998 research outputs found
Might Nailfold Capillaroscopy Be a "Proxy" for Lung Involvement in Connective Tissue Diseases?
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OP0035 Preliminary analysis of nailfold capillaroscopy in the very early diagnosis of systemic sclerosis (VEDOSS): the capi-vedoss experience
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Influence of diluent alkyl substitution on the extraction of Am(III) and Eu(III) by a 6,6’-bis(1,2,4-triazin-3-yl)-2,2’-bipyridine ligand dissolved in alkylated cyclohexanone Diluents
No full textSeveral alkylated cyclohexanones were investigated as potential diluents for the selective extraction of Am(III) from Eu(III) from nitric acid solutions by the CyMe4-BTBP ligand. No significant extraction of either of the metal ions was observed for these diluents themselves. In the extractions from 1 M HNO3, 3-methylcyclohexanone and 4-methylcyclohexanone gave comparable results to cyclohexanone whereas in the extractions from 4 M HNO3, 2-methylcyclohexanone, 3-methylcyclohexanone and 4-methylcyclohexanone all gave superior results. For the monomethylated diluents, DAm and SFAm/Eu decreased in the order of alkyl substitution 2 > 4 ~ 3. However, alkyl substitution of cyclohexanone significantly slows down the extraction kinetics compared to cyclohexanone, and the position of alkyl substitution was found to play an important role in the solvents properties. 3-Methylcyclohexanone was identified as the most promising of the diluent
Distinct expression of adhesion molecules on skin fibroblasts from patients with diffuse and limited systemic sclerosis: a pilot study
No full textAbstract
OBJECTIVE:
. Systemic sclerosis (SSc) is characterized by excessive production of collagen and other components of the extracellular matrix (ECM) by fibroblasts. The ECM receptors, integrins and CD44 (hyaluronan receptor), play a key role in the homeostasis of connective tissue and may also have a role in the pathogenesis of fibrosis. We investigated the expression of integrins and CD44 on skin fibroblasts from patients with limited and diffuse SSc.
METHODS:
We studied 13 patients with SSc, 8 with limited SSc, and 5 with diffuse SSc, and 8 control subjects. Fibroblasts were isolated and cultured from biopsies taken from the lesional skin of the second finger of the left hand. Cell-surface expression of beta1, beta3, alpha1-alpha6, alphav integrins, and CD44 was evaluated by immunofluorescence and flow cytometry analysis.
RESULTS:
Fibroblasts from limited SSc showed significantly decreased expression of alpha2, alpha3, alpha4 integrins, while diffuse SSc fibroblasts had significantly reduced expression of alpha5, alphav integrins, and CD44. Diffuse SSc also had significantly increased expression of alpha6 integrin on fibroblasts. In controls, the expression of alpha4 and alpha5 correlated positively, while in limited and diffuse SSc it did not.
CONCLUSION:
This is the first study evaluating separately the expression of adhesion molecules on skin fibroblasts from limited and diffuse subsets of SSc. We detected a distinct pattern of expression with decrease of collagen and fibronectin receptors in limited SSc, and downregulation of fibronectin and hyaluronan receptors in diffuse SSc. These results suggest that changes of fibroblasts/ECM interactions and mechanisms underlying the pathogenesis of fibrosis in SSc may differ in the single subset of the disease
The emerging application of semi-quantitative and quantitative capillaroscopy in systemic sclerosis
No full textSystemic sclerosis (SSc) is a connective tissue disease with high morbidity and mortality, characterized by autoimmunity, obliterative vasculopathy involving mainly the microvasculature, and fibrosis. SSc-specific nailfold capillaroscopic changes have been defined, and nailfold capillaroscopy (NFC) is now unequivocally accepted to be a cornerstone for the early diagnosis of SSc. However, the use of NFC in patients already diagnosed with SSc is still not standardized. Several studies have shown that NFC abnormalities correlate with disease activity and severity and are predictive for disease worsening, such as occurrence of new digital ulcers. More importantly, successful treatment has been shown to diminish NFC abnormalities in severe SSc cases. These findings support the importance of NFC in monitoring patients with SSc and even its role as an outcome measure in SSc clinical trials. It is a matter of debate if Semi-quantitative and Quantitative NFC would be a more sensitive tool than qualitative NFC for meeting these objectives. This review is presenting the emerging application of Semi-quantitative and Quantitative NFC in SSc and its potential benefits
Effects and safety of rituximab in systemic sclerosis: An analysis from the European Scleroderma Trial and Research (EUSTAR) group
No full textObjectives: To assess the effects of Rituximab (RTX) on skin and lung fibrosis in patients with systemic sclerosis (SSc) belonging to the European Scleroderma Trial and Research (EUSTAR) cohort and using a nested case-control design. Methods: Inclusion criteria were fulfilment of American College of Rheumatology classification criteria for SSc, treatment with RTX and availability of follow-up data. RTX-treated patients were matched with control patients from the EUSTAR database not treated with RTX. Matching parameters for skin/lung fibrosis were the modified Rodnan Skin Score (mRSS), forced vital capacity (FVC), follow-up duration, scleroderma subtype, disease duration and immunosuppressive co-treatment. The primary analysis was mRSS change from baseline to follow-up in the RTX group compared with the control group. Secondary analyses included change of FVC and safety measures. Results: 63 patients treated with RTX were included in the analysis. The case-control analysis in patients with severe diffuse SSc showed that mRSS changes were larger in the RTX group versus matched controls (N=25; -24.0±5.2% vs -7.7±4.3%; p=0.03). Moreover, in RTX-treated patients, the mean mRSS was significantly reduced at follow-up compared with baseline (26.6±1.4 vs 20.3±1.8; p=0.0001). In addition, in patients with interstitial lung disease, RTX prevented significantly the further decline of FVC compared with matched controls (N=9; 0.4±4.4% vs -7.7±3.6%; p=0.02). Safety measures showed a good profile consistent with previous studies in autoimmune rheumatic diseases. Conclusions: The comparison of RTX treated versus untreated matched-control SSc patients from the EUSTAR cohort demonstrated improvement of skin fibrosis and prevention of worsening lung fibrosis, supporting the therapeutic concept of B cell inhibition in SSc. © 2014 BMJ Publishing Group Ltd & European League Against Rheumatism
Evidenzbasierte Therapie des Raynaud-Syndroms
No full textDas Raynaud-Syndrom ist mit einer Prävalenz von 3-5% ein häufiges klinisches Problem. Dennoch ist die Wirkung der meisten Therapiemöglichkeiten nur unzureichend durch kontrollierte Studien belegt. Zu den Therapien mit höherem Evidenzgrad gehört der Kalziumantagonist Nifedipin, für den in Metaanalysen sowohl bei primärem als auch bei sekundärem Raynaud-Syndrom eine verbesserte periphere Durchblutung sowie eine Abnahme der Frequenz und des Schweregrades der Raynaud-Attacken nachgewiesen werden konnte. Ähnliches gilt für intravenös appliziertes Iloprost in der Therapie des sekundären Raynaud-Syndroms bei systemischer Sklerose. Intravenös verabreichtes Iloprost verbessert darüber hinaus das Abheilen von Fingerkuppenulzera bei Patienten mit systemischer Sklerose. Vielversprechende Therapieansätze stellen Angiotensin-II-Rezeptor-1-Antagonisten (Losartan), die Kalziumantagonisten Felodipin und Amlodipin, Serotonin-Reuptake-Hemmer (Fluoxetin) und Phosphodiesterase-V-Hemmer (Sildenafil, Vardenafil) dar, die sich in kontrollierten Einzelstudien als wirksam erwiesen haben. Jedoch fehlen Erfahrungen mit größeren Patientenzahlen und längeren Anwendungszeiten, um diese Therapiemöglichkeiten abschließend zu beurteile
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