scholarly journals Serum lysyl oxidase concentration increases in long-standing systemic sclerosis: Can lysyl oxidase change over time?

2021 ◽  
Author(s):  
Mohammad Hassan Jokar1† ◽  
Simin Jafaripour2† ◽  
Nafiseh Abdollahi1† ◽  
Maryam Nazemipour ◽  
Maliheh Moradzadeh ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lysyl Oxidase ◽  
Disease Onset ◽  
Significant Negative Correlation ◽  
Healthy Controls ◽  
Recent Onset ◽  
Modified Rodnan Skin Score ◽  
The Relationship ◽  
Circulating Levels ◽  
Onset Group

Objectives: This study aims to investigate the association of serum lysyl oxidase (LOX) levels with systemic sclerosis (SSc), to examine the relationship between LOX and disease onset, and to evaluate the probable effects of hyperlipidemia on the circulating levels of LOX among patients with SSc. Patients and methods: Between May 2017 and November 2018, a total of 39 patients with SSc (2 males, 37 females; mean age: 46.6±12.3 years; range, 18 to 65 years) and 35 healthy controls (4 males, 31 females; mean age: 43.1±14.1 years; range, 18 to 65 years) were included. Serum LOX concentration was measured using the enzyme-linked immunoassay in triplicate. Results: We found higher levels of serum LOX in patients with SSc compared to healthy controls. There was a significant relationship between serum LOX levels and disease onset. Patients with long-standing disease demonstrated increased levels of LOX in the blood compared to the recent-onset group. Hyperlipidemia did not have a significant effect on circulating levels of LOX. There was a significant negative correlation between LOX levels and modified Rodnan Skin Score in the subgroup of patients with skin involvement only and in patients without gastrointestinal involvement. Conclusion: Our study findings show an increased level of LOX protein level in the blood of patients diagnosed with SSc. Hyperlipidemia seems not to affect the concentrations of LOX in the peripheral blood of patients with SSc.

Theory of mind in the early course of schizophrenia: stability, symptom and neurocognitive correlates, and relationship with functioning

2015 ◽  
Vol 45 (10) ◽  
pp. 2031-2043 ◽  
Author(s):  
J. Ventura ◽  
A. Ered ◽  
D. Gretchen-Doorly ◽  
K. L. Subotnik ◽  
W. P. Horan ◽  
...  
Keyword(s):  
Theory Of Mind ◽  
Negative Symptoms ◽  
Chronic Schizophrenia ◽  
First Episode ◽  
Positive Symptoms ◽  
Healthy Controls ◽  
Role Functioning ◽  
Recent Onset ◽  
Early Course ◽  
The Relationship

BackgroundNumerous studies have reported links between theory of mind (ToM) deficits, neurocognition and negative symptoms with functional outcome in chronic schizophrenia patients. Although the ToM deficit has been observed in first-episode patients, fewer studies have addressed ToM as a possible trait marker, neurocognitive and symptom correlations longitudinally, and associations with later functioning.MethodRecent-onset schizophrenia patients (n = 77) were assessed at baseline after reaching medication stabilization, and again at 6 months (n = 48). Healthy controls (n = 21) were screened, and demographically comparable with the patients. ToM was assessed with a Social Animations Task (SAT), in which the participants’ descriptions of scenes depicting abstract visual stimuli ‘interacting’ in three conditions (ToM, goal directed and random) were rated for degree of intentionality attributed to the figures and for appropriateness. Neurocognition, symptoms and role functioning were also assessed.ResultsOn the SAT, patients had lower scores than controls for both intentionality (p < 0.01) and appropriateness (p < 0.01) during the ToM condition, at baseline and 6 months. The ToM deficit was stable and present even in remitted patients. Analyses at baseline and 6 months indicated that for patients, ToM intentionality and appropriateness were significantly correlated with neurocognition, negative symptoms and role functioning. The relationship between ToM and role functioning was mediated by negative symptoms.ConclusionsThe ToM deficit was found in recent-onset schizophrenia patients and appears to be moderately trait-like. ToM is also moderately correlated with neurocognition, negative and positive symptoms, and role functioning. ToM appears to influence negative symptoms which in turn makes an impact on role functioning.

scholarly journals Evaluation of thyroid volume in patients with systemic sclerosis; what did we find?

2021 ◽  
Author(s):  
Suade BADAK ◽  
Bozkurt GÜLEK ◽  
Esra KAYACAN ERDOĞAN ◽  
Hülya BİNOKAY ◽  
Eren ERKEN
Keyword(s):  
Systemic Sclerosis ◽  
Severity Score ◽  
Thyroid Dysfunction ◽  
Disease Duration ◽  
Thyroid Volume ◽  
Significant Negative Correlation ◽  
Skin Thickness ◽  
Cross Sectional ◽  
Skin Score ◽  
Modified Rodnan Skin Score

Introduction: Systemic sclerosis is a multisystemic disease. Thyroid involvement in systemic sclerosis is an issue that can be ignored. Our study aimed to evaluate the decreased thyroid volume in SSc. Also, we aimed to show the relationship between patients’ thyroid volume and severity score, clinical and laboratory parameters. Method: This was a single-center, cross-sectional study. Eighty-eight patients were included in the study. A radiologist evaluated patients’ thyroid volumes by ultrasonography. Demographic and clinical characteristics of the patients were recorded. Skin thickness was evaluated by the modified Rodnan skin score and the disease severity by the Medsger severity score. Findings were analyzed statistically. Results: Thyroid volume was in the atrophic range in 53.4% of the patients. There was a significant negative correlation between thyroid volume and mRSS, MSS, and disease duration. Logistic regression analysis showed that modified Rodnan skin score and disease duration were risk factors for thyroid atrophy. Conclusions: Many studies point out that thyroid autoantibodies are a cause of thyroid dysfunction in patients with SSc. However, in most of these studies, thyroid volume was not evaluated. As a result of our study, we saw that the major cause of thyroid dysfunction in our SSc patients was thyroid atrophy. Also, we observed that thyroid atrophy was more common in patients with ILD. We would like to draw attention to the fact that thyroid dysfunction and volume changes increase with the disease’s duration and severity in systemic sclerosis.

scholarly journals Increased circulating levels of FGF23: an adaptive response in primary hyperparathyroidism?

2012 ◽  
Vol 166 (1) ◽  
pp. 55-60 ◽  
Author(s):  
Janneke E Witteveen ◽  
Antoon H van Lierop ◽  
Socrates E Papapoulos ◽  
Neveen A T Hamdy
Keyword(s):  
Primary Hyperparathyroidism ◽  
Fibroblast Growth Factor 23 ◽  
Negative Relationship ◽  
Significant Negative Correlation ◽  
Fgf23 Concentration ◽  
Biochemical Evaluation ◽  
Turnover Markers ◽  
The Relationship ◽  
Circulating Levels

IntroductionFibroblast growth factor 23 (FGF23) and parathyroid hormone (PTH) are major players in the bone–parathyroid–kidney axis controlling phosphate homeostasis. In patients with primary hyperparathyroidism (PHPT), data on the relationship between PTH and FGF23 are scarce and not always concordant.ObjectiveThe aim of our study was to evaluate the relationship between PTH and FGF23 in patients with PHPT and in euparathyroid patients cured after successful parathyroidectomy (PTx).Patients and methodsTwenty-one patients with PHPT and 24 patients in long-term cure after successful PTx (EuPTH) were studied. All patients underwent biochemical evaluation of renal function, parathyroid status, vitamin D status, bone turnover markers, and serum intact FGF23 levels.ResultsMean serum FGF23 concentration was significantly higher in PHPT than in EuPTH patients (50.8±6.1 vs 33.1±2.6 pg/ml,P=0.01). FGF23 levels significantly correlated with PTH levels (r=0.361,P=0.02), also after correction for 1,25(OH)2D levels (r=0.419,P=0.01). FGF23 levels showed a significant negative correlation with 1,25(OH)2D, which was more pronounced in PHPT than in EuPTH patients (r=−0.674,P=0.001, vsr=−0.509,P=0.01).ConclusionOur findings suggest that in PHPT, FGF23 levels are increased independent of 1,25(OH)2D levels. The more pronounced negative relationship between FGF23 and 1,25(OH)2D in the presence of high circulating PTH levels suggests that the increase in FGF23 levels may be an adaptive mechanism to counteract the PTH-induced increase in 1,25(OH)2D levels, although not completely overriding it.

scholarly journals Vascular Leak Is a Central Feature in the Pathogenesis of Systemic Sclerosis

2012 ◽  
Vol 39 (7) ◽  
pp. 1385-1391 ◽  
Author(s):  
TRACY M. FRECH ◽  
MONICA P. REVELO ◽  
STAVROS G. DRAKOS ◽  
MAUREEN A. MURTAUGH ◽  
BOAZ A. MARKEWITZ ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Punch Biopsy ◽  
Central Feature ◽  
Healthy Controls ◽  
Digital Microscopy ◽  
Interstitial Edema ◽  
Primary Analysis ◽  
Vascular Leak ◽  
Recent Onset ◽  
Microscopic Features

Objective.The main histopathological focus of systemic sclerosis (SSc) has concentrated on fibrotic changes. We investigated the microvasculature alterations in the skin of patients with SSc at various stages of disease duration with whole-field digital microscopy.Methods.Twenty consecutive patients with SSc, 1 with Raynaud’s phenomenon (RP) without SSc, and 4 healthy controls underwent punch biopsy on the medial forearm. Eighteen patients were included in the primary analysis. Two with recent-onset diffuse cutaneous disease, 1 repeat SSc biopsy, and 1 patient with RP without SSc were also evaluated. All specimens were processed with histochemical stains and immunohistochemistry. We analyzed microvasculature abnormalities in an objective and systematic manner taking advantage of recent advances in whole-field digital microscopy. This analysis was coupled with ultrastructural evaluation performed with transmission electron microscopy (TEM).Results.Whole-field digital microscopy and TEM of SSc skin biopsies revealed that endothelial abnormalities are a universal feature regardless of clinical features and/or duration of disease. These features were not seen in any healthy control specimens or in the single RP patient samples. Whole-field digital microscopy identified increased interstitial edema (31.0% ± 9.6% vs 17.6% ± 3.3% in controls; p = 0.009) and fibrosis (75.6% ± 5.7% vs 66.1% ± 9.8% in controls; p = 0.02) in all patients with SSc. Lower CD34 staining was seen in SSc compared to healthy controls (0.32% ± 0.22% vs 1.31% ± 0.34%; p < 0.0001) and within the SSc population with interstitial lung disease (0.55% ± 0.22% vs 0.15% ± 0.16%; p = 0.01). Perivascular and interstitial infiltrate of mast cells was present in all SSc specimens.Conclusion.Whole-field digital microscopy offers a means of rapidly carrying out objective, fully quantitative, and reproducible measurements of microscopic features of SSc microvascular change. The universal morphologically abnormal endothelial cells and interstitial edema in all patients with SSc biopsied suggests that SSc may be intrinsically a disease of the endothelium characterized by vascular leak.

scholarly journals Ultrasound Elastography Assessment of Skin Involvement in Systemic Sclerosis: Lights and Shadows

2010 ◽  
Vol 37 (8) ◽  
pp. 1688-1691 ◽  
Author(s):  
ANNAMARIA IAGNOCCO ◽  
OLGA KALOUDI ◽  
CHIARA PERELLA ◽  
FRANCESCA BANDINELLI ◽  
VALERIA RICCIERI ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Imaging Modality ◽  
Ultrasound Elastography ◽  
Band Spectrum ◽  
Elastic Tissue ◽  
Healthy Controls ◽  
Skin Involvement ◽  
Tissue Elasticity ◽  
Skin Score ◽  
Modified Rodnan Skin Score

Objective.To assess skin elasticity in systemic sclerosis (SSc) by using a new imaging modality, ultrasound elastography (UE).Methods.Our study included 18 consecutive patients with SSc and 15 healthy controls. Modified Rodnan skin score, physical examination, and assessment of organ involvement were performed. UE was carried out on the middle forearm and on the fingers of the dominant arm. The echo signals recorded in real time during freehand operations of probe compression and relaxation produced images representing tissue elasticity, consisting of translucent colored bands superimposed on the B-mode ultrasonographic images. The color scale varied within a large band spectrum from red, indicative of soft and highly elastic tissue, to blue, which denoted hard and barely elastic tissue.Results.On the forearm of all patients, UE showed a homogeneous blue area corresponding to the dermis visualized in a B-mode ultrasonographic image; in controls, a blue pattern was never detected and a predominance of green with sporadic areas of pale blue was observed. At sequential evaluations, UE of fingers produced inconstant and changeable colored areas.Conclusion.The imaging pattern observed in the forearm of patients with SSc may represent the reduction of strain in the dermis due to loss of elasticity. The variable pattern obtained by finger evaluation demonstrated that UE can assess skin involvement in SSc only in those areas where the dermis is known to be thicker and where the bone hyperreflection is minimal. Further studies are needed to confirm our results and determine the validity of this new imaging modality.

scholarly journals Older age onset of systemic sclerosis – accelerated disease progression in all disease subsets

Rheumatology ◽  
2020 ◽  
Vol 59 (11) ◽  
pp. 3380-3389
Author(s):  
Pia Moinzadeh ◽  
Kathrin Kuhr ◽  
Elise Siegert ◽  
Ulf Mueller-Ladner ◽  
Gabriela Riemekasten ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Immunosuppressive Treatment ◽  
Disease Onset ◽  
Age Groups ◽  
The Elderly ◽  
Multisystem Disease ◽  
Significant Difference ◽  
Elderly Cohort ◽  
The Relationship

Abstract Objectives Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype. We here investigate the relationship between age at disease onset and symptoms in a large cohort of SSc patients (lcSSc, dcSSc and SSc-overlap syndromes). Methods Clinical data of the registry of the German Network for Systemic Scleroderma including 3281 patients were evaluated and subdivided into three age groups at disease onset (&lt;40 years, 40–60 years, &gt;60 years). Results Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age &lt;40 years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the lcSSc subset significantly more often. Furthermore, they had pulmonary hypertension more often, but digital ulcerations less often. Remarkably, the course of the disease was more rapidly progressing in the older cohort (&gt;60 years), except for gastrointestinal and musculoskeletal involvement. No significant difference was found for the use of corticosteroids. However, significantly, fewer patients older than 60 years received immunosuppressive treatment. Conclusion In this large registry, ∼25% of patients developed SSc at an age above 60 years with an increased frequency of lcSSc. In this age group, an onset of internal organ involvement was significantly accelerated across all three subsets. These findings suggest that, in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications.

Characteristics of Patients with Juvenile Onset Systemic Sclerosis in an Adult Single-center Cohort

2010 ◽  
Vol 37 (11) ◽  
pp. 2422-2426 ◽  
Author(s):  
IVAN FOELDVARI ◽  
SVETLANA I. NIHTYANOVA ◽  
ANGELA WIERK ◽  
CHRISTOPHER P. DENTON
Keyword(s):  
Systemic Sclerosis ◽  
Survival Rates ◽  
Disease Onset ◽  
High Survival ◽  
Adult Onset ◽  
Single Center ◽  
Juvenile Onset ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Onset Group

Objective.Systemic sclerosis (SSc) is a rare connective tissue disease in childhood. We compared the characteristics of adult patients with juvenile-onset SSc (jSSc) from a single-center cohort to an adult-onset group.Methods.Patients with disease onset before the age of 17 years were included in the jSSc cohort, while subjects with SSc onset after age 17 formed the adult-onset cohort.Results.We identified 52 adult subjects with jSSc and compared them to 954 patients with adult-onset SSc. The mean ± SD age at disease onset of the patients with jSSc was 14 ± 2 years, 39 (75%) of them were women, and 24 (46%) had the diffuse cutaneous subset of SSc (dcSSc). There were no differences between the 2 cohorts in terms of sex and disease subset. Overlaps were significantly more frequent among the jSSc cohort (37%) compared to the adult-onset group (18%; p = 0.002). Autoantibody analysis demonstrated significantly more antitopoisomerase I antibody-positive subjects (33% vs 20%; p = 0.034) and significantly fewer anticentromere antibody-positive subjects (2% vs 25%; p < 0.001) in the jSSc cohort. Compared to the adult-onset group at 10 years from disease onset, survival was significantly higher among the subjects with jSSc (98% vs 75%; p = 0.001), pulmonary arterial hypertension had a significantly lower incidence (2% vs 14%; p = 0.032), and there was no difference in terms of pulmonary fibrosis (22% vs 21%) and cardiac scleroderma (3% vs 2%) between the 2 groups.Conclusion.The high survival rates and lower proportion of dcSSc in the adult jSSc cohort may represent a survival bias.

scholarly journals High acute phase protein levels correlate with pulmonary and skin involvement in patients with diffuse systemic sclerosis

2018 ◽  
Vol 46 (4) ◽  
pp. 1634-1639 ◽  
Author(s):  
Anna Lis-Święty ◽  
Małgorzata Widuchowska ◽  
Ligia Brzezińska-Wcisło ◽  
Eugeniusz Kucharz
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Involvement ◽  
Control Group ◽  
Healthy Controls ◽  
Protein Levels ◽  
Reactive Protein ◽  
Amyloid A ◽  
Diffuse Systemic Sclerosis ◽  
Modified Rodnan Skin Score ◽  
Serum Crp

Objective This study was performed to evaluate the serum amyloid A (SAA) and C-reactive protein (CRP) levels in patients with diffuse systemic sclerosis (dSSc) in relation to a control group, disease duration, and skin and pulmonary involvement. Methods This case-control study included 18 patients with early dSSc, 15 patients with late dSSc, and 15 healthy controls. The SAA and CRP levels, modified Rodnan skin score (mRSS), and diffusing capacity of the lungs for carbon monoxide (DLCO) were determined in all patients. Results The SAA and CRP levels were significantly higher in patients with early and late dSSc than in healthy controls. The frequency of detection of elevated SAA and CRP levels was approximately 66% and 85%, respectively. A significant correlation was found between the SAA and CRP levels in patients with dSSc. The SAA and CRP levels were inversely correlated with DLCO. The CRP level was positively correlated with the mRSS. Conclusions High SAA and CRP levels could serve as biomarkers for pulmonary involvement. The serum CRP level accurately reflects the extension of skin thickening in patients with dSSc.

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