Examination of the Level of Circulating Plasmablasts and Their Characteristics as Diagnostic Tools for Immunoglobulin G4-related Disease.

Background: Immunoglobulin G4-related disease (IgG4-RD) is a chronic, immune-mediated condition characterized by fibro-inflammatory lesions with lymphoplasmacytic infiltration. Diagnosis traditionally relies on histopathological findings, including the presence of IgG4+ plasma cells. However, due to challenges in biopsy accessibility, additional measures are needed to facilitate diagnosis.

Treatment of Scleroderma-Related Microstomia Using Hyaluronic Acid: An Interventional Study.

Systemic sclerosis (SSc) or scleroderma is a rare, systemic, autoimmune connective tissue disease. It causes increased collagen synthesis, leading to multi-organ sclerosis, including the skin and joints. Patients' overall health and quality of life are harmed dramatically.

Nonpharmacological interventions for 'no-option' refractory angina patients.

Refractory angina pectoris (RAP) defined as chronic anginal chest pain because of coronary artery disease (CAD) is a major problem. The increase in the number of patients with RAP in recent years is because of the increasing aging population and improved survival rates among patients with CAD. Management of patients with RAP is often extremely challenging.

Prevalence of anti-synthetase antibodies among systemic sclerosis patients.

Background: we aimed to estimate the prevalence of Amino acyl-transfer ribonucleic acid synthetase antibodies (Anti-ARS); myositis specific antibodies, among patients with systemic sclerosis (SSc), to evaluate the clinical associations of anti-ARS antibodies in SSc patients and to identify risk factors for development of interstitial lung disease (ILD) in SSc.

Methods: A prospective study of 71 systemic sclerosis patients in our rheumatology clinic in Israel. Sera were tested for myositis antibodies.

Functional IgG Autoantibodies against Autonomic Nervous System Receptors in Symptomatic Women with Silicone Breast Implants.

The association between the clinical picture of symptomatic women with silicone breast implants (SBI) and dysregulated immunity was in dispute for decades. In the current study, we describe for the first time the functional activity of purified IgG antibodies derived from symptomatic women with SBIs (suffering from subjective/autonomic-related symptoms), both in vitro and in vivo. We found that IgGs, derived from symptomatic women with SBIs, dysregulate inflammatory cytokines (TNFα, IL-6) in activated human peripheral blood mononuclear cells, compared to healthy-women-derived IgGs.

αvβ3 Integrin as a Link between the Development of Fibrosis and Thyroid Hormones in Systemic Sclerosis.

Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs. Key players mediating fibrosis are myofibroblasts (MF) that, following transforming growth factor β (TGFβ) exposure, produce a collagen-rich extracellular matrix (ECM) that induces myofibroblast differentiation. Myofibroblasts express αvβ3 integrin (a membrane receptor for thyroid hormones) and miRNA-21 that promotes deiodinase-type-3 expression (D3), causing the degradation of triiodothyronine (T3) that attenuates fibrosis.

Efficacy and Safety of Lenabasum, a Cannabinoid Type 2 Receptor Agonist, in a Phase 3 Randomized Trial in Diffuse Cutaneous Systemic Sclerosis.

Objective: This phase 3 study was undertaken to investigate the efficacy and safety of lenabasum, a cannabinoid type 2 receptor agonist, in patients with diffuse cutaneous systemic sclerosis (dcSSc).

Methods: A multinational double-blind study was conducted in 365 dcSSc patients who were randomized and dosed 1:1:1 with lenabasum 20 mg, lenabasum 5 mg, or placebo, each twice daily and added to background treatments, including immunosuppressive therapies (IST).

Results: The primary end point, the American College of Rheumatology combined response index in dcSSc (CRISS) at week 52 for lenabasum 20 mg twice a day versus placebo, was not met, with CRISS score of 0.

Catastrophic antiphospholipid syndrome in lupus-associated immune thrombocytopenia treated with eltrombopag A case series and literature review.

Background: Eltrombopag, a thrombopoietin receptor (TPO-R) agonist, is considered a second-line treatment for patients with refractory immune thrombocytopenia (ITP). Systemic lupus erythematosus (SLE) is frequently associated with ITP. In some cases, thrombocytopenia in SLE patients is attributed to concurrent antiphospholipid antibodies (APLA).

The Coronary Sinus Reducer Stent for the Treatment of Refractory Angina Pectoris: From the Conception of Innovation to Clinical Application.

The Neovasc Coronary Sinus Reducer Stent (CSRS) was developed for the treatment of patients who continue to suffer from disabling symptoms despite optimal medical therapy. This patient population with refractory angina symptoms is expected to grow, since life expectancy of individuals with atherosclerotic coronary artery disease is increasing. In this paper, we discussed the development of a novel device the CSRS and the upside-down strategy to rebuild a retrograde coronary pressure that was attenuated by the atherosclerotic disease.

Total Facial Autologous Fat Grafting for Treating Skin Manifestations in Scleroderma.

Systemic sclerosis (SSc) or scleroderma, is a rare, systemic autoimmune connective tissue disease that can cause fibrosis of cutaneous tissue and visceral organs. Facial involvement can have a deleterious effect on patients' function, cosmetic appearance and quality of life. This study describes our experience and results with total facial autologous fat grafting for treating scleroderma.

Steroid treatment suppresses the CD4 T-cell response to the third dose of mRNA COVID-19 vaccine in systemic autoimmune rheumatic disease patients.

Prolonged steroid treatment has a suppressive effect on the immune system, however, its effect on the cellular response to mRNA vaccine is unknown. Here we assessed the impact of prolonged steroid treatment on the T-cell and humoral response to the SARS-CoV-2 spike (S) peptide following the third dose of the BNT162b2 vaccine in systemic autoimmune rheumatic disease patients. We found that CD4 T-cell response to the S peptide in patients on high-dose long-term steroid treatment showed significantly less S-peptide specific response, compare to low-dose or untreated patients.

Thyroid Disorders in Patients with Systemic Sclerosis: Biochemical and Sonographic Characteristics. A Prospective Cohort Study.

Background: Patients with systemic sclerosis (SSc) are at increased risk for autoimmune thyroid diseases, but information regarding thyroid nodules and cancer in SSc is scarce. Objectives: To evaluate the thyroid gland in patients with SSc at a single Israeli center.

Methods: Thyroid workup was conducted in consecutive SSc patients: thyroid-stimulating hormone (TSH), free thyroxine (fT4), anti-thyroid peroxidase, and anti-thyroglobulin antibodies, as well as thyroid ultrasound and fine needle aspiration (FNA) when appropriate.

Immunogenicity and safety of the mRNA-based BNT162b2 vaccine in systemic autoimmune rheumatic diseases patients.

Background: The COVID-19 outbreak has led to the rapid development and administration of the COVID-19 vaccines worldwide. Data about the immunogenicity and adverse effects of the vaccine on patients with systemic autoimmune rheumatic diseases (SARDs) is emerging.

Aim: To evaluate Pfizer/BioNTech (BNT162b2) mRNA-based vaccine second-dose immunogenicity and safety, and the relation between them, in patients with SARDs.

Elevated Serum Levels of IgG4 in Patients with Heart Failure with Reduced Ejection Fraction: A Prospective Controlled Study.

(1) Background: Immunoglobulin gamma subclass 4 (IgG4) is a serum protein belonging to the immunoglobulin superfamily. It has a central role in certain immune-mediated conditions defined as IgG4-related disease. There is a paucity of data regarding the potential association of IgG4 and cardiovascular diseases.

Cognitive Impairment, Sleep Disturbance, and Depression in Women with Silicone Breast Implants: Association with Autoantibodies against Autonomic Nervous System Receptors.

Background: Silicone breast implants (SBIs) has been shown to be associated with an increased risk of autoimmune diseases. In the current study, we aimed to explore the potential association between circulating autoantibodies against the autonomic nervous system and cognitive impairment, memory deficit, and depressive symptoms reported by women with SBIs.

Methods: ELISA assays were used to quantify anti-adrenergic receptors (α1, α2, β1, β2), anti-muscarinic receptors (M1-M5), anti-endothelin receptor type A, and anti-angiotensin II type 1 receptor titers in the sera of 93 symptomatic female subjects with SBIs and 36 age-matched healthy female controls.

MiRNAs in Systemic Sclerosis Patients with Pulmonary Arterial Hypertension: Markers and Effectors.

Background: Pulmonary arterial hypertension (PAH) is a major cause of death in systemic sclerosis (SSc). Early detection may improve patient outcomes.

Methods: We searched for circulating miRNAs that would constitute biomarkers in SSc patients with PAH (SSc-PAH).

Low dose naloxone for pruritus in systemic sclerosis: Case series and literature review.

Rationale: Pruritus is a common symptom in patients with systemic sclerosis and has a tremendous effect on the quality of life. Nevertheless, current therapeutic options are limited. The pathogenesis of pruritus in systemic sclerosis is not completely understood; however, opiate-mediated neurotransmission has been postulated to be involved.

Autonomic Nervous System Indices in Patients with Systemic Sclerosis without Overt Cardiac Disease.

Background: Systemic sclerosis (SSc) is a connective tissue disease that may affect the heart and the autonomic nervous system (ANS). There is little knowledge regarding the degree of ANS involvement in SSc patients with unknown cardiac disease.

Objectives: To evaluate cardiac and pupillary autonomic functions in patients before cardiac involvement has emerged.

Tumor-like Lesions in Patients with Granulomatosis with Polyangiitis: A Case Series.

Background: Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis. It usually involves the respiratory tract and kidney. Rarely, tumor-resembling inflammatory changes ensue.