The Clinical Relevance of Antifibrillarin (anti-U3-RNP) Autoantibodies in Systemic Sclerosis.

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 antifibrillarin autoantibodies (AFA) in patients with SSc. The clinical features of 37 patients with SSc 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. Antifibrillarin 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. Comparing AFA+ and AFA- patients, AFA+ patients were significantly younger at disease onset (36.9 versus 42.9; P = 0.02), more frequently male (P = 0.02) and of Afro-Caribbean descent (65% versus 7.7%; P < 0.001). At diagnosis, the Rodnan skin score evaluating the cutaneous manifestations was higher (13.3 versus 8.7; P = 0.01) and myositis was also more common in the AFA+ group (31.4% versus 12.2%; P < 0.01). Patients with AFA+ were not associated with diffuse cutaneous SSc or with lung involvement and no difference in survival was observed. Antifibrillarin autoantibodies are associated with patients of Afro-Caribbean origin and can identify patients with SSc who are younger at disease onset and display a higher prevalence of myositis.

Serum CC chemokine ligand-18 predicts lung disease worsening in systemic sclerosis.

Elevated serum CC chemokine ligand (CCL)18 reflects lung fibrosis activity in systemic sclerosis (SSc) and could be an early marker of lung function worsening. Therefore, we sought to evaluate whether serum CCL18 levels at baseline could predict worsening of lung disease in SSc. In this prospective study, 83 SSc patients were analysed longitudinally over a 4-yr observation period for the risk of occurrence of combined deleterious events, defined as a 10% decrease from baseline of total lung capacity or forced vital capacity % predicted, or death, according to serum CCL18 at inclusion. Receiver operating characteristic (ROC) curve analysis was performed for prediction of events during the first year after inclusion. The best cut-off level of serum CCL18 for prediction of a combined event within the follow-up period was 187 ng · mL(-1), with 53% sensitivity and 96% specificity (area under the ROC curve 0.86; p < 0.001). After a mean ± SD follow-up of 33.7 ± 10.8 months, a higher rate of disease progression occurred in the group with serum CCL18 levels >187 ng · mL(-1). The adjusted hazard ratio was 5.36 (95% CI 2.44-11.75; p < 0.001). In summary, serum CCL18 is an accurate predictive biomarker for the identification of patients with a higher risk of subsequent scleroderma lung disease worsening.

NLRP1 influences the systemic sclerosis phenotype: a new clue for the contribution of innate immunity in systemic sclerosis-related fibrosing alveolitis pathogenesis.

BACKGROUND: Recent evidence has highlighted a potential role of interleukin 1ß (IL-1ß) in systemic sclerosis (SSc). NLRP1 provides a scaffold for the assembly of the inflammasome that promotes the processing and maturation of pro-IL-1ß. In addition, NLRP1 variants were found to confer susceptibility to autoimmune disorders. OBJECTIVE: /st> To study a possible association of the NLRP1 rs6502867, rs2670660 and rs8182352, rs12150220 and rs4790797 with SSc in the European Caucasian population. METHODS: NLRP1 single nucleotide polymorphisms were genotyped in 3227 individuals comprising a discovery set (870 SSc patients and 962 controls) and a replication set including individuals from Germany (532 SSc patients and 324 controls) and Italy (527 SSc patients and 301 controls), all individuals being of European Caucasian origin. RESULTS: Conditional analyses revealed a significant association for the NLRP1 rs8182352 variant with both anti-topoisomerase-positive and SSc-related fibrosing alveolitis (FA) subsets under an additive model: p=0.0042, OR 1.23 (95% CI 1.07 to 1.41) and p=0.0065 OR 1.19 (95% CI 1.05 to 1.36), respectively. Logistic regression analysis showed an additive effect of IRF5 rs2004640, STAT4 rs7574865 and NLRP1 rs8182352 risk alleles on SSc-related FA. CONCLUSIONS: Our results establish NLRP1 as a new genetic susceptibility factor for SSc-related pulmonary fibrosis and anti-topoisomerase-positive SSc phenotypes. This provides new insights into the pathogenesis of SSc, underlining the potential role of innate immunity in particular in the FA-positive SSc subphenotype, which represents a severe subset of the disease.

Association of a KCNA5 gene polymorphism with systemic sclerosis-associated pulmonary arterial hypertension in the European Caucasian population.

OBJECTIVE: Pulmonary arterial hypertension (PAH) has emerged as a leading cause of death in systemic sclerosis (SSc). The genetic basis of PAH has been unraveled in recent years, with a major role played by transforming growth factor ß receptors; however, some other candidate genes have also been advocated, including potassium voltage-gated channel, shaker-related subfamily, member 5 (KCNA5). We undertook this study to determine whether KCNA5 polymorphisms confer susceptibility to SSc and its vascular phenotype, including PAH. METHODS: Four KCNA5 single-nucleotide polymorphisms (SNPs), rs10744676, rs1860420, rs3741930, and rs2284136, were genotyped in a discovery set of 638 SSc patients and 469 controls. In addition, rs10744676 was genotyped in an independent replication sample (938 SSc patients and 564 controls) and in a cohort of 168 patients with different PAH subtypes. RESULTS: The KCNA5 rs10744676 variant was found to be associated with SSc in the discovery sample, with an odds ratio (OR) of 0.62 (95% confidence interval [95% CI] 0.48-0.79, adjusted P = 0.0003) in comparison with controls (C allele frequency 11.4% versus 17.2%). When subphenotypes were investigated, an association was found solely for PAH associated with SSc (OR 0.31 [95% CI 0.13-0.71], adjusted P = 0.04). The other KCNA5 SNPs tested were not associated with any SSc subset. The above association with PAH associated with SSc was replicated in the second set. In the combined population, rs10744676 was strongly associated with PAH associated with SSc in comparison with controls (OR 0.36 [95% CI 0.21-0.63], P = 0.0002). In the independent cohort of patients with PAH, after investigating PAH subtypes, only rs10744676 showed an association with PAH associated with SSc. CONCLUSION: Our results provide the first evidence for an association between the KCNA5 rs10744676 variant and PAH associated with SSc.

Long-term outcome of patients with polymyositis/ dermatomyositis and anti-PM-Scl antibody.

BACKGROUND: To date, no series has analysed long-term outcome in patients with polymyositis/dermatomyositis (PM/DM) with anti-PM-Scl antibody. OBJECTIVES: The aims of the present study were: (i) to assess clinical features and long-term outcome, including organ complications, functional course and mortality rate, in patients with isolated PM/DM with anti-PM-Scl antibody; and (ii) to evaluate prevalence, characteristics and long-term outcome of interstitial lung disease (ILD) in patients with isolated PM/DM with anti-PM-Scl antibody. METHODS: The medical records of 20 consecutive patients with isolated PM/DM with anti-PM-Scl antibody were reviewed. RESULTS: Two patients (10%) achieved remission of PM/DM, whereas 14 (70%) improved and four (20%) had a worsened clinical status. Short-term recurrences (during tapering of therapy) occurred in nine patients and long-term recurrences (after discontinuation of therapy) in three patients. Moreover, patients with PM/DM with anti-PM-Scl antibody exhibited severe complications, as follows: oesophageal involvement (n = 4) requiring enteral feeding in three cases, ventilatory insufficiency (n = 3) requiring mechanical ventilation in two cases; three other patients had cancer. Interestingly, patients with PM/DM with anti-PM-Scl antibody often presented symptoms that are usually found in antisynthetase syndrome, i.e. hyperkeratotic rhagadiform hand symptoms (n = 2; 10%), Raynaud's phenomenon (n = 8; 40%), arthralgia/arthritis (n = 7; 35%) and ILD (n = 12; 60%). In our cohort, the associated ILD often required combined therapy of steroids and immunosuppressive agents. CONCLUSIONS: Our series suggests that the presence of anti-PM-Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung and oesophageal involvement; in addition, anti-PM-Scl antibody may coexist with malignancy in patients with PM/DM. Furthermore, anti-PM-Scl antibody-positive patients with PM/DM often exhibit 'mechanic's hands', Raynaud's phenomenon and joint involvement. Our latter findings raise the possibility that the immunogenetic background influences the autoantibody status of these patients; HLA-DR3 has, in fact, been found in association with antisynthetase syndrome antibodies and with anti-PM-Scl antibodies.

Association of hypoxia-inducible factor 1A (HIF1A) gene polymorphisms with systemic sclerosis in a French European Caucasian population.

OBJECTIVE: Systemic sclerosis (SSc) is a connective tissue disease characterized by generalized microangiopathy leading to chronic hypoxia. The aim of this study was to determine whether polymorphisms of the hypoxia-inducible factor 1A gene (HIF1A) affects susceptibility to SSc in a large French European Caucasian population. METHODS: A case-control study was performed in 659 SSc patients and 511 healthy matched controls. Three tag single nucleotide polymorphisms (SNPs) of the HIF1A gene (rs12434438 A/G, rs1957757 C/T, and rs11549465 C/T) were genotyped allowing whole gene coverage according to HapMap data. RESULTS: The frequency of genotypes carrying at least one G allele (A/G and/or GG) of the rs12434438 SNP was significantly higher in SSc patients than in controls [p(corr) = 0.018, odds ratio (OR) 1.44, 95% confidence interval (CI) 1.08-1.91]. Regarding SSc subgroup analyses, the heterozygous genotype A/G was associated with SSc (p(corr) = 0.012, OR 1.47, 95% CI 1.13-1.9), with the limited cutaneous form of SSc (p(corr) = 0.04, OR 1.43, 95% CI 1.08-1.91), and with positive anti-centromere antibodies (ACA; p(corr) = 0.016, OR 1.61, 95% CI 1.16-2.23). No association was detected for the remaining two HIF1A SNPs tested. Haplotype analyses did not detect any association with SSc. CONCLUSIONS: We observed an association between the HIF1A gene and SSc in a European Caucasian population, supporting a role for HIF1 in the pathophysiology of SSc.

Diagnostic value of exhaled nitric oxide to detect interstitial lung disease in systemic sclerosis.

BACKGROUND AND AIM: 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. METHODS: 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. RESULTS: 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 value > 95%) when CA(NO) > or = 10.8 ppb, and ruled out C(ANO) values < or = 3.8 ppb (negative predictive value > 95%). CONCLUSION: CA(NO) could be a valid non-invasive biological marker of ILD in SSc, and be of use in clinical practice.

[DRESS syndrome caused by phenylbutazone]. / DRESS syndrome à la phénylbutazone.

The drug rash with hypereosinophilia and systemic symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity syndrome. We report a 57-year-old woman suffering from a DRESS syndrome 15 days after phenylbutazone exposure. She had a skin eruption, liver involvement and hypereosinophilia. She fully recovered after drug withdrawal.

[Short-term effect of dietary-sodium intake on arterial blood pressure of patients treated with systemic corticosteroids: a prospective, randomised, crossover study]. / Effet à court terme des apports sodés sur la pression artérielle des patients recevant une corticothérapie systémique: étude prospective, randomisée, croisée.

INTRODUCTION: It is unknown if the level of dietary-sodium intake influences blood pressure in patients receiving systemic corticosteroids. METHODS: 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 versus >6 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. RESULTS: Between June 2006 and June 2008, 49 patients were randomized, 24 in group 1 (first period regimen=salt<3g/day; women: 63%; mean age: 56+/-21 years; baseline prednisone dosage: 54+/-19 mg/day) and 25 in group 2 (first period regimen=salt>6g/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). CONCLUSION: At short-term, sodium intake does not seem to influence blood pressure variations in patients starting systemic corticosteroids therapy.

[Internal medicine physicians' perception of frequency and impact of corticosteroid-induced adverse events]. / Perception par les médecins internistes de la fréquence et de la gêne induite par les effets indésirables d'une corticothérapie systémique prolongée.

INTRODUCTION: Whereas internal medicine physicians frequently prescribe systemic corticosteroids, it is unknown if they assess adequately the frequency and the discomfort caused by corticosteroid-induced adverse events. METHODS: Using an e-mail questionnaire sent to the 813 internal medicine physicians, members of the French National Society of Internal Medicine, we assessed their perception of the frequency and the discomfort induced by the adverse events of long-term (that is, over or at three months) corticosteroid therapy. At the same time, 121 corticosteroid-treated patients, consulting in a department of internal medicine completed an anonymous questionnaire about the frequency and the discomfort caused by the adverse events of their therapy. RESULTS: Three hundred and thirty-six out of 813 internal medicine physicians answered to the questionnaire (response rate: 41%) and 115 of the 121 questionnaires distributed to patients were exploitable. The physicians were predominantly male (71%) working mainly in tertiary centers (53%). The mean length of corticosteroids therapy for patients was 44+/-38 months and the mean daily dosage was 15+/-14mg. Lipodystrophy, trophic skin disorders, neuropsychiatric disorders and insomnia were frequent and reported by more than half of patients. The frequency of neuropsychiatric and skin disorders and of lipodystrophy estimated by practitioners was markedly lower than the frequency reported by patients. If morphological changes (weight-gain and lipodystrophy) were cited by practitioners as the most discomforting adverse event, in agreement with patients' opinion, physicians underestimated the discomfort caused by neuropsychiatric disorders and insomnia. CONCLUSION: Frequency and discomfort caused by corticosteroid-induced neuropsychiatric disorders are underestimated by internal medicine physicians.

[Liver infarcts with early HELLP syndrome and probable catastrophic antiphospholipid syndrome]. / Infarctus hépatiques associés à un syndrome HELLP précoce et un probable syndrome catastrophique des antiphospholipides.

INTRODUCTION: Among microangiopathic disorders of pregnancy, catastrophic antiphospholipid syndrome (CAPS) is a maternal and fetal life-threatening disorder. Hepatic involvement of this multi-systemic disorder can be confused with HELLP syndrome, occurring usually later in the course of pregnancy. CASE REPORT: We report a case of probable CAPS with hepatic disease in a pregnant woman at 13 week's gestation, with antiphospholipid syndrome and biological features of HELLP syndrome. Unspecific hepatic imaging, well-described in our case allowed undelayed therapy. CONCLUSION: CAPS and HELLP syndrome, both severe microangiopathic disorders, may be associated. Nosological distinction does not modify treatment strategy, which is a maternal and foetal emergency, but their overlapping requires aggressive and early management.

[Primary chronic autoimmune neutropenia successfully treated with sirolimus]. / Neutropénie auto-immune primaire avec autoanticorps antineutrophiles traitée avec succès par sirolimus.

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.

[Schwannoma of the duodenojejunal angle]. / Schwannome kystique de l'angle duodénojéjunal.

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.

[Münchausen syndrome with forgery on biologic results. A case report]. / Pseudoleucémie par falsification d'examens biologiques: genèse d'un syndrome de Münchausen.

Münchausen syndrome is a disorder defined by the following: acute factitious symptoms leading to inappropriate investigation and therapy, a restless journey from hospital to hospital and autobiographical falsification. We report here a 20-year-old woman who presented at our hospital consultation of internal medicine with laboratory-test results suggesting the diagnosis of leukemia. A new complete blood cells count and a medullogram by sternal puncture did not show any abnormality. Comparative examination of laboratory-test sheets lead to the diagnosis of Münchausen syndrome as some results had been falsified. With unlimited access to information through internet and word or image processing softwares, laboratory results have become easy to falsify nowadays, particularly for patients with Münchausen syndrome, who may then be quite difficult to diagnose accurately in the context of medical consultation.

[Long-term systemic corticosteroid-therapy associated measures: description of the French internal medicine physicians' practices]. / Mesures adjuvantes à une corticothérapie systémique prolongée : description des pratiques des médecins internistes français.

INTRODUCTION: 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. METHODS: 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). RESULTS: 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. CONCLUSION: 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.

ANCA-related crescentic glomerulonephritis in systemic sclerosis: revisiting the "normotensive scleroderma renal crisis".

The scleroderma renal crisis is characterized by acute onset of severe hypertension and by rapidly progressive hyperreninemic renal failure. There is, however, a very limited subset of patients with rapidly progressive renal failure who remain normotensive and develop ANCA-positive crescentic glomerulonephritis. We report a case of normotensive acute renal failure secondary to anti-MPO antibody-associated crescentic glomerulonephritis in a patient with diffuse systemic sclerosis. She was referred to our department with normal blood pressure and no extrarenal clinical manifestation ofvasculitis. She presented with rapidly progressive renal failure, microscopic hematuria and minimal proteinuria. P-ANCA were positive by immunofluorescence, with ELISA-confirmed specificity for myeloperoxidase. Renal biopsy revealed typical features of pauciimmune glomerulonephritis with crescent formation and fibrinoid necrosis. The patient was initially treated with i.v. cyclophosphamide only. Because of ongoing deteriorating renal function, additional treatment with intravenous pulses of methylprednisolone followed by oral prednisone was started and allowed renal function improvement. After 9 months, serum creatinine had almost returned to normal level with minimal proteinuria, no hematuria and negative ANCA testing. Control kidney biopsy only revealed scar lesions. The association of ANCA-positive crescentic glomerulonephritis and systemic sclerosis is a very rare event. Treatment with intravenous cyclophosphamide and corticosteroids allows rapid and long-term improvement of renal function. The onset of typical scleroderma renal crisis triggered by high-dose corticosteroids is unlikely but requires a close follow-up of patients with overlapping systemic sclerosis. Diagnosis and treatment are discussed and previously published cases are reviewed.

[Systemic corticosteroid therapy: patients' adherence to dietary advice and relationship between food intake and corticosteroid-induced lipodystrophy]. / Corticothérapie systémique et alimentation: suivi des recommandations diététiques et relation entre apports alimentaires et apparition d'une lipodystrophie.

PURPOSE: No data is available about: 1) the adherence of corticosteroid-treated patients to dietary advice provided by physicians; 2) the relationship between food intake and the corticosteroid-induced lipodystrophy (CIL). METHODS: We conducted a cohort study in 2 French tertiary centers between June 2003 and May 2005 and enrolled all consecutive patients starting long-term systemic corticosteroid therapy. They received individual dietary advice from a qualified dietetician and were asked to record on a standardized questionnaire everything they ate during one week of the first and third months of treatment, including details of each meal. Each questionnaire was analysed by two qualified dieteticians for daily calorie, carbohydrate, fat, protein and sodium intake. Moreover, 3 investigators assessed the development of CIL from standardized patients' photographs. The relationship between food intake and CIL was investigated by a multiple logistic regression model. RESULTS: Eighty-eight patients were included and 80 were monitored until at least month 3 (women: 76%, mean age: 59.1+/-18.7 years). Most patients (65%) had giant-cell arteritis or connective tissue disease. The mean initial dosage of prednisone was 54+/-17 mg/day and the mean M3 dosage was 31+/-15 mg/day. Most patients were adherent to dietary advice during the first 3 months of therapy except for protidic ration which was below expected value. Sodium restriction was more strictly followed by women than by men. Multivariate analysis showed independent relationship between CIL and higher calorie intake (>30 kcal/kg/day). No relationship was evidenced between carbohydrate, protein, fat or sodium intake and the risk of CIL. CONCLUSION: During the first 3 months of therapy, corticosteroid-treated patients are adherent to dietary advice. A calorie-controlled alimentation could be beneficial to limit the risk of CIL.

[In-hospital mortality and stay duration of internal medicine patients: prognosis value of biochemical markers commonly performed on admission]. / Mortalité hospitalière et durée de séjour des patients non programmés en médecine interne: valeur pronostique de paramètres biochimiques usuels à l'admission.

PURPOSE: Little is known about prognosis values of biochemical markers in internal medicine patients. We have examined retrospectively the relationship between inhospital mortality or stay duration and several biochemical markers commonly performed on admission in internal medicine patients. METHODS: Among all stays unplanned in our department during the year 2004, we collected data about 8 blood biochemical markers (sodium, potassium, chloride, bicarbonate, anion gap, urea nitrogen, creatinin, proteins), performed between the day before and the day after admission. Mixed Cox regression models computed hazard ratios for mortality associated with biochemical markers concentration. The relationship between biochemical markers concentration and duration stay was investigated in mixed linear regression models. RESULTS: In 2004 our department totalized 1199 unplanned stays by 1054 distinct patients (age: 69.9+/-19.2 y, women: 59.2%), among which 59 deceased during stay. Biochemical markers were available for 977 (81.5%) stays (stay duration: 17.5+/-16.0 days). Inhospital mortality was significantly associated with plasma concentration on admission of potassium, proteins, anion gap and with urea nitrogen/creatinin ratio. Among survivors, duration stay was significantly associated with plasma concentration on admission of sodium, chlore, and anion gap. CONCLUSION: Biochemical markers performed on admission need particular attention as they provide immediate information about short term prognosis of internal medicine patients.

[Natural history of corticosteroid-induced lipodystrophy: a prospective follow-up of 37 patients]. / Histoire naturelle de la lipodystrophie cervicofaciale cortico-induite: suivi prospectif d'une cohorte de 37 patients.

PURPOSE: 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. METHODS: 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). RESULTS: 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]). CONCLUSION: Lipodystrophy is frequently observed in corticosteroid-treated old patients. It appears precociously after the initiation of therapy and is usually reversible.