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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

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.

To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM.The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed.Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich-like pattern, initial diffusing capacity of carbon monoxide45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti-Jo-1 antibody, and characteristic microangiopathy were significantly more frequent.Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti-Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti-Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.

Survival in patients with giant cell arteritis (GCA) has generally been found to be similar to that of the general population. The aim of our study was to assess outcome and survival of different subgroups of patients with GCA in relation to clinical, biological data or treatment modalities.From 1977 and 1995, 176 patients were treated in the Department of Internal Medicine for GCA. The patient, family or local practitioner were contacted prior to the study (July-October 1995). Treatment modalities and follow-up were obtained for 133 patients. All patients (except 11) had 3 or more 1990 ACR classification criteria for GCA. The 11 patients with 2 criteria had a positive temporal biopsy and were included in the study.Relapse during corticosteroid tapering treatment was observed in 83 patients (62.4%) with a mean 1.57 relapses per patient. No correlation was found in age, sex, initial dose or type of steroid used (i.e. prednisone or prednisolone). Only a slight correlation in the initial erythrocyte sedimentation rate (ESR) was observed (p0.01, r = 0.23). In 56 patients free of treatment (mean treatment duration: 40 months), 27 (48%) developed a relapse of the disease 1 to 25 months later. No correlation was found in age, sex, initial dose of steroid, number of relapses during treatment, or initial ESR. Survival analysis was performed using the Kaplan-Meier and Mantel-Menszel methods for comparison of groups. At the time of the study, 41 patients had died (30.7%). A significant reduction of survival was found with the presence of permanent visual loss vs absence (p = 0.04), in patients who required more than 10 mg/d of glucocorticoid (p0.001) at 6 months treatment and in patients treated with prednisone (vs prednisolone) (p0.01). However, these factors were not independently associated with survival in the multivariate analysis.Relapse was observed in 62.4% of the patients during corticosteroid tapering (correlated with initial ESR). A relapse of the disease was also observed in 48% of patients 1 to 25 months after the end of the treatment and was associated with prednisolone use. Long term survival was better in patients with no initial ocular manifestations, in patients who took less than 10 mg/day of corticosteroids at 6 months of the treatment and in patients treated with prednisolone.

Endostatin is an angiogenesis inhibitor derived from type XVIII collagen. The aim of this study was to determine the concentrations of circulating endostatin in patients with systemic sclerosis (SSc), and to assess the relationship between these concentrations, extension of tissular sclerosis, and presence of cutaneous scars or ulcers.The study involved 50 patients with SSc and 30 healthy subjects. Cutaneous extension of sclerosis was graded according to Barnett's classification system: 33 patients had grade I SSc and 17 patients had grades II or III SSc. The results of pulmonary function tests were abnormal in 31 of 50 patients, 8 of whom also had abnormalities on chest radiograms. Cutaneous scars or ulcers were found in 22 of 50 patients. Endostatin concentrations were determined using a competitive enzyme immunoassay method.The mean circulating endostatin concentration was significantly higher in the SSc group than in the healthy subjects group (mean +/- SD 53.2 +/- 22.4 ng/ml versus 9.9 +/- 9.7 ng/ml; P10(-4)), in patients with grade II or grade III SSc than in patients with grade I SSc (63.2 +/- 20.2 ng/ml versus 45.1 +/- 15.6 ng/ml; P10(-2)), in patients with abnormal findings on chest radiograms than in patients with normal findings on chest radiograms (67.6 +/- 22.4 ng/ml versus 50.4 +/- 21.6 ng/ml; P0.05), and in patients with cutaneous scars or ulcers than in patients without these manifestations (60.9 +/- 25.9 ng/ml versus 47.2 +/- 13.3 ng/ml; P10(-2)).Circulating endostatin concentrations are significantly increased in patients with SSc. Production of endostatin may result from tissular sclerosis and could contribute to the development of ischemic manifestations.

To assess endothelial cell activation in patients with systemic sclerosis (SSc).Concomitant study of salivary gland biopsy tissues and sera for expression of E-selectin and its potent activator tumor necrosis factor alpha (TNF alpha), using immunostaining and enzyme-linked immunosorbent essay.E-selectin was overexpressed in SSc patients, but not in controls. TNF alpha was detected in mast cells.Mast cell-derived TNF alpha may contribute to endothelial cell activation in SSc.

The probability that patients with collagen vascular diseases (CVD) will develop fibrosis is unpredictable. Since changes in bronchoalveolar lavage (BAL) cell data can be observed in CVD patients without evidence of lung involvement, we investigated whether the study of soluble components in BAL could help to distinguish CVD patients with lung involvement (n = 15) from those without pulmonary disease (n = 37). Our results demonstrate that the alveolitis observed in patients with overt lung involvement is associated with an increase of BAL alpha 2-macroglobulin (alpha 2-MA). In contrast, the BAL alpha 2-MA levels were found to be normal in CVD patients without evidence of pulmonary disease as well as in CVD patients with overt lung involvement treated with steroids. This was observed even in the presence of high neutrophil or lymphocyte counts in BAL. In conclusion, when neutrophils or lymphocytes accumulate in the lungs of CVD patients without evidence of lung damage, in the majority of patients this cell accumulation is not associated with an increase of BAL soluble components.

Polyclonal B cell activity in systemic lupus erythematosus (SLE) may be under T cell control. The use of nitrocellulose immunoblots for the analysis of recognition by peripheral blood lymphocytes of nucleoplasmic proteins in SLE patients led to the characterization of significant proliferative responses to 68K (U1 RNP); SS-B; B-B' and D (Sm) antigen in 15 of 20 patients. Variations of proliferative response were parallel to disease activity over a follow-up period of greater than or equal to 6 months, conferring some prognostic value to the assay of lymphocyte response to nucleoplasmic antigens. The pattern of reactivity differs from the corresponding serum antibody profile, and purified T cell suspensions (greater than 95% pure) were shown to proliferate in response to soluble nucleoplasmic antigens, indicating that T and B cell repertoires against nucleoplasmic proteins may differ. This suggests that activated helper T cells contribute to the fine modulation of B cell reactivity to subcellular particles to determine the particular antibody profile of the patients.

To the Editor: Disseminated nocardiosis due to Nocardia farcinica has mostly been reported in immunocompromised patients with underlying malignancies, HIV infection, solid-organ transplants or in those receiving long-term corticosteroid therapy (1). We present a second case of disseminated nosocomial nocardiosis following iliac artery stent infection occurring in an immunocompetent patient. A previously healthy 57-year-old man presented with a two-week history of generalized weakness, fever, petechial rash from the right knee down, and right knee and ankle joint pain. Empirical antibiotic therapy was initiated with intravenous (IV) vancomycin (2 g daily) and rifampin (1200 mg daily), without effect. One month before symptom appearance, he had undergone an angioplasty with stenting for a symptomatic right iliac stenosis. Multiple blood cultures, knee synovial fluid cultures and cultures of petechial lesion skin biopsies were all negative for pyogenic organisms, mycobacteria and fungi. There was no history, clinical examination or standard laboratory findings suggestive of an immunocompromised state; HIV testing was negative. At admission, the laboratory findings included a white blood cell count of 10.5×109/L and an elevated C-reactive protein level of 95 mg/L. Antibiotic therapy was discontinued to perform new investigations. At the same time, the patient underwent a second angioplasty for a new stenosis of the right iliac artery. One week later, the patient’s clinical state deteriorated. Physical examination revealed a painful, tender, palpable, right ilioinguinal mass. A computed tomography (CT) scan revealed a fissured pseudoaneurysm formation (6.2 cm × 4 cm in diameter) around the stent (Figure 1). The dilated iliac stented segment was immediately excised and the patient underwent femoral bypass grafting. Abdominal and lower body CT scans showed multiple abscesses of the right lower limb, right knee arthritis, quadricipital myofasciitis and right femoral osteolytic lesions. Figure 1) Computed tomography scan showing a fissural pseudoaneurysm formation around the stent (arrow) Right knee synovial fluid culture yielded Gram-positive branching rods, and the patient was treated simultaneously with IV imipenem (1 g every 8 h) and IV amikacin (1250 mg daily), but he remained septic with fever (39°C), and had a white blood cell count of 28×109/L and a further elevated C-reactive protein level. 16S ribosomal sequencing (reverse transcription, real-time polymerase chain reaction using SYBR green fluorescent dye) of the patient’s synovial fluid was performed, and N farcinica was identified four days later. This bacteria was also isolated in large amounts in excised pseudoaneurysm and stent cultures. One week later, the isolate was found to be resistant in vitro to ticarcillin, cephalosporins, imipenem, trimethoprim-sulfamethoxazole (TMP-SMZ), vancomycin, rifampin, erythromycin and gentamicin; moderately susceptible to amoxicillin and ciprofloxacin; and susceptible to amikacin, minocycline and linezolid by the National Reference Laboratory (Institut Pasteur, France) standards. We initiated IV amoxicillin (12 g daily), ciprofloxacin (1200 mg daily) and tigecycline (100 mg daily) in combination with TMP-SMZ (TMP 240 mg and SMZ 1200 mg daily). The patient improved clinically while on this treatment. Following six months of this regimen, a repeat CT scan showed an improvement, which allowed a switch to oral TMP-SMX (TMP 240 mg and SMZ 1200 mg daily), ciprofloxacin (1500 mg daily) and linezolid for a total of 12 months. No evidence of recurrent infection was noted in the two years following the discontinuation of antibiotic therapy. Femoral bypass grafting is scheduled in the near future. Nocardia species are ubiquitous, soilborne, aerobic Actinomyces. The main route of acquisition is usually through direct inhalation of contaminated particles from soil or water, or by direct inoculation through the skin (1). Nosocomial outbreaks of nocardiosis have been reported most frequently for immunocompromised patients in heart or liver transplantation units (2), with possible transmission via health care workers. Our observation suggests nosocomial intra- or perioperative contamination of the initial stent. Saubolle and Sussland (1) estimated that less than 10% of patients with disseminated nocardiosis have no identified underlying predisposing factors (1). Ours is the second reported case of N farcinica infection of a vascular prosthesis in an immunocompetent patient (3). Compared with other Nocardia asteroides complex organisms, N farcinica infections manifest themselves not only as pulmonary or systemic disease, but also as severe postoperative wound infections due to their virulence, affinity to medical material and resistance to antibiotics (4). The described case was also rather atypical in implemented therapy. Combination therapy with a sulpha-containing agent or imipenem in combination with amikacin has been recommended for disseminated nocardiosis (3). However, our strain was subsequently found to be resistant to both antibiotics, by which time the patient was severely ill with severe sepsis and osteoarticular abscesses, leading us to consider linezolid and tigecycline therapy as alternatives. We decided to continue TMP-SMZ because of reports (4,5) of the clinical success of TMP-SMZ therapy despite its in vitro resistance. Treatment options were further complicated because the infection occurred on a vascular prosthesis. The necessary duration of nocardiosis therapy is uncertain, but it has often been reported to have continued for many months after clinical cure because of high relapse rates (1). The number of patients suffering from nocardiosis is constantly rising worldwide. Our case highlights the fact that Nocardia infection can occur in an immunocompetent patient, particularly in case of persisting surgical site infection that fail to respond to conventional antimicrobial therapy. Molecular methods could improve diagnosis and the chances of survival.