Genetic variants in LGALS3 are related to lower galectin-3 serum levels and clinical outcomes in systemic sclerosis patients: A case-control study

CONCLUSION: The LGALS3 rs4652 A/C was more frequent in SSc patients and related to lower Gal-3 levels. These findings were corroborated through a GLM to estimate Gal-3 values. Also, by model equations, Gal-3 levels may be predicted by HAQ, SHAQ, PAH, myopathy, and LGALS3 rs4652 and rs2075601 factors. In these ways, we suggest that galectins may be promising biomarkers to identify susceptibility to SSc as well as to identify HAQ, SHAQ, PAH, and myopathy outcomes.PMID:33973825 | DOI:10.1080/08916934.2021.1919881
Source: Autoimmunity - Category: Allergy & Immunology Authors: Source Type: research

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We report the case of an elderly male with SSc-SLE overlap syndrome combined with scleroderma renal crisis and SSc-TMA. Diagnoses: The patient has typical of SSc on the face and hands, combined with pulmonary artery hypertension, interstitial lung disease, heart failure and malignant hypertension, as well as SLE, lupus nephritis class V, and TMA, which were definitively diagnosed by clinical laboratory examination and renal histopathology. Interventions: The patient was treated with prednisone, cyclophosphamid, renin-angiotensin system inhibitors, diuretics, and acetylcysteine. Outcomes: The patient died suddenly ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
We present a clinical case of SRC with a typical presentation of hypertensive emergency and acute kidney injury.
Source: High Blood Pressure and Cardiovascular Prevention - Category: Cardiology Source Type: research
ConclusionWnt ‐1 and Wnt‐2 were found higher in scleroderma and organ involvement. They may play a role in the pathogenesis of the disease.
Source: International Journal of Rheumatic Diseases - Category: Rheumatology Authors: Tags: ORIGINAL ARTICLE Source Type: research
This study was u ndertaken to gain insight into the association between characteristics of the anti–topo I antibody response and clinical disease course in SSc patients positive for anti–topo I antibodies.MethodsLevels of anti –topo I IgG, anti–topo I IgM, and anti–topo I IgA were assessed in consecutive serum samples obtained from patients at baseline who were positive for anti–topo I IgG in the Leiden Combined Care In Systemic Sclerosis (CCISS) cohort. One‐year disease progression was defined by a relevant inc rease in modified Rodnan skin thickness score (MRSS), decline in pulmonary...
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Original Article Source Type: research
Authors: Kato M, Sugimoto A, Atsumi T Abstract INTRODUCTION: Pulmonary arterial hypertension (PAH), also referred to as group 1 pulmonary hypertension, occurs either primarily or in association with other diseases such as connective tissue diseases (CTD). Of CTD, systemic sclerosis (SSc), systemic lupus erythematosus and mixed connective tissue disease are commonly accompanied with PAH. It is of note that SSc-PAH is associated with distinctive histopathology, an unfavorable outcome, and a blunted responsiveness to modern PAH therapies. AREAS COVERED: The data in articles published until May 2020 in peer-reviewe...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
This study seeks to determine whether CBC parameters associated with organ involvement, when evaluated together with clinical features and autoantibodies, can additionally contribute to risk estimation. METHODS: Adult patients with SSc (n = 130) and healthy control (n = 129) groups were enrolled in the study. Epidemiological, clinical, laboratory, and radiological findings were obtained by examining patient records. RESULTS: PLR, NLR, and MLR were related to organ involvement. Statistically significant results were obtained with hemoglobin (≤ 13.0 g/dl), lymphocyte count (≤ 1,900 × 103/ml), and mean pla...
Source: Acta Dermatovenerologica Alpina, Panonica, et Adriatica - Category: Dermatology Tags: Acta Dermatovenerol Alp Pannonica Adriat Source Type: research
Abstract Nailfold videocapillaroscopy (NVC) changes in systemic sclerosis (SSc) are correlated with vascular complications, such as pulmonary arterial hypertension (PAH), supporting a potential link between peripheral and internal organ vasculopathy. The current stage of knowledge regarding NVC and PAH is discussed, focusing on the assessment of peripheral microangiopathy and a potential relationship with functional, echocardiographic, and haemodynamic markers of cardiac dysfunction. A comprehensive literature search was carried out to identify all studies focusing on NVC findings in patients with PAH, diagnosed w...
Source: Scandinavian Journal of Rheumatology - Category: Rheumatology Authors: Tags: Scand J Rheumatol Source Type: research
ConclusionILD confers a worse prognosis to SSc ‐PH. Response to PAH specific therapy is poor clinically in SSc‐PH‐ILD but was not found hemodynamically different from that observed in SSc‐PAH.
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: FULL LENGTH Source Type: research
Authors: Rosato E, Leodori G, Gigante A, Di Paolo M, Paone G, Palange P Abstract OBJECTIVES: Major vascular complication, such as digital ulcers (DUs), pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC) are hallmarks of systemic sclerosis (SSc). Interstitial lung disease (ILD) is the major cause of mortality in SSc. The aim of study is to identify cardiopulmonary exercise testing (CPET) variables that predict MVC and mortality for ILD in SSc patients. METHODS: In this cohort study, 45 SSc patients underwent clinical evaluation, echocardiography, pulmonary function tests (PFTs), high resolu...
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
AbstractPurpose of ReviewSystemic sclerosis or scleroderma (SSc) is a systemic, immune-mediated disease characterized by abnormal cutaneous and organ-based fibrosis that results in progressive end-organ dysfunction and decreased survival. SSc results in significant challenges for the practicing anesthesiologist due to its rarity, multi-system involvement, and limited evidence-based guidance for optimal perioperative care. In this update, we briefly discuss the recent evidence on the pathophysiology and current management of SSc, review the anesthesia-related literature, and extrapolate these observations into an optimal pe...
Source: Current Anesthesiology Reports - Category: Anesthesiology Source Type: research
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