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Objectives Systemic sclerosis (SSc) is a heterogeneous autoimmune disease associated with several antinuclear autoantibodies useful to diagnosis and prognosis. The aim of the present multicentric study was to determine the clinical relevance of anti-fibrillarin autoantibodies (AFA) in patients with SSc. Methods The clinical features of 37 SSc patients positive for AFA (AFA+) and 139 SSc patients without AFA (AFA-) were collected retrospectively from medical records to enable a comparison between AFA- and AFA+ patients. Anti-fibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HEp2 cells and identified by an in-house Western blot technique and/or an EliA test. Results Comparing AFA+ and AFA– patients, AFA+ patients were significantly younger at disease onset (36.9 vs. 42.9; p=0.02), more frequently male (p=0.02) and of Afro-Caribbean descent (65% vs. 7.7%; p

Increased alveolar concentration of nitric oxide (CA(NO)) is related to the severity of interstitial lung disease (ILD) in systemic sclerosis (SSc). However, cut-off levels of CA(NO) to rule out, or to rule in, the presence of ILD in individual patients are unknown. We aimed to assess the validity of CA(NO) for the diagnosis of ILD in SSc and to determine the thresholds of CA(NO) that can be used in clinical practice to predict the likelihood of ILD in SSc.Lung HRCT scan, PFTs and partitioned exhaled NO measurements were performed in 65 consecutive SSc patients. ILD was diagnosed on pulmonary HRCT according to the presence of ground glass or reticular opacities. Diagnostic performance of CANo for ILD diagnosis was assessed using ROC curves.38 out of 65 SSc patients had ILD. CA(NO), at a cut-off level of 4.3 ppb, had a sensitivity and specificity for the diagnosis of ILD of 87% (95% CI: 77 to 99) and 59% (95% CI: 41 to 78), respectively. The same cut-off level of CA(NO) could detect impairment of gas exchange with a sensitivity and specificity of 78% (95% CI: 67 to 90) and 73% (95% CI: 46 to 99), respectively. Moreover, ILD could be ruled in (positive predictive value95%) when CA(NO)or = 10.8 ppb, and ruled out C(ANO) valuesor = 3.8 ppb (negative predictive value95%).CA(NO) could be a valid non-invasive biological marker of ILD in SSc, and be of use in clinical practice.

Chronic meningococcemia is an unusual clinical presentation within the spectrum of infections due to Neisseria meningitidis.We report a 32-year-old man who presented with a 15-day history of fever and maculopapular skin rash, in the absence of meningeal irritation or severe sepsis manifestation. Blood culture identified N. meningitidis. Clinical course was uneventful after antibiotic treatment was initiated.Early diagnosis of chronic meningococcemia is crucial for optimal management of the patient and his/her contacts. Such a diagnosis should be suspected in the presence of the characteristic clinical triad (recurrent fever, skin rash and arthralgia), and this clinical presentation should be distinguished from systemic vasculitis as inadequate prescription of corticosteroids may be deleterious.

Mycotic aneurysms are rare, remain asymptomatic for a long time, and may be life threatening by their rupture if therapy is delayed. Historically associated with Streptococcus pyogenes and Staphylococcus aureus, they now frequently involve Salmonella species in elderly or immunodeficient patients, and complicate vascular investigation or surgical procedures. Frequently located in the abdominal aorta, they can also be found rarely in other location. Therapy associates antibiotics and surgical debridement with reestablishment of vascular continuity. We report a case of ruptured popliteal aneurysm with Salmonella bredney bacteraemia.

CXCL10, a gamma-interferon-induced chemokine seems to play a relevant role in lung involvement that occurs in systemic sclerosis (SSc). The objective of this study was to assess the serum level of CXCL10 in interstitial lung disease (ILD) associated with SSc.Serum level of CXCL10 was assayed in 23 healthy volunteers (60.0 years; 58.0-67.3) and 29 SSc patients (63.1 years; 60.1-69.4) by ELISA method. Pulmonary function tests (PFTs), lung CT-scan and echocardiogram were also performed in the patients. Serum levels from patients and healthy controls were compared and a comparison among SSc patients between those with and without ILD, as documented by lung CT-scan, was also performed.Median CXCL10 level from patients with SSc was significantly higher than that from healthy volunteers (110.0 pg/ml; 60.8-223.8 versus 52.0; 41.3-65.8; p0.001). Fifteen out of the 29 patients had ILD on lung CT-scan; the median CXCL10 level from SSc patients with ILD was significantly higher than that from SSc patients without ILD (210.0 pg/ml; 115.0-307.5 versus 76.0; 55.0-110.0; p=0.02).Our findings suggest that CXCL10 is specifically increased in the lung involvement of SSc and plays a role in scleroderma lung disease.

A peripheral neuropathy occurs rarely during the course of an inflammatory myopathy. Once the classical aetiologies of peripheral neuropathies are ruled out, the diagnosis of neuromyositis can be accepted. We report a patient with dermatomyositis who presented a peripheral neuropathy revealed by dysautonomia. The presence of associated vasculitis led us to consider that this constituted the mechanism of the neurological involvement.

It is unknown if the level of dietary-sodium intake influences blood pressure in patients receiving systemic corticosteroids.Randomized, single centre, crossover trial involving patients starting systemic corticosteroid therapy and having initial blood pressure less or equals to 159/99 mm Hg. The first period of sodium regimen was randomized (3 g/j versus6 g/j) and each period of sodium regimen lasted 3 weeks. No washout period was performed. Blood pressure was recorded for each patient at inclusion and after 3 weeks and 6 weeks. Moreover, all patients were asked to record on a standardized questionnaire everything they ate during 1 week of each period regimen. Questionnaires were analysed by a dietician for mean daily energy and sodium intakes during each period. Mixed models were used to estimate the relationship between sodium intake and blood pressure variations.Between June 2006 and June 2008, 49 patients were randomized, 24 in group 1 (first period regimen=salt3g/day; women: 63%; mean age: 56+/-21 years; baseline prednisone dosage: 54+/-19 mg/day) and 25 in group 2 (first period regimen=salt6g/day; women: 56%; mean age: 60+/-19 years; baseline prednisone dosage: 56+/-16 mg/day). Mean daily salt intakes were 2.5+/-1.8 and 9.3+/-1.9 g/day during the first period and 7.8+/-3.2 and 3.8+/-2.9 g/day during the second period, respectively for group 1 and group 2. Blood pressure variations were not significantly associated with daily salt intakes or with randomisation group. No order effect was evidenced. By comparison with baseline, systolic blood pressure increased by greater than 20 mm Hg at week 6 in five patients (2 in group 1 and 3 in group 2).At short-term, sodium intake does not seem to influence blood pressure variations in patients starting systemic corticosteroids therapy.

Except for the prevention of osteoporosis, no consensual recommendations are available regarding the therapeutic measures associated with the prescription of long-term corticosteroid therapy. The aim of this study was to assess the internal medicine physicians' practices regarding the prescription of long-term corticosteroid therapy.In September 2007, we sent, by e-mail, a questionnaire to 813 internal medicine physicians, members of the French National Society of Internal Medicine. With this questionnaire, we assessed the frequency of prescription of measures sometimes associated with systemic corticosteroids and for whom no consensual recommendations were available (dietary advices, physical training, potassium supplementation, gastric protection, influenza vaccination and prescription of hydrocortisone).Three hundred and thirty-six out of 813 internal medicine physicians completed the questionnaire (response rate: 41%). The practitioners were predominantly male (71%) and mainly engaged in tertiary centres (53%). Regarding the dietary measures associated with the prescription of corticosteroids, low-sodium diet was recommended by most of the physicians, 69% of them prescribing such dietary regimen in more than 80% of their corticosteroid-treated patients. The concomitant prescription of caloric restriction, low-carbohydrate diet and/or high-protein diet was not consensual. The prescription of muscular physiotherapy was unusual, 74% of physicians prescribing such reeducation in less than 20% of their patients. The frequency of recommendation for daily physical training varied between physicians as well as for potassium supplementation, gastric protection, influenza vaccination or hydrocortisone prescription.There is no consensus between French internal medicine physicians regarding most of the measures, which must be prescribed in association with a long-term corticosteroid therapy.

We report a 74-year-old man with severe chronic primary neutropenia (neutrophil count: 390 per millimeter cube) uncovered following surgery for perianal abscess collection. Clinical, laboratory and roentgenographic findings revealed no abnormality. Antineutrophil antibodies were positive in two consecutive serum samples. Under cyclosporine, neutrophil count reached 1970 per millimeter cube. However, this therapy was discontinued due to new onset of severe renal failure. After six weeks, neutrophil count was 950 per millimeter cube and sirolimus was started, resulting in renal function improvement and resolution of neutropenia.

Neurogenic tumors of the small intestine are extremely rare. Although schwannoma is often clinically indolent for many years, complications such as gut compression or bleeding might occur. In these cases, surgical management is required. We reported a case of asymptomatic schwannoma of the duodenojejunal angle. Surgical treatment was performed to provide definitive immunohistochemistry diagnosis and to prevent complications.

No data is available about the natural history of the corticosteroid-induced lipodystrophy. The purpose of this study is to describe the natural history of corticosteroid-induced lipodystrophy in a selected and homogenous population.We conducted a cohort study between June 2003 and September 2005 and enrolled all consecutive patients starting long-term systemic corticosteroid therapy for giant cell arteritis (because of a standardized therapeutic schedule). After enrollment, patients were seen every month until the end of the corticosteroid therapy. After the drug withdrawal, they consulted every 3 months during 6 months. At each consultation, they were photographed in a standardized way. At the end of the study, the development of lipodystrophy was assessed by analyzing these photographs. We evaluated the incidence of corticosteroid-induced lipodystrophy during the course of giant cell arteritis therapy and the time between initiation of therapy and its apparition. Lastly, we evaluated the time between the prednisone withdrawal and the disappearance of lipodystrophy (or the mean prednisone dosage if the disappearance was observed under treatment).Thirty-seven patients were included (women: 73%; mean age: 75+/-7 years; mean initial daily prednisone dosage: 44+/-13 mg). The mean duration of follow-up was 23.6+/-7.4 months. Incidence of corticosteroid-induced lipodystrophy was 48% after 3 months and 60% after 12 months of therapy. The median time between treatment initiation and appearance of lipodystrophy was short (3 months [1-4]). During the decrease of the therapy or the 6 months following its end, we observed a regression of lipodystrophy in 71% of the patients. The median time between corticosteroids initiation and disappearance of lipodystrophy was 19 months [16-22] (concomitant median daily dosage of prednisone: 4 mg [0-7]).Lipodystrophy is frequently observed in corticosteroid-treated old patients. It appears precociously after the initiation of therapy and is usually reversible.

To evaluate the association between human leukocyte antigens (HLA) of the major histocompatibility complex (MHC) and thoracic tuberculosis (TB) in immunocompetent adults.We searched Medline, Pascal, Pascal Biomed and Francis databases (all years) with the terms 'tuberculosis' and 'HLA'. Case-control studies were included that reported frequencies for the full range of antigens analysed by serological methods in healthy controls and adult patients not treated with glucocorticoids or immunosuppressive drugs, human immunodeficiency virus status negative or not reported, no debilitating chronic disease, and with a diagnosis of thoracic TB based on microbiological or histological criteria. Two authors independently abstracted the data and resolved disagreements by consensus.We summarised 60 HLA antigens reported in at least four of 22 studies totalling 1988 patients and 2897 controls. A lower risk of thoracic TB was found in carriers of B13 (OR 0.64, 95% CI 0.50-0.81, P0.0001), DR3 (OR 0.72, 95% CI 0.59-0.89, P = 0.002), and DR7 antigens (OR 0.65, 95% CI 0.53-0.80, P0.0001). Carriers of DR8 were at higher risk for thoracic TB (OR 1.72, 95% CI 1.21-2.46, P = 0.003). For these antigens, we found no significant heterogeneity between samples or evidence of publication bias. The risk of thoracic TB tended to be higher in carriers of DR2 (OR 1.67, 95% CI 1.16-2.41, P = 0.006), but the results were not consistent between studies (P value for heterogeneity0.0001).Susceptibility to TB is modulated by class I and II HLA antigens. However, these results based on the serological determination of antigens require confirmation by DNA-based methods to precisely identify those alleles involved.

Pulmonary hypertension (PHT) represents one of the severest complications and is life-threatening for patients suffering from systemic sclerosis (SSc). In France, the modalities for screening and treating PHT related to SSc are not well codified and no consensus has been reached. We conducted a survey among physicians inscribed on the list of the French Research Group on Sclerosis (GRFS - Groupe de Recherche Francais sur la Sclerodermie) to gather information on the status of the management of PHT related to SSc.In 2002, we sent a questionnaire to 160 physicians, members of the GRFS, to assess the epidemiology and clinical profile of SSc patients as well as the modalities of screening and management of PHT in these patients.Eighty-eight physicians in 71 centres replied to the questionnaire. Each centre followed-up a mean of 33 SSc patients, with a global distribution of 53% limited and 47% diffused SSc. These physicians saw a mean of 5 new cases of SSc per year. The patients had been referred by town practitioners (53%) or from the hospital (47%). The mean number of SSc patients with PHT was of 5.1 per physician (1.5 new SSc + PHT patients per year). Almost all the centres (65/67) who replied systematically screened for PHT in SSc patients using Doppler echocardiography a mean of every 1.3 years. For the management of the patients exhibiting PHT, the majority (41/63) of centres collaborated with a specialized unit. Around one third of the centres treated these patients with calcium channel inhibitors (82%) and/or prostacyclin (90%). All the patients were followed-up by Doppler echocardiography. The majority of the physicians (72%) were interested in a research protocol on the subject and each could have included 4 patients, i.e., a total of 160.Pulmonary hypertension, a severe complication of SSc is screened for by the physicians of the GRFS using echocardiography with a frequency similar to Who guidelines (1.3 versus once/year).

Mooren's ulcer (MU) is a chronic peripheral corneal ulceration featuring conjunctival immunoglobulin deposits. It is considered as the result of a limbic immune process with hyperactivation of T and B lymphocytes. The etiology remains unknown. The response to topical steroid therapy and surgical procedures usually poor and the visual outcome can be devastating.Clinical follow-up of 3 patients who had rebel MU to conventional therapy, and were treated with 1g monthly intravenous cyclophosphamide.First patient was a 24-years-old man who had MU in his left eye. The response to surgical procedure and intravenous steroid treatment was poor and corneal perforation occurred. The affected cornea healed after 9 months of Cy treatment. The second patient was a 50-years-old man who had MU in his left eye, which did not improved with lamellar keratoplasty and topical steroid therapy. Corneal healing was obtained after 20 months of Cy treatment. The third patient was a 70-years-old man who presented with a furrowed MU in his right eye which healed with conjunctival resection and 4 months of Cy perfusion. No adverse effects of Cy was noted as opposed to Cy given orally.We report the effectiveness of 1g monthly intravenous cyclophosphamide (Cy) treatment in rebel MU. We suggest that immunosuppressive therapy using IV monthly Cy may be proposed in severe rebel MU.

The North American Symptomatic Carotid Endarterectomy Trial has confirmed the benefit of carotid endarterectomy in comparison to medical treatment in stroke prevention in symptomatic patients having a carotid stenosis of 70% or more. The Asymptomatic Carotid Atherosclerosis Study has concluded that the benefit of surgical treatment remains significant in asymptomatic patients with 60% (or more) stenosis of the ipsilateral internal carotid artery, when mortality rate remains inferior to 3%. In these two trials, angiography has been used to quantify the stenosis. Though this test is carrying some neurological and renal risks, replacing the angiography stenosis grading for a non or less invasive test, seems to be permissible.In our retroprospective study, the assessments of the carotid stenosis by several non-invasive tests findings were compared to the angiography results. Nineteen carotid arteries of fifteen patients, both symptomatic and asymptomatic, having a carotid stenosis at least 60% or more and being detected by the Doppler ultrasound were explored either by magnetic resonance angiography (MRA), spiral computed tomography angiography (SCTA) and angiography.The ultrasonography and angiography findings were well correlated (r=0,88; p0.002) according to the Spearman test. The assessments of the MRA were better correlated to the angiography than to the SCTA (respectively r=0.91, p0.0001 and r=0,68, p0.001). Using both ultrasonography and MRA as a confirmatory test, the rate of injustified carotid endarterectomy was 25%. And this rate rose up to 33% when the ultrasonography was used with the SCTA. It is noteworthy that negative predictive value of ARM was 100%. To reduce the mortality rate, several surgical teams managed the carotid stenosis without angiography.MRA could replace angiography, on condition that the rate of unjustified carotid endarterectomy lowers and becomes acceptable. Far reaching complementary studies are necessary to confirm the fiability of those non-invasive tests. In order to raise the benefit to carotid endarterectomy, the research studies should turn to the predictive score determination of a surgical international risk and towards the "High benefit" patients groups after endarterectomy.

Chronic fatigue remains a medical mystery and a therapeutic failure. The subgroup of chronic fatigue postinfectious fatigue (CPIF) is an interesting one since it is quite frequent in general practice.We studied sulbutiamine (Su), isobutyryl-thiamine disulfide in this context. We included 326 general-practice patients suffering from CPIF: they received randomly either Su, 400 mg daily (n = 106), or Su, 600 mg daily (n = 111), or placebo (n = 109) for 28 days in a double-blind, parallel-group study. 315 patients completed the study.The evaluation of fatigue, by multiple means including mainly MFI, a validated multidimensional fatigue scale, showed overall no significant difference between the groups. On the 7th day, however, women receiving Su, 600 mg had less fatigue (P0.01), but the figures were quite diverse and no persistent effect was noted at the 28th day.Thus, we showed for the first time that a high level general-practice study of fatigue is feasible using specific tools. Whether the effect observed after 1 week in women represents a true finding needs additional research. Further studies are in progress in order to characterize better the potential usefulness of Su in chronic fatigue.

The cytomegalovirus (CMV) is the only microbial agent implicated in three particular types of arterial disease: coronary disease of the transplanted heart, post-angioplasty restenosis and atherosclerosis. The object of this article is to analyse the recent data on the role of CMV in these pathologies with an exhaustive review of the literature. The available data is mainly epidemiological but the interpretation is difficult because of the multiplicity and imperfections of the diagnostic techniques of the infection. However, the results are quite concordant in favour of a real association. Different physiopathological mechanisms are proposed. In coronary disease of the transplanted heart, the lesions could be initiated by an inflammatory process. In post-angioplasty restenosis, the virus seems able to trigger cellular proliferation by inhibiting the mechanisms of apoptosis. Finally, in atherosclerosis, CMV infection seems to promote atherothrombotic processes and accelerate the progression of atherosclerotic plaques by activating inflammatory cells. Direct methods of detection of viral DNA show the presence of the virus within these lesions. There is, therefore, epidemiological, anatomo-pathological and physiopathological evidence in favour of a relationship between CMV infection and these three forms of arterial disease.

The larynx is a rare site of deposition for amyloidosis. Diagnosis may be delayed and evoked in patients with prolonged hoarseness. We have reported two cases of laryngeal amyloidosis.One man and one woman suffered from hoarseness during one and three years, respectively. Laryngoscopic examination showed diffuse infiltration of the larynx. Amyloidosis was confirmed by the characteristic Congo-red staining of laryngeal biopsies. The search for other localizations of amyloidosis was negative. No monoclonal plasma cell proliferation was detected. Both patients received endoscopic CO2 laser excision. In one case, chemotherapy was initially associated due to dystrophic plasma cells in bone marrow aspiration and then withdrawn because of clinical failure. With a 6-month follow-up, hoarseness remained stable.Laryngeal amyloidosis is an essentially localized disease revealed by hoarseness. Treatment is endoscopic by laser excision. In nearly half of the cases in the literature, it had to be repeated due to localized recurrent lesions. Long-term prognosis of localized laryngeal amyloidosis is better than systemic AL amyloidosis.