Collagen-vascular disorders (CVD) are commonly associated with chronic interstitial lung disease. Clinicopathologic observations suggest that inflammatory process of the lower respiratory tract may appear prior to fibrosis. Subclinical pulmonary involvement, as assessed by bronchoalveolar lavage (BAL) was evaluated in 61 patients with various CVD but free of clinical pulmonary symptoms and with normal chest roentgenograms. Eight of 61 had abnormal pulmonary function tests (PFT) at entry to the study. Total BAL cell yield from nonsmokers was greater in patients with abnormal than in those with normal PFT (p less than 0.05). Abnormal differential count of BAL cells was noted in 29 of 61 patients (48%). Lymphocyte alveolitis (lymphocytes greater than or equal to 18%) was a characteristic finding in patients with primary Sjögren's syndrome (11 of 25) or Sjögren's syndrome associated with another CVD (4 of 8). Neutrophil alveolitis (neutrophils greater than 4%) with or without increased percentage of lymphocytes occurred in patients with CVD classically associated with pulmonary fibrosis: progressive systemic sclerosis (6 of 10), rheumatoid arthritis (1 of 4), dermatopolymyositis (2 of 3), and mixed connective tissue disease (3 of 8). An increased percentage of eosinophils was detected in 1 patient with progressive systemic sclerosis. Bronchoalveolar lavage abnormalities were more frequently detected in patients with active and severe extrapulmonary disease. On follow-up PFT 12 months later, 11 patients with normal BAL and 10 patients with lymphocyte alveolitis had not deteriorated. In marked contrast, the presence of neutrophils in BAL was associated with a progressive deterioration of PFT in 6 of 7 untreated patients, whereas 4 corticosteroid-treated patients with neutrophil alveolitis had not deteriorated.(ABSTRACT TRUNCATED AT 250 WORDS)

Objective. To assess sialyltransferase expression in peripheral blood mononuclear cells (PBMC) of patients with systemic sclerosis (SSc) and to correlate this expression with the clinical features of the disease.Methods. Using a multiplex reverse transcription polymerase chain reaction (RT-PCR) method, we simultaneously measured the expression of 5 sialyltransferases (ST3Gal IV, ST3GaI III, ST3Gal I, ST3Gal II, and ST6Gal I) and of one reference housekeeping gene, Tata box binding protein (TBP), in PBMC of 28 patients with SSc and 18 healthy controls. Expression of each sialyltransferase was defined by the ratio sialyltransferase amplification product intensity/TBP amplification product intensity, and was evaluated according to the skin sclerosis extension and the presence of lung fibrosis and/or of pulmonary hypertension.Results. ST3Gal I and ST6Gal I expressions were lower in patients with SSc than in healthy controls (median 0.48 vs 1.30, p < 0.0001, and 0.71 vs 1.96, p < 0.01, respectively). No difference was observed for ST3Gal III and ST3Gal IV expression. ST3Gal IV/ST6Gal I ratio was higher in SSc patients than in controls (0.37 vs 0.28, p = 0.03). ST3Gal II was either weakly or more often not expressed in both groups. Ten patients had isolated pulmonary hypertension. They had higher ST3GaI IV expression than patients without pulmonary hypertension (0.73 vs 0.29, p = 0.04) and a higher ST3Gal IV/ST6Gal I ratio than patients without pulmonary hypertension (1.03 vs 0.27, p = 0.03) and controls (1.03 vs 0.28, p = 0.02). No difference was found in the sialyltransferase expression and soluble E-selectin concentration according to the cutaneous sclerosis extension or the presence of lung fibrosis.Conclusion. Sialyltransferase expression is modified in PBMC of patients with SSc compared to healthy subjects. Low ST6Gal I expression associated with normal ST3Gal IV expression, resulting in a higher ST3Gal IV/ST6Gal I ratio, suggests an enhanced expression of Sialyl-Lewis(X) at the surface of PBMC in SSc, and therefore an active interaction between activated endothelial cells and PBMC through the binding between E-selectin and Sialyl-Lewis(X). This suggested that higher expression of Sialyl-Lewis(X) concerned patients with isolated pulmonary hypertension more specifically. Binding between PBMC surface Sialyl-Lewis(X) and activated endothelial cell E-selectin might therefore play a role in the pathogenesis of SSc-related isolated pulmonary hypertension.

This study inform the medical community about the type of patients treated by the specialists in Internal Medicine, precise the exact nature of their professional exercise and their real need in medical post-university formation.

PURPOSE:Rhabdomyolysis and myositis are rare, dose-related complications of statins and fenofibrates. The outcome is favorable as a rule with rapid regression after stopping the responsible drug. Recently, various auto-immune disease with evidence of hypersensitivity to HMG-CoA reductase inhibitors or fibrates drugs have been reported. Less than ten cases of dermatomyositis and polymyositis due to cholesterol-lowering drugs (CLD) have been previously reported. Five more cases polymyositis associated with CLD are reported.METHODS:Symptoms were compatible with diagnosis of polymyositis according to Bohan and Peter and with previous reported criteria for drug-induced myopathy in all cases. None of these patients had previous other connective tissue disorders.RESULTS:Five patients (median age 68 [54-78], female N =4) with CLD treatment (statin N =4, fenofibrates N =1) have developed iatrogenic polymyositis. All of them presented both proximal muscular weakness and increased muscle enzyme levels. One patient had iatrogenic antisynthetase syndrome characterized by mechanic's hand, Raynaud's phenomenon and anti JO1 antibodies. One other had sclerodermic hand oedema. Antinuclear antibodies were positive in 4 cases and muscle biopsy revealed polymyositis infiltrate in 4 cases. CLD treatment was discontinued with partial clinical improvement in 3 cases. Clinical remission was obtained with corticosteroid (N =5) in association with immunosuppresive agents in 3 cases.CONCLUSION:Muscular symptoms in patient with CLD treatment could be the first symptom of a polymyositis revealed or increased by this treatment and must encourage physician with antinuclear antibodies screening especially in case of proximal muscular weakness and increased muscle enzyme levels.

Propos. – Cette enquête d'épidémiologie descriptive réalisée en mars 2002 avait un double but : 1) contribuer à l'élaboration d'un Livre blanc sur la spécialité ; 2) analyser les besoins de formation des internistes.

The aim of this study is to determine prevalence, clinical significance of antiphospholipidantibodies (aPL) including anticardiolipin antibodies (aCL), anti-b2GP1 and lupus anticoagulant (LA) in a cohort of 74 patients with primary Sjögren’s syndrome (pSS) according to revised European criteria. aPL were found in 25 (34%) patients; IgG in 23 (12 had low titres, six moderate titres and five high titres) and IgM in five (three and two had respectively moderate and high titres). Eight (11%) patients were found to have LA; anti-b2GP1 antibodies were detected only in three (4%) patients. Only two patients with LA, aPL and b2GP1 had recurrent venous thrombosis. One patient with moderate titres of aPL exhibited recurrent spontaneous foetal losses. Peripheral neuropathies without cryoglobulinemia were more frequent in the aPL group. Other systemic involvements of pSS were the same in both groups with or without aPL. Patients with aPL have more concurrentimmunological diseases such as thyroiditis and primary biliary cirrhosis and a higher prevalence of hypergammaglobulinemia (P < 0.05). Even if aPL prevalence reached 30% in pSS, titres were usually low, with a close correlation with hypergammaglobulinemia but not with antiphospholipid syndrome, which is related to positivity of both LA and aPL.

Leptospirosis is a re-emerging anthropo-zoonotic infection of worldwide significance caused by the pathogenic spirochete (Leptospira interrogans) of the genus Leptospira, predominant in tropical/temperate regions and endemic to areas receiving heavy rainfall and flooding. Clinical presentation is similar to that of other febrile illnesses exhibiting mild symptoms which are often self-limiting. Hence, Leptospirosis is often mis-diagnosed and remains untreated progressing to Weil's Disease which is fatal. As only 30% of cases are diagnosed in endemic countries, Leptospirosis remains as a neglected zoonotic disease of tropical regions, due to poor diagnostic facilities and mild, asymptomatic disease manifestations which are often neglected. As this zoonosis is reported to cause periodical outbreaks, it is a major public health concern. Although diagnostic facilities are available, they are not accessible in technology-limited settings and are limited to certain hospitals and reference laboratories. This review is about the various methods used for the detection of Leptospirosis and their significance. It highlights the need for an appropriate diagnostic test for the rapid detection of leptospirosis in order to initiate immediate antibiotic therapy.

PURPOSE Antiphospholipid antibodies (aPL), anticardiolipin antibodies (aCL) or lupus anticoagulant (LA), are indispensable for the diagnosis of antiphospholipid syndrome (APS). However, antiphospholipid assays can generate false positive results. MATERIALS We have studied the influence of hypergammaglobulinemia (HG) on aPL antibodies titers in 232 patients twice as positive for aPL antibodies. RESULTS Out of 232 patients, 93 have an APS (76 primary APS, 17 secondary APS). Thrombosis occurred 138 times in APS patients. Of 139 patients without APS, 95 have an auto-immune disease, 28 have an isolated prolonged KCT and 16 an evolutive neoplasia. LA seems to be the best marker of APS. On the other hand aCL IgG and M, anti-beta2-GP1 IgM titers are significantly higher in patients without APS but with HG. CONCLUSION Those results suggest that biological APS diagnosis should be carefully performed in patients with HG. In this case, other additional risk factors must be considered for the etiological diagnosis of thrombosis.

SUMMARYThe aim of this study was to evaluate the presence and the role of the serum soluble costimulatory molecule CD28 in patients with systemic lupus erythematosus (SLE), primary Sjögren's syndrome (SS), and systemic sclerosis (SSc). Soluble CD28 concentration was determined by ELISA in 45 patients with SLE, 45 patients with primary SS, 30 patients with SSc, and 45 healthy subjects. We also evaluated CD28 mRNA expression by semiquantitative RT-PCR, and the biological activity of recombinant soluble CD28 on T lymphocyte activity. Concentrations of soluble CD28 were significantly higher in patients with SLE, primary SS and SSc than in healthy subjects. Soluble CD28 concentrations were higher in patients with systemic primary SS than in patients with glandular-limited primary SS. PCR analysis suggested that soluble CD28 resulted from the shedding of the membrane form. In vitro assay revealed that soluble CD28 inhibits the anti-CD3 mAb induced T cell proliferation. Soluble CD28, which modulates the proliferation of T lymphocytes, could be associated with disease severity in patients with autoimmune disease, especially primary SS. These results suggest that soluble CD28 could play an important role in the regulation of autoimmune diseases.

Le sommeil de l’adolescent est caractérisé par un retard de phase physiologique fréquemment exacerbé par l’utilisation intensive des technologies d’information et de communication. La restriction de sommeil ainsi induite en période scolaire a des conséquences sur la santé physique et psychologique de l’adolescent. D’autre part, la plainte sommeil peut correspondre à un certain nombre de troubles pédopsychiatriques chez l’adolescent. Ainsi lorsque cette plainte s’associe à un absentéisme scolaire, la prise en charge somnologique seule s’avère souvent insuffisante. Afin de comprendre le pourquoi de cette inefficacité, nous avons revu cette problématique selon le point de vue du sommeil et de la pédopsychiatrie, puis souhaité mieux caractériser le profil de ces adolescents.Pour cela nous avons élaboré un « kit de dépistage » somnologique et pédopsychiatrique duquel découle un arbre décisionnel de prise en charge. Ce kit a été testé sur l’année 2017 dans le cadre de consultations conjointes au centre du sommeil de Lyon auprès des adolescents consultants pour une plainte de somnolence ou d’insomnie associée à un absentéisme scolaire.Les résultats préliminaires sur 11 patients, montrent la prédominance d’un retard de phase du sommeil ou d’une absence de diagnostic sommeil associé dans plus de 90 % des cas à des difficultés anxiodépressives allant de l’épisode dépressif caractérisé aux situations de refus scolaire anxieux et ses troubles anxieux sous-jacents. Les somatisations sont également fréquentes.Ces premières données semblent confirmer la nécessité d’une évaluation pédopsychiatrique afin de prendre en charge les difficultés psychologiques de ces adolescents en parallèle de leur plainte sommeil et ainsi de leur offrir les meilleures chances d’amélioration, de rescolarisation et d’insertion sociale.Adolescent sleep is characterized by a physiological delayed sleep phase disorder frequently exacerbated by the intensive use of information and communication technologies. The sleep restriction thus induced during schooling has consequences on the physical and psychological health of the adolescent. On the other hand, the sleep complaint may correspond to psychiatric disorders in the adolescent. Thus, when this complaint is associated with school absenteeism, the management of sleep alone is often insufficient. In order to understand the reason for this inefficiency, we wanted to better characterize the profile of these adolescents.We have developed a somnological and psychiatric “screening kit” resulting in a management decision tree. This kit was tested in 2017 as part of joint consultations at the Lyon Sleep Center in adolescents who presented a complaint of sleepiness or insomnia associated with school absenteeism.These preliminary results on 11 patients show the predominance of a delayed sleep phase syndrome or an absence of sleep diagnosis associated in more than 90 % of cases with anxiety-depressive difficulties ranging from the mood depressive disorder to the school refusal behavior and underlying anxiety disorders. Somatization is also common.These first data seem to confirm the need for a child and adolescent psychiatric assessment to deal with the psychological difficulties of these adolescents in parallel with their sleep complaint so as to offer them the best chances of improvement, re-schooling and social insertion.