in the case-control study (odd ratio (OR) = 1.55, 95% confidence interval (CI) 1.03-2.33. Moreover, the A allele was also associated with atopic asthma (P = 0.008), but not with asthma severity. The transmission disequilibrium test (TDT) analysis in this family study did not suggest a preferential transmission of the IL-18/ -607 C/A polymorphism to affected children. There is no correlation between the IgE level and the IL-18 -607 C/A promoter polymorphism. Our data indicate that IL-18 -607 C/A promoter polymorphism is associated with susceptibility to developing asthma in Tunisian population.

CD4+CD25(bright) T cells and NKT cells are potent immunoregulatory cells and are therefore attractive targets for immunotherapy. The possibility of modulating CD4+CD25(bright) T cell function using NKT cells, and vice versa, reveals a new route for harnessing suppressor T cells with therapeutic properties. However, the questions of where, when and how NKT cells interact with CD4+CD25(bright) T cells are still unknown. Disease studies have investigated the regulatory properties of these T-cell subsets independently of each other. Recent reports have provided evidence for cross-talk between CD4+CD25(bright) T cells and NKT cells and consequently, the immunoregulatory networks are seen in a new perspective. Activated NKT cells seem to modulate quantitatively and qualitatively CD4+CD25(bright) T cell function through IL-2-dependent mechanisms, whereas CD4+CD25(bright) T cells can suppress the proliferation, cytokine release and cytotoxic activity of NKT cells by cell-contact-dependent mechanisms. Importantly, CD4+CD25(bright) T cells and NKT cells share crucial signalling pathways that could be responsible for immune dysregulations in Behçet's disease (BD). The advances in our understanding of the interactions between distinct subsets of regulatory T cells might unveil new waves for modulating these cells with the inflammatory process in BD.

To analyze demographic, clinical and genetic features of Behçet's disease (BD) in Tunisia and to compare them with other ethnic and geographic groups.Two hundred and sixty patients with BD (International Study Group criteria) received in the Department of Internal Medicine of the University Hospital La Rabta in Tunisia, from 1987 to 2006, were retrospectively studied. Demographic, clinical, and genetic data were recorded and analyzed using SPSS 11.0.The cohort consisted of 188 males and 72 females (M/F = 2.61). The mean age at the onset of the disease was 29 years. Oral and genital ulcers (GU) were seen in 100% and 83% respectively. The most other common clinical features of BD were ocular involvement (51%), arthritis (38.8%), venous thrombosis (33%) and neuropsychiatric symptoms (24.2%). Only 1.5% had gastrointestinal lesions. HLA-B51 frequency was significantly higher in patients with BD (54% vs. 25.5% in healthy controls, p0.05). DVT and pseudofolliculitis were significantly more frequent in men whereas arthritis and erythema nodosum were significantly more frequent in women. DVT was also significantly more frequent in patients with GU and those with neurological involvement. GU, positive pathergy test and DVT were significantly less frequent in patients with ocular involvement. Neurological involvement consisted of 47 cases with CNS parenchymal lesions and 22 with cerebral vascular lesions (13 had both lesions); HLA B51 was significantly less frequent in patients with neurological involvement.Our series was characterized by particular aspects such as high frequency of DVT and neuropsychiatric involvement, and rare occurrence of gastrointestinal lesions. The results confirm the ethnic and geographic variation of BD expression.

Interleukin-15 (IL-15) is a novel proinflammatory cytokine, involved in the pathogenesis of inflammatory/autoimmune disease. The objective of our study was to measure serum and cerebrospinal fluid (CSF) IL-15 levels in patients with Behçet's disease (BD). CSF/serum IL-15 ratio was introduced to assess the origin of elevated IL-15 levels. We measured serum and CSF-IL-15 levels in 40 patients with BD (20 patients in active stage). Inflammatory and non-inflammatory neurological disease patients acted as controls. Active BD patients have significantly higher serum IL-15 levels (median 10.4 pg/ml; range 5.3-17.4) compared with BD in remission (6.05 pg/ml; 4-10.4) and healthy controls (4.65 pg/ml; 3.9-6.2). Similar serum IL-15 levels were found in active neuro-BD and inflammatory neurological disease (9.5 pg/ml; 5-13). Elevated levels of IL-15 were observed in CSF samples from neuro-BD patients (11 pg/ml; 8.5-15) and inflammatory neurological disease patients (10 pg/ml; 6.5-14) compared with patients with non-inflammatory neurological disease (4 pg/ml; 4-5.5; P0.001). Vascular cerebral BD lesions were associated with high CSF/serum IL-15 ratio. Our findings suggest that IL-15 is involved in BD inflammatory process, particularly in vasculitis foci, as an elevated CSF/serum IL-15 ratio characterizes vascular cerebral lesions.

To investigate whether the CD4+CD25+ regulatory T cell (Treg) population, which plays important role in autoimmune diseases is related to the pathophysiology of Behçet's disease (BD).Forty-two patients with BD (20 patients in active disease) fulfilling the criteria of the International Study Group of BD. Twenty age-matched healthy controls were studied. We analyzed CD4+CD25+/high T cells and the mRNA expression of Foxp3, cytotoxic T lymphocyte-associated antigen 4 (CTLA-4), and transforming growth factor beta (TGF-beta) in BD. We have studied the ability of CD4+CD25+ (Treg) to regulate proliferation of CD4+CD25- T cells during active BD stage.Active BD patients had significantly higher CD4+CD25+/high T cells, as compared with BD in the remission stage, and healthy controls. There was no significant differences in the CD4+ CD25+/high T cells expression between healthy controls and remission BD. In active BD, mRNA for Forkhead box p3 (Foxp3) and cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) were highly expressed when compared to remission BD and healthy controls. There was no differences in the mRNA expression for TGF-beta in active BD, remission BD and healthy controls. Functionally, CD4+CD25+/high T cells in active BD were impaired in their proliferative responses and could suppress the proliferation of their CD4+CD25- counterparts.These data demonstrate that CD4+CD25+ Treg cells, with the potential to regulate suppression of effector T cells, were increased in the peripheral circulation of active BD patients. The role of CD4+CD25+/high T cells in the regulatory process of the inflammation in active BD, could be taken in account.

IL-18 expression and functional activity has been identified in several autoimmune and infectious diseases. To clarify the potential role of IL-18 during pulmonary Behçet's disease (BD), we have explored the capacity of IL-18 to induce the expression of IFN-gamma.We studied bronchoalveolar lavage (BAL) from 12 patients with BD, 10 patients with silicosis as the control disease and 10 BAL from healthy subjects. BAL fluid, and BAL fluid cell cultures were investigated for IL-18 estimation by ELISA. Analysis of IL-18 and IFN-gamma gene expression was carried out before and after LPS stimulation.BD patients had significantly elevated levels of IL-18 in BAL fluid compared with control disease and healthy subjects. Induction of IFN-gamma and IL-18 were observed from BD-BAL fluid cells both spontaneously and after LPS stimulation, at higher levels compared to silicosis patients and healthy subjects (HC). Spontaneously only BD BAL cells expressed IL-18 mRNA and IFN-gamma mRNA. Forty-eight hours after LPS stimulation IL-18 mRNA and IFN-gamma mRNA were observed in BD, silicosis and HC cells. Recombinant IL-18 induced IFN-gamma production in BD- BAL fluid cells.Administration of IL-18 induced greater IFN-gamma production in BD-BAL fluid cells, than in normal BAL fluid cells. Our data indicate that IL-18 up-regulation is a feature of BD and suggest that IL-18 and IFN-gamma may contribute to the local inflammatory response in BD.

Our aim was to investigate the TCR gamma delta+ subset in Behçet's disease (BD) inflammatory sites, which better reflects changes associated with the pathologic process than peripheral blood.Forty-five patients with active BD, 10 patients with recurrent aphthous ulcers, 12 patients with rheumatoid arthritis, 5 patients with noninflammatory neurologic diseases and 15 healthy individuals were studied. Three monoclonal antibodies TCR delta 1, BB3, and A13 were used to assess the percentage of TCR gamma delta+ in peripheral blood mononuclear cells (PBMC), in bronchoalveolar lavage and cerebrospinal fluid (CSF). CD11a/CD18 was used to study adhesion molecules. TCR gamma delta+ cells isolated by immunomagnetic separation were tested for cytolytic activity against K562 target cells after interleukin 2 stimulation.The PBMC TCR gamma delta BB3+ subset was significantly increased in BD. In BD inflammatory sites, TCR gamma delta+ cells were also present, composed mainly of A13+ cells from these sites also expressed CD11a marker. TCR gamma delta+ cells from inflammatory sites displayed a higher cytotoxic activity than controls, mediated by the A13+ subset.The accumulation of cytotoxic TCR gamma delta+ cells at the sites of inflammation suggests their involvement in the local injury process.

We assessed T cell subpopulations using 2-color flow cytometry with phycoerythrin conjugated anti-CD45RA and anti-CD29 and fluorescein conjugated anti-CD4 and anti-CD8 monoclonal antibodies, on peripheral blood lymphocytes from 19 patients with Behçet's disease (BD) and 18 healthy control subjects. The percentage of CD4+ cells was significantly lower in patients with BD (34 +/- 2%) than in control subjects (46 +/- 3%) (p less than 0.001). Among CD4+ cells, the percentage of suppressor-inducer (CD4+CD45RA+) cells was significantly lower in patients with BD (31 +/- 4%) than in control subjects (45 +/- 2%) (p less than 0.01), while the percentages of helper-inducer (CD4+CD29+) cells were similar in patients and controls. The percentage of CD8+ cells was significantly higher in patients with BD (36 +/- 2%) than in control subjects (26 +/- 2%) (p less than 0.001) involving both CD45RA+ and CD29+ subpopulations. Within CD4+ cells, the percentage of suppressor-inducer (CD4+CD45RA+) cells was significantly decreased in patients with active BD (26 +/- 3%) compared with control subjects (45 +/- 2%) (p less than 0.001), whereas in patients with inactive BD the difference was statistically insignificant. Our results suggest that the defective suppressive function in patients with active BD may be related to the decreased suppressor-inducer subpopulation (CD4+CD45RA+).

B and T cell function were studied in 10 patients with active Behçet's disease (BD) and in 10 normal subjects. Peripheral B lymphocytes infected with Epstein-Barr virus (EBV) were cultured for 20 days in the presence or absence of autologous T cells. Immunoglobulin M and G secretions into the supernatants were assessed with an enzyme-linked immunosorbent assay. The extent of suppression of EBV-induced B cell activation by autologous T cells was significantly decreased in active BD patients as compared to normal subjects at a T:B ratio of 1:1, whereas the suppression ratio was in the normal range at a T:B ratio of 4:1. The IgM and IgG secretions in purified B cell cultures were significantly higher in active BD patients as compared to control subjects. Thus, an increased B cell function associated with a defective EBV-specific T cell suppressive function could explain at least in part the immunological disorders in BD patients.

SUMMARY In view of the pulmonary manifestations in Behcet's disease (BD), we investigated the production of endothelin-1 (ET-1)'by the respiratory tract in active BD. A group of 10 patients with active BD with pulmonary manifestations and 10 control subjects were studied. Immunoreactive ET-1 was measured in BAL and in serum by RIA. All the BD patients exhibited higher BAL ET-1 levels than controls. ET-1 expression may contribute to the functional and morphological abnormalities of the vasculature associated with BD. BEHCET'S disease (BD) is recognized as a systemic disease with vascular complications: aneurysm forma- tion and venous thrombosis (1). Involvement of the pulmonary vascular tree in BD has been reported by Erkan and Cavdar (2), who evaluated its frequency as 10-15%. In view of the pulmonary manifestations in BD, we wondered whether the production of endothelin-1 (ET-1) by the respiratory tract may contribute to the inflammation in vivo and to disease activity. A group of 10 BD subjects in active stage were studied who met the diagnostic criteria proposed by the Internationa) Study Group (ISG) for BD (3). They were suspected to have pulmonary manifestations and underwent a bronchofibrosc opy with bronchoalveolar lavage (BAL). Macroscopic lung involvement was confirmed in seven cases (chronic cough associated with interstitial shadows on the chest roentgenogram or pulmonary aneurysms). None of the patients had received immunosuppressive agents for at least 3 months prior to the study. We studied 10 control subjects (males, with an average age of 42.3 ± 5.2 yr) with no evidence of interstitial lung disease. They were undergoing routine bronchoscopy for suspected bronchial carcinoma. In all of them, the lung lavaged was roentgenographically normal and BAL cytology showed a normal differential count. Informed consent was obtained from all the patients. BAL was performed, as we have recently reported (4). Serum (0.5 ml) and BAL fluid samples (10 ml) were injected into prepared SepPak* Cn cartridges (Waters Milli- pore, Milford, MA, USA), washed with deionized water, and eluted with 2 ml of 60% acetonitrile and 0.6% trifluoroacetic acid. Cn cartridges were prepared with 10 ml serial washes of methanol, deionized water, acetone (5). Eluted samples were concentrated by evaporation and reconstituted in 0.25 ml assay buffer.

Forty-two Tunisian patients suffering from Behçet's disease (23 with uveitis) were typed for HLA-DR and DQ antigens. There was a significant excess of HLA-DQw3 (p less than 0.01) but also an important deficiency of HLA-DRw6 and DQw1 (p less than 0.01). A substantial increase of HLA-DR2 (p less than 0.01) for those with uveitis, and of HLA-DR4, DR7, for the others has been recorded (p less than 0.01).