Putative functional pathogenic autoantibodies in systemic sclerosis

Abstract

The aim of the European Journal of Rheumatology is to cover various aspects of rheumatology for its readers, encompassing the spectrum of diseases with arthritis, musculoskeletal conditions, autoinflammatory diseases, connective tissue disorders, osteoporosis, translational research, the latest therapies and treatment programs. European Journal of Rheumatology publishes original articles, invited reviews, case based reviews, letters to the editor and images in rheumatology. The publication language of the journal is English.

Iloprost Attenuates Oxidative Stress-Dependent Activation of Collagen Synthesis Induced by Sera from Scleroderma Patients in Human Pulmonary Microvascular Endothelial Cells

“…Similarly, an SSc-induced increase in COL1A1 synthesis resulted in significantly attenuated HPMECs exposed to sera of iloprost-treated SSc patients, suggesting collagen synthesis activation as a potential target of the loprost therapeutic effect ( Figure 1 D). A possible explanation of the iloprost treatment’s beneficial effect might be the ability to decrease the levels of circulating pro-oxidant and pro-fibrotic factors, which has been reported as increased in the SSc sera [ 28 , 34 , 35 , 36 , 37 , 38 ]. In this context, consonant with our data, multiple lines of evidence indicate the ability of Iloprost to modulate oxidative markers at systemic levels [ 25 , 39 , 40 , 41 ].…”

Systemic Sclerosis: From Pathophysiology to Novel Therapeutic Approaches

“…The latter report highlights, once more, the role of the immune system in SSc. Dysregulation of the immune system is witnessed by the presence of autoantibodies, several of which are exclusive of this disease and associated with clinical complications and specific phenotypes ( Table 1 ) [ 6 , 16 ]. Some of these autoantibodies have been directly implicated in the pathogenesis of SSc.…”

“…While there are still many unanswered questions, understanding of these pathways has greatly improved in recent years. In particular, the central role of immune system cells and inflammatory mediators, fibroblasts, and other cells determining the regulation of the extracellular matrix (ECM) is now recognized [ 6 , 7 ].…”

Set Up for Failure: Pre-Existing Autoantibodies in Lung Transplant

“…Interestingly, many CTDs can be categorized as autoimmune disorders suggesting that ILD may also comprise an autoimmune component. For example, ILD manifests and is the leading cause of mortality in Systemic-Sclerosis, an extensively studied autoimmune disease with anti-nuclear autoantibodies (ANA) (anti-centromere antibodies, anti-topoisomerase I antibodies, and anti-RNA polymerase antibodies) and anti-endothelial cell antibodies (AECA) in greater than 90% of patients with potential pathogenic roles ( 18 ). Additionally, ILD occurs in upwards of 80% of patients with myositis, a rare autoimmune disease that presents with myositis-specific autoantibodies (MSAs; anti-synthetase and anti-CADM140/MDA5).…”