Purpose. - Clinical reasoning and treatment challenges within the scope of general practice led to the development of an internal medicine assistance line provided by Nantes University Hospital. The primary outcome of this study was to describe callers' profile, their requests and answers provided.Methods. - A prospective, cross-sectional, observational, descriptive study was undertaken. For each call were identified the calling physician, her/his specialty and work setting, the call's object and adequacy, the answer provided, the time needed to connect with the assistance line, the time devoted by the internal medicine physician to provide an answer to the request, and whether the assistance line prevented a visit to the emergency room. Each calling physician was then called back to obtain demographic and professional characteristics, and data relating to the call and to the assistance line.Results. - Sixty-three days were analyzed and 276 calls identified. The 237 identified calling physicians were mainly females (54%, n = 93), with a mean age of 46 years, graduated from Nantes University (65%, n = 86), practicing ambulatory general medicine (69%, n = 164) in Loire-Atlantique department area (82%, n = 176) for a mean duration of 15 years. Calls were mostly associated with diagnostic challenges (61%, n = 166) concerning clinical issues (57%, n = 155). A sole telephone advice was the main type of answer provided (56%, n = 147) and a visit to the emergency room was prevented for 17% of calls.Conclusion. - The assistance line activity is adequate with its missions and seems to facilitate patients' healthcare delivery advocating for the development of similar structures in other units. Improvements relating to the information, availability and physicians' training should be considered. (C) 2015 Societe nationale francaise de medecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

Poncet's disease is a rare polyarthritis occurring in patients with tuberculous infections. We describe two cases of polyarthritis, one secondary to pulmonary tuberculosis, the other secondary to tuberculous infection of the elbow joint. In both cases the arthritis rapidly subsided with chemotherapy.La maladie de Poncet est une polyarthrite rare qui survient chez les tuberculeux. Nous decrivons deux cas de polyarthrite, l'un secondaire à une tuberculose pulmonaire, l'autre secondaire à une infection tuberculeuse de l'articulation du coude. Dans les deux cas, l'arthrite a rapidement cédéà la chimiothérapie.La enfermedad de Poncet es una poliartritis que se observa raramente en los tuberculosos. Describimos dos casos de poliartritis, uno secundario a una tuberculosis pulmonar, el otro secundario a una infeccion tuberculosa de la articulacion del codo. En ambos casos la artritis cedio rapidamente a la quimioterapia.

Background Aortitis is a potential life-threatening complication of GCA. Up to 30-50% of cases of GCA may have clinical or radiological signs of aortitis (1). Images of aortic involvement may be non symptomatic and their real outcome remain elusive. Objectives This study was conducted to describe the long-term outcome of patients with or without suspicion of aortitis at the time of diagnosis of GCA. Methods Twenty-two patients with newly diagnosed biopsy-proven GCA were explored in 1999 using aortic computed tomodensitometry (CT). Ten patients (group 1) had aortic inflammatory thickenings ≥3mm (n=7) and/or aneurism (n=3), whereas 12 patients had no aortitis (group 2). A retrospective study of these 2 groups was conducted in 2011. We contacted and questioned the patients, their family and general practitioner, and analysed each medical file. The following items were investigated: demographic data, cardio-vascular risk factors, total and cardio-vascular mortality, cardio-vascular events, GCA relapses, corticosteroids regimen. Satistics were made using R development Core Team (2009) software. Results This study included 17 women and 5 men (mean age at diagnosis = 73.7±7.2 y). Inflammatory parameters and cardio-vascular risk factors were similar in group 1 and 2. The mean follow-up was 94.8 months. Twelve years after diagnosis of GCA, the total mortality was 50% without differences between group 1 (7/10) and group 2 (5/12). However, the 12y cardio-vascular mortality was statistically higher in patients with initial suspicion of aortitis (50%), than in patients without (0%, p=0.029, Log rank test). In group 1, the causes of deaths of cardiovascular origin were: rupture of abdominal aortic aneurism (n=1), thoracic aortic dissection (n=1), stroke (n=1), heart failure (n=1), peripheral arterial disease (n=1). Twelve cardio-vascular events occured in 7/10 patients of group 1 whereas only 5 occured in 4/12 patients of group 2. Stroke were statistically more frequent in group 1 (40% vs 0% in group 2, p=0.03). Recurrent GCA relapses were noted in 5/10 patients of group 1, 0/12 patients of group 2 and this difference was statistically significant (p=0.01). Moreover, definitive steroid treatment discontinuation was more frequent in group 2 (n=2) than in group 1 (n=8, p<0.05). Conclusions Despite the limitations due to its retrospective character and its small number of patients, our study suggests that GCA clinical course may differ according to initial CT signs of aortic involvement. CT suspicion of aortitis may lead to aortic fatal events and aortic thickenings deserve to be monitored. Initial aortic CT involvements seem to sign a particular form of GCA, with higher rate of cardio-vascular events and mortality, and with frequent relapses requiring longer steroids treatment. References Agard C, Barrier JH, Dupas B, et al. Arthritis Rheum 2008;59(5):670-6 Disclosure of Interest None Declared

complement component. On a longitudinal analysis of SSc duration, as measured by the interval from the onset of the first nonRaynaud’s symptom, the proportion of patients with hypocomplementaemia remained unchanged. Similarly, there was no significant difference between hypocomplementaemic and normocomplementaemic patients with the changes in mRSS, pulmonary arterial pressures, diffusing capacity of the lung for carbon monoxide (DLCO), and forced vital capacity (FVC) at follow-up (data not shown). The prevalence of hypocomplementaemia in our multicentre analysis was lower than that registered by others (14–17%) (9, 10). Cuomo et al reported an association between hypocomplementaemia and skin, vascular, heart, and lung involvement (10). These discrepancies cannot be explained by a higher proportion of SSc overlap syndromes, as they had been excluded in the study of Cuomo et al, but their analysis might have included patients with autoantibodies other than anti-Scl70 and anti-centromere. Our study is limited by the lack of a centralized assessment of the labile analyte. Measuring degradation products could have been a more sensitive measure of complement activation. On the contrary, complement activation may occur in vitro during blood processing and increase the prevalence of hypocomplementaemia. This artefact, however, cannot account for the relatively low prevalence of hypocomplementaemia encountered in our study. Finally, overlap syndromes patients are not specifically excluded in EUSTAR, but they are more likely to be hypocomplementaemic than those with pure SSc (9). In summary, our results do not support a role of C3 and C4 measurements in the assessment of pure SSc. Acknowledgements

INTRODUCTION:Myelitis occurs in less than 5% of the patients during the disease course of systemic lupus erythematosus (SLE). Longitudinal myelitis, characterized by inflammatory involvement of at least four medullar segments, is a particular form of myelitis.CASE REPORT:We report a 31-year-old woman with SLE, admitted for paraparesia and delirium. Lumbar puncture and MRI led to the diagnosis of longitudinal myelitis. The patient rapidly improved after corticosteroid therapy.CONCLUSION:Transverse myelitis in SLE patients has been already commonly reported, but longitudinal myelitis is uncommon. Longitudinal myelitis has to be suspected in case of paraplegia or tetraplegia, with sensory defects and bladder dysfunction. MRI shows typically T2 medullar hypersignals. This may result in neurologic sequela. Cyclophosphamide has been used in patients where corticosteroids were inefficient.

L'hypertension artérielle pulmonaire (HTAP) est une complication grave de la sclérodermie systémique (ScS) et une cause importante de mortalité. Des publications récentes évaluent la prévalence de l'HTAP à environ 10 à 15%. Le pronostic reste plus sombre que celui de l'HTAP idiopathique.Les recommandations de l'OMS sont de dépister annuellement l'HTAP chez tout patient atteint de ScS même en l'absence de symptôme clinique par échocardiographie. Le cathétérisme cardiaque droit est obligatoire pour confirmer le diagnostic. Malgré ces recommandations, plus de 50% des patients atteints de ScS développant une HTAP sont en classe III ou IV NYHA au moment du diagnostic.La prostacycline apportée par voie intraveineuse en perfusion continue (époprosténol) a démontré son efficacité chez les patients atteints d'HTAP tant idiopathique qu'associée à la sclérodermie. Les analogues de la prostacycline (comme le tréprostinil et l'iloprost) sont d'autres options thérapeutiques possibles.Le bosentan est le premier antagoniste des récepteurs de l'endothéline. Il est approuvé en Europe pour le traitement de l'HTAP idiopathique et l'HTAP associée aux connectivites chez les patients en classe III de dyspnée.Le sildénafil, un inhibiteur sélectif de la phosphodiestérase de type 5, a récemment démontré son efficacité dans l'HTAP idiopathique et associée aux connectivites. Il est aujourd'hui approuvé en Europe pour le traitement de l'HTAP idiopathique et de l'HTAP associée aux connectivites en classe III de dyspnée, mais jusqu'ici nous n'avons aucune donnée à long terme dans la sclérodermie.Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc) and a leading cause of death in patients with it. Recent publications suggest that a prevalence of 10-15% is likely. The prognosis remains poor compared to that of idiopathic PAH.WHO recommends annual echocardiography for PAH screening of patients with SSc. Right heart catheterization is necessary to confirm the diagnosis. Nevertheless, more than half of all SSc patients have symptoms classified as WHO functional class III or IV at diagnosis.Prostacyclin therapy, delivered via continuous intravenous infusion (epoprostenol), has been demonstrated to be effective in patients with severe PAH (both idiopathic and scleroderma-related). Prostacyclin analogs (such as treprostinil and iloprost) are other options.Bosentan is the first endothelin receptor antagonist approved in the EU for the treatment of PAH, both idiopathic and related to connective tissue diseases such as scleroderma, in patients in WHO functional class III.Sildenafil by its selective inhibition of phosphodiesterase type 5 is also effective against both types of PAH. It too is now approved in the EU for this purpose in patients in WHO functional class III, but we do not yet have any information about its long-term effects in scleroderma.

Introduction. - Myelitis occurs in less than 5% of the patients during the disease course of systemic lupus erythematosus (SLE). Longitudinal myelitis, characterized by inflammatory involvement of at least four medullar segments, is a particular form of myelitis.Case report. - We report a 31-year-old woman with SLE, admitted for paraparesia and delirium. Lumbar puncture and MRI led to the diagnosis of longitudinal myelitis. The patient rapidly improved after corticosteroid therapy.Conclusion. - Transverse myelitis in SLE patients has been already commonly reported, but longitudinal myelitis is uncommon. Longitudinal myelitis has to be suspected in case of paraplegia or tetraplegia, with sensory defects and bladder dysfunction. MRI shows typically T2 medullar hypersignals. This may result in neurologic sequela. Cyclophosphamide has been used in patients where corticosteroids were inefficient. (C) 2011 Societe nationale francaise de medecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

La maladie de Rendu-Osler ou télangiectasie hémorragique héréditaire touche une personne sur 5000 à 8000. Cette maladie est caractérisée par la présence d'épistaxis récidivantes, de télangiectasies cutanéo-muqueuses avec un mode de transmission autosomique dominant. Les complications des malformations vasculaires cutanéo-muqueuses sont avant tout hémorragiques alors que les manifestations viscérales, pulmonaires, hépatiques ou neurologiques centrales sont liées à la présence de fistules artério-veineuses. Les trois gènes identifiés dans la maladie de Rendu-Osler (ENG, ACVRL1, MADH4) codent pour des protéines impliquées dans la voie de signalisation du TGFβ dans la cellule endothéliale, à l'origine d'une hyperprolifération endothéliale. La prise en charge des patients repose actuellement sur le dépistage de malformations artério-veineuses viscérales et des mesures symptomatiques. Pour autant, le caractère angiogénique de cette pathologie laisse entrevoir une possibilité thérapeutique intéressante par le recours à des modulateurs de l'angiogenèse.Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is a development disorder of the vasculature characterized by telangiectases and arteriovenous malformations in specific locations. Among monogenic disorders, it is one of the most common, though affected individuals are widely underdiagnosed. The most common features of this disorder, nosebleeds, and telangiectases on the lips, hands, and oral mucosa are often quite subtle. Mutations in at least five genes may result in hereditary hemorrhagic telangiectasia, but mutations in two genes (ENG and ACVRL1/ALK1) account for approximately 85% of cases. Optimal management requires understanding the specific clinical patterns of these vascular malformations, especially their locations and timing during life. Therapeutic modulation of angiogenesis may be an effective therapy.