No abstract

Pulmonary hypertension is not infrequent in TA patients and all the potential causes of PH should be carefully evaluated. Patients with severe or treatment-resistant disease are prone to have PH. PAH-specific agents may be effective in patients with group 4 PH.

There is a need for better definition of polyarteritis nodosa (PAN) subphenotypes and the influence of ethnicity and geography. This study is aimed to study the demographic and clinical features of PAN cohorts from the UK and Turkey (TR) and to compare and contrast disease characteristics. A retrospective survey of databases from two vasculitis centres between 1990 and 2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. All paediatric-onset adult patients met the Ankara 2008 (EULAR/PReS endorsed) criteria for childhood PAN. Those with typical angiographic and/or histopathologic findings consistent with PAN were included. 93 (M/F: 51/42) patients (UK: 47, TR: 46) were included. Three were HBV-related, 20 (21.5%) had paediatric onset and 16 (16.5%), cutaneous PAN. TR patients had younger age of disease onset 44 (28.5-59.0) vs. 24.5 (11.8-40.5), p = 0.002. Twelve (26%) of TR patients had monogenic disease (Familial Mediterranean Fever association (n = 7), deficiency of adenosine deaminase 2, DADA2, (n = 5). No difference was found in phenotype between paediatric and adult onset patients except for frequency of cutaneous lesions (p = 0.002). During a median 67.5 (32-126) months follow-up, 13 patients died (12.7% in UK vs. 15.2% in Turkish cohorts). No difference was found between two cohorts in relation to relapse rate, death and vasculitis damage index. This study defined a diagnosis of PAN according to the EMEA algorithm. The TR group had a younger age of disease onset and more cases of monogenic disease; however, disease extent, relapse rate, damage index and death rates were similar between groups.

No abstract

Objectives and AimsNetrin-1 is a laminin class protein that guides the axonal during the first embryonic development, has pushing and pulling properties and axonal chemoattractant activity. Netrin-1 has been shown to increase the development of fibrosis in mouse lung and human SSc lung cell culture. In this study, we aimed to investigate the relationship between Netrin-1 and Systemic sclerosis (SSc) and to emphasize the role of Netrin-1 in the pathophysiology of SSc, by increasing the known VEGF and M2 macrophage expression, which supports the fibrotic process.MethodsThe study included 56 SSc patients with a mean age of 48.08±13.59 years and 58 healthy volunteers with a mean age of 48.01±11.59 years. SSc organ involvements were scanned retrospectively from patient files and patients were grouped according to SSc complications. Calculation of Netrin-1 levels was performed using a quantitative sandwich enzyme immunoassay method with an ELISA kit (Elabscience, Texas, USA; catalog number: E-EL-H2328; lot number: GZWTKZ5SWK). Modified Rodnan scoring (MRS) was used for skin thickness scoring in SSc patients.ResultsThe mean of Netrin-1 was found to be significantly higher in SSc (309.51±211.86) than in controls (125.36±83.8) (p<0.001). In ROC analysis, a cut-off value of 354.24 for Netrin in SSc was found to provide a diagnostic sensitive confidence interval with 32.8% sensitivity and 98.3% specificity (AUC[95% CI]: 0.746-0.895, p<0.001). No significant correlation was found between netrin-1 level, organ involvement in SSc, and MRS (p>0.05).ConclusionIn this study, we found that there is a significant relationship between Netrin-1 levels and SSc disease. Our study is the first clinical study in which Netrin-1 elevation was demonstrated in SSc patients.KEY MESSAGESWhat is already known about this subject?Netrin-1 has proangiogenic, antiapoptotic,c, and anti-inflammatory properties and is closely related to the development of fibrosis.What does this study add?SSc hasta popülasyonunda Netrin-1 plazma düzeylerini değerlendiren bir çalışma bulunmamaktadır. Bu çalışmada SSc’de Netrin-1 ortalamasını sağlıklı kontrollere göre anlamlı derecede yüksek olduğunu bulunmuştur (p<0,001).How might this impact clinical practice or future developments?Considering the known roles of VEGF and M2 macrophages in the development of SSc and their close relationship with Netrin-1, further studies in this area seem to create a fertile field in understanding the complex pathophysiology of SSc and offering new therapeutic options.

No abstract

No abstract