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El sindrome de Sjogren es una exocrinopatia autoinmunitaria caracterizada por la asociacion de queratoconjuntivitis seca, xerostomia y manifestaciones sistemicas de naturaleza inmunoinflamatoria. La presencia de anticuerpos antinucleares es frecuente, en particular de tipo anti- SSA y anti-SSB. La biopsia de glandulas salivales accesorias es el elemento clave del diagnostico. Ademas del tratamiento sintomatico del sindrome seco, pueden indicarse una corticoterapia e inmunosupresores en las formas graves con manifestaciones extraglandulares. A largo plazo, el riesgo de linfoma es mas elevado que en la poblacion general.

Background Vascular thromboembolism (VTE) complicating cytomegalovirus (CMV) primary infection is increasingly reported in immunocompetent adults. No guideline is, however, currently available for the management of these infections and particularly for the antiviral therapy indication. Methods We performed a literature review of VTE complicating CMV primary infection in immunocompetent adults using PubMed. Results Sixty-nine case patients of VTE complicating CMV primary infection were reported. The main sites of venous thrombosis were the splanchnic veins (30 patients) or those of the lower limbs (18 patients). One-third of patients presented with pulmonary embolism (25 patients). Forty-nine patients (76%) had at least one VTE risk factor, inherited or acquired thrombophilia for 37 patients (58%), and another risk factor for 27 patients (42%). Only 11 patients received an antiviral therapy. A positive outcome was observed in all patients. Conclusion We suggest that antiviral therapy should be considered for patients presenting with severe VTE, VTE with a negative outcome despite anticoagulation, severe organ involvement, or for patients managed in the intensive care unit.

Gaucher disease is a genetic lysosomal storage disorder due to a glucocerebrosidase deficiency. Type 3, including neurological impairment, may have a specific phenotype in the context of the D409H mutation.We report the case of a 22-year-old woman who presented with Gaucher disease. Enzyme replacement therapy by imiglucerase was followed by rapid clinical and biological improvement. However, communication difficulties, which were initially attributed to the language barrier, revealed neurological impairment. After complementary assessment, the diagnosis of type 3 Gaucher disease was suspected. Gene analysis of the glucocerebrosidase showed a homozygous D409H mutation.This mutation results in calcified heart valves, corneal opacities, alteration of oculomotricity and hydrocephalus. The mild manifestation at onset and the late neurological involvement in the medical history make the diagnosis more difficult. This particular clinical phenotype deserves to be known in adult medicine departments.

Summary Background To date, there are no large studies on videocapsule endoscopy in systemic sclerosis (SSc). Consequently, the prevalence and features of gastrointestinal mucosal abnormalities in SSc have not been determined. Aims To determine both prevalence and characteristics of gastrointestinal mucosal abnormalities in unselected patients with SSc, using videocapsule endoscopy. To predict which SSc patients are at risk of developing potentially bleeding gastrointestinal vascular mucosal abnormalities. Methods Videocapsule endoscopy was performed on 50 patients with SSc. Results Prevalence of gastrointestinal mucosal abnormalities was 52%. Potentially bleeding vascular mucosal lesions were predominant, including: watermelon stomach (34.6%), gastric and/or small intestinal telangiectasia (26.9%) and gastric and/or small intestinal angiodysplasia (38.5%). SSc patients with gastrointestinal vascular mucosal lesions more often exhibited: limited cutaneous SSc (P = 0.06), digital ulcers (P = 0.05), higher score of nailfold videocapillaroscopy (P = 0.0009), anaemia (P = 0.02), lower levels of ferritin (P

Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. A heteroquestionnaire was offered to scleroderma patients the Lille University Hospital. Data were cross-checked with the clinical database of the National Observatory of scleroderma patients. We used 104 questionnaires of which 84% are women. A total of 62.5%, (95% CI [52.5 to 71.8]) patients had experienced DP related to SSc, 55% reported symptoms worsened at work, 41% lower efficiency and a 19% job loss. Symptoms responsible for their DP were mainly asthenia, Raynaud syndrome, arthralgias and finger ulcerations. Only 40% of patients in difficulty were initiating steps with the county home for disabled people and 45% in informing their doctor at work (MT). The MT information absence was related to a misunderstanding of possible aid in 45% of cases, 57% of patients had not found a solution, 41% of them have lost their jobs and 31% of patients in the cohort experienced a drop in income. The impact of SSc the work is important. The aid job retention devices are under-used due to lack of knowledge of steps to take and the role of MT. Taking into account the symptoms responsible for PD must be improved to participate in this retention.

More than 30 years after its individualization, chronic fatigue syndrome (CFS) remains a debilitating condition for the patient and a confusing one to the physicians, both because of diagnostic difficulties and poorly codified management. Despite the numerous work carried out, its pathophysiology remains unclear, but a multifactorial origin is suggested with triggering (infections) and maintenance (psychological) factors as well as the persistence of inflammatory (low grade inflammation, microglial activation…), immunologic (decrease of NK cells, abnormal cytokine production, reactivity to a variety of allergens, role of estrogens…) and muscular (mitochondrial dysfunction and failure of bioenergetic performance) abnormalities at the origin of multiple dysfunctions (endocrine, neuromuscular, cardiovascular, digestive…). The complexity of the problem and the sometimes contradictory results of available studies performed so far are at the origin of different pathophysiological and diagnostic concepts. Based on a rigorous analysis of scientific data, the new American concept of Systemic Disease Exertion Intolerance proposed in 2015 simplifies the diagnostic approach and breaks with the past and terminologies (CFS and myalgic encephalomyelitis). It is still too early to distinguish a new disease, but this initiative is a strong signal to intensify the recognition and management of patients with CFS and stimulate research.

La sindrome di Sjogren e un’esocrinopatia autoimmune caratterizzata dall’associazione di una cheratocongiuntivite sicca e di una xerostomia e da manifestazioni sistemiche di natura immunoinfiammatoria. La presenza di anticorpi antinucleo e frequente, in particolare di tipo anti-SSA e SSB. La biopsia delle ghiandole salivari accessorie e l’elemento fondamentale della diagnosi. Oltre al trattamento sintomatico della sindrome sicca, la corticoterapia e gli immunosoppressori possono essere indicati nelle forme gravi con manifestazioni extraghiandolari. A lungo termine il rischio di linfoma e piu elevato che nella popolazione generale.

Resume Les blocs auriculoventriculaires congenitaux d’origine immunologique sont lies au passage transplacentaire d’anticorps anti-SS-A/Ro et/ou anti-SS-B/La maternels pouvant s’integrer dans le cadre d’une connectivite. L’echographie avec Doppler est essentielle pour le depistage de ces blocs auriculoventriculaires au cours des grossesses a risque. Elle permet le diagnostic des blocs du premier ou deuxieme degre qui constituent probablement des etapes preliminaires dans l’atteinte des voies de conduction. Un traitement maternel par corticoides peut dans cette situation etre propose au couple, en raison d’un possible effet de cette therapeutique sur les blocs du premier ou deuxieme degre. En tant que signes precoces d’une lesion nodale, ils peuvent toutefois manquer : certains fœtus developpent un bloc complet sans qu’aucun bloc de degre inferieur n’ait ete identifie prealablement. De meme, la valeur semiologique du bloc du premier degre n’est pas totalement claire puisqu’il semble pouvoir etre spontanement reversible. D’autres marqueurs de lesion nodale seraient donc precieux. En cas de bloc complet, l’echographie est utile pour identifier des signes de decompensation cardiaque et guider la conduite obstetricale et l’extraction fœtale sur la base de signes pronostiques defavorables.

Pulmonary fibrosis in systemic sclerosis (SSc) involves inflammatory processes in the lower respiratory tract. Analysis of exhaled breath condensate (EBC) is a non-invasive method for studying inflammatory mediators, such as cytokines, which are of interest from both physiological and therapeutic perspectives. The aim of this study was to assess and compare cytokine concentrations in the EBC of SSc patients and controls.EBC was collected from 19 SSc patients and 19 controls. We used a multiplex assay test kit to assay interleukin (IL)-2, -4, -6, -10, tumour necrosis factor-alpha, and interferon-gamma in samples concentrated by lyophilization.Cytokine concentrations in EBC were higher in SSc patients than in controls. The stepwise analyses showed that IL-4 was the biomarker which contributed most to the discrimination between controls and patients (Wilk's Lambda = 0.55, p0.001). We observed significant negative correlations of EBC cytokines with total lung capacity and diffusion capacity of the lung for carbon monoxide.These findings suggest that EBC sampling permits the non-invasive study of inflammation in SSc patients, and may be correlated with the severity of interstitial lung disease.

The vascular disorders in systemic lupus erythematosus (SLE) result from various mechanisms and presentations (inflammatory disease or vasculitis, atherosclerosis).We report on a 34-year-old man with cutaneous, articular, neurological and nephrologic SLE. He presented with catastrophic haemorrhage on microaneurysm rupture of the left hepatic artery. After blood transfusions and immunosuppressive treatments, his condition improves.Uncommon complication in SLE patients, digestive vasculitis with microaneurysms may occur as in polyarteritis nodosa. In the literature, we identified 10 additional cases of hepatic microaneurysms in SLE patients. The main issue is an earlier diagnosis in order to give appropriate treatment and improve prognosis.

Hyperhomocysteinemia is a biological marker that could be identified in the venous thrombotic events and rarely during acute arterial thrombotic events. The consequences can be serious. Effective diagnostic strategy is needed to optimize the management.Following bariatric surgery, a 40-year-old patient was admitted with an acute encephalopathy associated with peripheral lower limb arterial ischemia. The diagnostic work-up identified a major hyperhomocysteinemia whose causes were several. Surgical treatment and anticoagulation was associated with vitamins and trace elements supplementation. Correcting deficiencies allowed delirium and hyperhomocysteinemia improvement. Once treatment established, the patient did not present a recurrent thrombotic episode.Major hyperhomocysteinemia seems to be associated with an increased risk of acute arterial thrombosis. This marker might be considered in nutritional deficiency situations with appropriate support on the vascular, metabolic and nutrition level.

The main objective of the study was to assess the adequacy of antibiotic therapy for urinary tract infections (UTI) in a French hospital medical department. The secondary objective was to identify factors associated with inadequacy of the antibiotic therapy.A retrospective single centre cohort study was performed in the Post-Emergency Medicine Department (PEMD) of the university hospital of Lille. All patients presenting with an UTI from May 2012 to April 2014 were included. Adequacy of antibiotic therapy was assessed with reference to local guidelines. Factors associated with inadequacy of antibiotic prescription were determined using a multivariate logistic regression model.Two hundred and twenty-eight patients were included. The antibiotic prescription was fully adequate in 173 patients (76%) with appropriate use of a single or a combination antibiotic therapy in 96%, appropriate drug in 80%, appropriate dosage in 89% and appropriate route of administration in 95%. The risk for antibiotic inadequacy was significantly higher in patients with cystitis than in those with pyelonephritis (OR 12.01; 95% CI 4.17-34.65), when antibiotics were prescribed in the Emergency Department (OR 6.84; 95% CI 2.29-20.47) or before hospital admission (OR 382.46; 95% CI 19.61≥999.99) compared to when antibiotics were first administered in the PEMD, and in patients with severe UTI (OR 19.55; 95% CI 2.79-137.01).Adequacy of antibiotic therapy for UTI is relatively high in our study, reflecting the effective dissemination of antibiotic guidelines. However, antibiotic therapy is still inappropriate in cystitis, severe UTI and in case of prescription before the admission in the PEMD.

Among the biological assays used for the diagnosis of systemic lupus erythematosus (SLE), the detection of anti-double strand DNA antibodies (dsDNA Ab) is regarded as highly specific. However this biological parameter is negative among 20 to 40% of patients. Recent studies have revealed potential interest of the anti-nucleosome antibodies in the diagnosis of the lupus, in particular when any anti-dsDNA antibody activity could be detected. We selected 80 sera in order to evaluate four commercial anti-nucleosome enzyme-based immunoassays (EIA) kits. Their sensitivity and specificity values were compared with those obtained by the detection of anti-dsDNA Ab, carried out with both a Farr assay and two EIA kits. No anti-nucleosome EIA kits reached performances of the Farr assay for the diagnosis of lupus. On the other hand, our results show an higher diagnostic value for some anti-nucleosome EIA kits compared with 2 anti-dsDNA EIA kits. Apart from SLE, anti-nucleosome antibodies can be observed in others auto-immune diseases, in particular Sjogren's syndromes, the primary antiphospholipid syndrome, the systemic sclerosis and the mixed connective tissue disease. Compared results of the four anti-nucleosome EIA kits highlight many discordances. These variations, testifying to the absence of standardization for this new parameter, must encourage with a careful interpretation of results, according to the clinical context.

Patients hospitalized in internal medicine often have unexplained clinical symptoms for which a drug origin can be considered. The prevalence of patients hospitalized for iatrogenic is estimated between 4-22%. We wanted to evaluate the diagnostic value of the regional center of pharmacovigilance to identify or confirm an iatrogenic disease in the department of internal medicine of Lille and characterize factors associated with drug-related side effect.This is a single-center prospective diagnostic study. We included all subsequent requests from the department of internal medicine with the Nord-Pas-de-Calais regional pharmacovigilance center between 2010 and 2012. The opinion of the regional pharmacovigilance centre was held on the record of the adverse drug reaction in the national pharmacovigilance database and analyzed according to the conclusion of iatrogenic used by clinicians in internal medicine (reference diagnosis) with a follow-up to June 2013. The variables relating to the patient, medication and adverse events were analyzed by binary logistic regression.We analyzed 160 contacts: 118 concordant cases, 38 false-positives (drug-related side effect retained by the regional pharmacovigilance center only), 4 false negatives. Registration in the national pharmacovigilance database had a sensitivity of 96% (95% CI [0.92 to 0.99]), a specificity of 46% (95% CI [0.38 to 0.53]), a value positive predictive of 69% (95% CI [0.62 to 0.76]), a negative predictive value of 89% (95% CI [0.84 to 0.94]) and a negative likelihood ratio of 0.1. False-positive had chronological and semiological accountabilities questionable (adjusted RR=2.1, 95% CI [1.2 to 2.8]).In our study, the regional pharmacovigilance center confirms the clinician's suspicion of drug-related side effects and helps to exclude drug-induced with a high negative predictive value.

Multicentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia.We report a 32-year-old woman with an association of oligoanuria, anasarca, thrombotic microangiopathy with features compatible with adult-onset Still disease. The outcome was initially favorable with corticosteroids, immunoglobulins and plasmapheresis but with the persistence of relapses marked by severe autonomic syndrome and necessity of high dose corticosteroids. The diagnosis of mixed type Castleman's disease, HHV8 and HIV negative, was obtained four years after the onset of symptoms by a lymph node biopsy. The outcome was favorable after tocilizumab and corticosteroids but tocilizumab had to be switched to anakinra to ensure a proper and long-lasting control of the disease.Our patient partially fits the description of TAFRO syndrome (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly), a MCM rare variant, recently described in Japanese patients.

A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.

This review outlines the skin, vascular and musculoskeletal symptoms affecting the hand during systemic inflammatory diseases other than rheumatoid arthritis. Skin lesions are diagnosed clinically and their symptomatology is documented through an extensive series of photographs. These conditions may require specific care before a surgical procedure can be performed. Vascular lesions are also diagnosed clinically and their symptomatology is described in detail. It is important to recognize that acrocyanosis is always benign. The surgeon should be able to distinguish between primary, but benign Raynaud's disease and secondary Raynaud's syndrome, which has a high risk of finger necrosis. Current preventative and curative treatments for finger necrosis are described. The clinical, radiological, progressive and therapeutic features of musculoskeletal lesions are reviewed, namely those associated with psoriatic arthritis, systemic sclerosis and lupus.

To assess the outcome of interstitial lung disease (ILD) in anti-Jo-1 patients with antisynthetase syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti-Jo-1 patients with and without ILD.Ninety-one anti-Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans.Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 12), progressive onset of lung signs (n = 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n = 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age ≥55 years. Furthermore, anti-Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels.Our findings confirm that ILD is a frequent complication in anti-Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti-Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.

Systemic lupus erythematosus (SLE) is an autoimmune disease, which primarily affects skin and joints. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. A classification, published in 1999 by the American College of Rheumatology (ACR) research committee, described 12 CNS syndromes and seven peripheral neurologic syndromes compatible with "neuropsychiatric systemic lupus erythematosus" (NPSLE). Despite this consensus, studies which have been published since 1999 have reported a prevalence of NPSLE varying from 20 to 97 %, which shows the diagnosis difficulty and the heterogeneity of neuropsychiatric manifestations in SLE. In order to understand the limits of this classification, we propose in this first part an exhaustive review of publications describing neuropsychiatric manifestations according to the ACR 1999 classification. We also detail case definitions, prevalence and risk factors, clinical characteristics and diagnosis of each lupus-related psychiatric and CNS manifestation.

Neurological and psychiatric manifestations of systemic lupus erythematosus are a heterogeneous set of clinical manifestations grouped under the term of "neuropsychiatric systemic lupus erythematosus". The classification of these manifestations published in 1999 has harmonized the definitions cases used in the studies but did not help the clinician to positively identify a specific manifestation of lupus or a neurological or psychiatric event occurred independently of the disease. Published cases series help us to identify neurological or psychiatric manifestations of lupus but modern diagnosis tools contribution have to be evaluated in order to optimize diagnosis management of such manifestations and to distinguish specific events related to lupus and independent manifestations. In this second part of our literature review about neuropsychiatric lupus, we propose to identify arguments, which could be in favor of lupus responsibility in front of a neurological or psychiatric event, and immunosuppressive treatments which are recommended.

In patients with visual hallucinations, diagnostic strategy is unclearly codified. In patients known to have giant cell arteritis, the main diagnostic assumption is disease relapse. Indeed, this should lead to rapid corticosteroid therapy. However, the Charles Bonnet syndrome, that is a poorly known etiology of visual hallucinations usually observed in elderly people, should be part of the differential diagnosis. We report a 87-year-old woman, with a 2-year history of giant cell arteritis who was admitted with an acute onset of visual hallucinations and who met all the criteria for Charles Bonnet syndrome.

International audience; OBJECTIVE: To study evolution of pSS immunological profile, impact on pSS activity and the long-term evolution of patients with atypical auto-antibodies in a bicentric cohort of patients with pSS (n=445, mean age 53.6+/-14years, mean follow-up 76.1+/-51months). RESULTS: 212 patients were SSA positive and 131 were both SSA and SSB positive. During follow-up, SSA antibodies disappear in 8 patients; 2 of them exhibit new systemic complications of pSS. 68 patients had cryoglobulinemia. 52 patients had other anti-nuclear antibodies (ANA) specificities: anti-RNP (n=12), anti-centromere (n=14), anti-DNA native (n=19), anti-Scl70 (n=3), anti-JO1 (n=3), anti-Sm (n=3) and anti-histone (n=1). Fourteen patients developed ANA-associated auto-immune disease during the follow-up: 5 polymyositis (mean apparition delay 78months), 6 systemic lupus erythematosus (mean occurrence delay 77months) and 2 systemic sclerosis (mean occurrence delay 133+/-64months). Among these 14 patients, only three presented atypical-ANA at pSS diagnosis. Cryoglobulinemia and anti-SSA and SSB antibodies at diagnosis were associated with new systemic involvements. IN CONCLUSION: Cryoglobulinemia and SSA/SSB positivity are associated with systemic activity after diagnosis in pSS. Although atypical ANA are found in 12% of the cases, long-term evolution to ANA associated auto-immune diseases concerned patients with active immunological profile and extra-glandular manifestations.

Valvular incompetence with reflux and postthrombotic syndrome are the most common features of varicose veins, a common disease. More rare etiologies must be evoked when these two main causes have been ruled out. We report herein the case of a 47-year-old man who has been suffering from varicosis and complained with left leg pain since 15 years. He had already been managed by standard stripping, saphenous ligations, phlebectomies but was not completely relieved. X-ray findings of the tibia, doppler ultrasonography and magnetic resonance imaging led us to the diagnosis of tibial intraosseous venous drainage anomaly. We then report diagnosis and therapeutic decisional approach.

Use of low-molecular-weight heparin (LMWH) is widespread. Clinicians are well aware of LMWH-related thrombopenia, but reports of thrombocytosis are more exceptional. We evaluated prospectively the incidence of thrombocytosis (450 G/L) in the general medicine setting among patients needing LMWH treatment.We followed for the duration of treatment 95 consecutive patients receiving LMWH and managed in a general medicine setting. Thrombotic events, bleeding and platelet counts were noted.Among the 95 patients, 29 developed thrombocytosis during the follow-up (587±102 G/L). In 15 patients, thrombocytosis occurred early after discharge ; in 14 others the counts rose to a pathological level on average 5.4±0.7 days after discharge then returned to normal levels spontaneously in 10.7±7.9 days. Only one clinical event (erysipelas) was reported, potentially associated with this thrombocytosis. There were no thrombotic or hemorrhagic events during the follow-up.In our population, the observed cases of thrombocytosis were moderate. Incidence was however not exceptional despite the absence of any notable adverse event, in agreement with the rare data in the literature.

Systemic sclerosis (ScS) is very heterogeneous in its clinical presentation and its therapeutic care is not codified. A better knowledge of the patients' needs and complaints could improve the patient educational strategies and their global care.A self-administered questionnaire aimed to the ScS patient was developed by subspecialty physicians and nurses involved in patient education. It was a cross-sectional study that also included several validated scales: the health control locus scale, the Mactar, HAD and sHAQ scales.One hundred and eight patients (91 women; 18 limited ScS, 71 limited cutaneous ScS, 19 diffuse ScS) filled in the questionnaires. Fatigue was the main complaint in all types of ScS, independently of the ScS type. The aesthetic discomfort mentioned by the patients suffering from cutaneous sclerosis or from telangectasia was important and reached 52±33mm on a 100-mm visual scale. It was more common in the patients presenting a diffuse form of the illness but the difference did not reach a statistical significance (P=0.06). Twenty-seven percent of the patients said they were very or extremely worried because of the degradation of their physical appearance. The functional discomfort linked to the cutaneous sclerosis was rated 50±32mm on a 100-mm visual scale. The intensity of the pain, the importance of the functional discomfort linked to the sclerosis and the intensity of the dyspnea were correlated to the sHAQ (P0.001). Patients having more frequent recurrent digital ulcers had higher sHAQ scores (P=0.04). The repercussions on the professional life were linked to fatigue first, to the Raynaud's syndrome and to arthralgia. The repercussions on the personal life were mainly linked to the fatigue, the pain and the dyspnea. The patients' compliance was good.Fatigue, pain, dyspnea and discomfort linked to sclerosis are major chronic symptoms of the patients with ScS. Identifying the needs and complaints of the patients with ScS should help to improve their care by implementation of an educational program.

Temporal arteritis is a large-vessel vasculitis predominantly affecting the external carotid and its branches. Venous thrombosis is rarely found at the onset of temporal arteritis, particularly when venous symptoms precede arterial involvement. We report the case of a 70-year-old woman consulting for bilateral superficial frontal venous thrombosis. Superficial bilateral temporal venous thrombosis occurred under adequate anticoagulation before the onset of arterial symptoms suggestive of temporal arteritis. We then discuss the pathophysiology of venous thrombosis in patients with temporal arteritis.

Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of progressive systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, polymyositis/dermatomyositis, with a high anti-snRNP antibody titer. Respiratory manifestations, such as interstitial lung disease (ILD), are not well-described. Thirteen patients who met the diagnostic criteria for MCTD and showed ILD on high-resolution CT were analysed retrospectively. A restrictive pattern was found in 73% of cases and TLCO abnormalities in 90%. Exercise hypoxemia was observed in nine out of ten cases. The CT-scan pattern was compatible with non-specific interstitial pneumonia in seven cases and with usual interstitial pneumonia in five. Bronchoalveolar lavage showed lymphocytic alveolitis in two patients, neutrophil alveolitis in eight. Fifty percent ILD patients respond to steroids and immunosuppressive drugs. Progressive ILD (six in 13; 46%) compared with non-progressive ILD associated more systemic sclerosis manifestations (p0.05). Progressive ILD tend to have more frequent pulmonary hypertension, neutrophilic alveolitis and honey combing pattern. MCTD-ILD characteristics are not specific. When systemic sclerosis manifestations are present, MCTD-ILD seems to associate more frequently pulmonary hypertension and progressive ILD.

Pneumocystis pneumonia is a serious opportunistic infection that frequently occurred in HIV-seropositive patients, prior to the advent of highly active antiretroviral therapy. This infection can also occur in patients with systemic diseases. The diagnostic value of a positive Pneumocystis jirovecii PCR in patients with systemic diseases has not yet been clearly defined.We conducted a retrospective study of patients with a systemic disease who presented clinical symptoms consistent with Pneumocystis pneumonia to assess the diagnostic value of a positive P. jirovecii PCR in respiratory samples.During a 10-year period, 73 patients with respiratory symptoms underwent respiratory sampling with tests for the presence of P. jirovecii. P. jirovecii PCR was positive in 20 patients: Pneumocystis pneumonia was diagnosed in nine patients and for six of these nine patients, the microscopic examination was negative. Patients with Pneumocystis pneumonia differed from those who were solely colonized in that they had a lower CD4+ T lymphocyte count, were more likely to have received immunosuppressive treatment, and were not receiving primary prophylaxis against Pneumocystis pneumonia. Chronic pulmonary involvement was more frequent among colonized patients.A positive P. jirovecii PCR does not always indicate overt infection. However, in a context of severe immunosuppression and in the absence of prophylaxis against Pneumocystis pneumonia, a specific treatment should be considered.

There are a few reports only on cytomegalovirus (CMV) reactivation in lupus. Diagnosis of this infection is difficult and can be associated with of a poor outcome. We report three cases of infection with CMV that occurred in patients with lupus and review the literature.The three reported patients presented with fever, polyarthritis, myocarditis and enteritis. Lupus was longstanding and the patients were receiving corticosteroids or cyclophosphamide. There was no major CD4 lymphopenia. The diagnosis was obtained with the presence of antigenemia pp65. The outcome was favorable with antiviral therapy in two patients, while the remaining patient died. In the English literature, pulmonary and intestinal involvement seem frequent, and associated with poor prognosis.In systemic lupus CMV infection is often serious and difficult to diagnose. Risk factors, treatment and prophylaxis remain to be evaluated in this population. The incidence of this infection could increase among patients receiving a biotherapy.

Euthyroid Graves' disease is defined as an ophtalmopathy without any clinical or biological signs of thyroid dysfunction. It highlights the apparent dissociation between orbitopathy and thyroid disease. Diagnosis is often too late while early treatment could really improve functional prognosis. We report a 57-year-old woman who presented with this entity and that illustrates both the diagnostic difficulty and disease course after intravenous corticosteroid therapy.

Isolated congenital heart block is linked to transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies that may be related to a connective tissue disease. Ultrasonography and Doppler are essential to screen fetus at risk. They allow the diagnosis of first- and second-degree blocks which are probably preliminary stages in conducting tissue's injury. In these situations, a maternal treatment by fluorinated steroids can be proposed because of its possible effect on partial blocks. However, these early signs of nodal injury can be lacking: some fetus present a complete heart block without previously detected less advanced block. Moreover, the significance of first-degree block is unclear since it could reverse spontaneously. Other markers of nodal injury would be valuable. In case of complete congenital heart block, ultrasonography is useful to detect congestive heart failure and help the obstetrical management when unfavorable prognostic signs occur.

International audience; OBJECTIVE: To determine whether there were any clinical and biological differences between male and female patients with primary Sjogren's syndrome (pSS) in a large bicentric series of patient. METHODS: We studied 419 consecutive patients (mean age at onset 53.6 years, mean disease outcome 73 months) with pSS according to American-European criteria, attending two different Departments of Internal Medicine in France. The 42 (9%) male patients in this cohort comprised the male group described in this study. RESULTS: Extraglandular manifestations during the course of the disease were present in 37 (89%) of our male patients with pSS. The extraglandular manifestations were similar among the two groups except that the male patients showed a lower frequency of depression or asthaenia (5% vs. 20%, p = 0.014) compared with the females. A significantly greater percentage of women reported lymphopaenia (26% vs. 8%, p = 0.02) and leucopaenia (18% vs. 3%, p = 0.015) at onset, but thrombopaenia was more common in the male patients (21% vs. 6%, p = 0.001). Lymphoma development was slightly more common in the male patients, but with no statistical significance (10% vs. 3%, p = 0.06), and occurred earlier after the SS diagnosis (log rank test p = 0.04). CONCLUSION: Although pSS is typically a disease affecting women, clinicians should be aware that it may be diagnosed in male patients. Except for haematological presentation, we could not find any notable differences in clinical and immunological characteristics between male and female patients with pSS.

Background: Treatments currently used in acrosclerosis for patients with systemic sclerosis (SS) are not very efficient and are associated with adverse effects. Several reports concern the efficacy of ultraviolet A1 (UVA1) phototherapy for localized scleroderma. Recent studies appear to indicate the interest of UVA1 in acrosclerosis for patients with SS. However, these studies are uncontrolled. Objective: To determine whether UVA1 phototherapy is effective for acrosclerosis in SS with a randomized, investigator-blinded, controlled study. Methods: Nine patients with SS completed the study. The duration of disease ranged from 6 to 21 years. None of them had received glucocorticoids or immunosuppressive agents. Low-dose UVA1 phototherapy (40 J/cm2) of the randomized hand was performed three times weekly over a period of 14 weeks. The other hand served as control. The clinical evaluation used a modified semiquantitative skin scoring system, the index flexion and extension, and a visual analog scale (VAS) was performed at baseline and after treatment. Results: The mean of skin score and VAS improved significantly (P