Eleonora Ballanti MD, Maria Sole Chimenti MD PhD and Roberto Perricone MD
Systemic vasculitides are a group of uncommon diseases characterized by blood vessel inflammation. The complement system is involved in the pathogenesis and clinical manifestations of several autoimmune diseases, including systemic vasculitides. This enzymatic system is a component of the innate immune system. Its main function was initially believed to be limited to the recognition and elimination of pathogens, but research in recent years has demonstrated the important role that complement proteins play in modulating adaptive immunity and in bridging innate and adaptive responses. Its activation is also critical for the development of T cell immunity and natural antibodies as well as for the regulation of autoreactive B cells. In systemic vasculitides, particularly small-medium vessel vasculitides, the complement system has been shown to contribute to the development of inflammatory damage. In view of these crucial functions, the complement system represents an attractive therapeutic target for a wide range of diseases, including vasculitic disorders.
Luca Cantarini MD PhD, Giuseppe Lopalco MD, Marco Cattalini MD, Antonio Vitale MD, Mauro Galeazzi MD and Donato Rigante MD
Autoinflammatory and autoimmune disorders are characterized by chronic activation of the immune system, which leads to systemic self-directed inflammation in genetically predisposed individuals. Mutations in inflammasome-related proteins have been associated with autoinflammatory disorders, and the link between inflammasome and autoimmune disorders is becoming increasingly clear. As researchers learn more about these two areas, other disorders that were once thought to be autoimmune are now being considered autoinflammatory, or as having at least an autoinflammatory component. This review depicts the role of interleukin-1 as “Ariadne’s thread” on the path through the labyrinth of autoinflammatory and autoimmune disorders and emphasizes the blurred boundary between innate and adaptive immune systems.
Daphna Paran MD and Yaakov Naparstek MD
In the past decade we have witnessed a dramatic change in the management of autoimmune diseases, such as rheumatoid arthritis and spondyloarthropathies, due to the development of new biologic drugs designed to target key mediators in the autoimmune process. However, the development of similar target-specific drugs for the management of SLE has not been as successful. The B cell has long been considered central to the pathogenesis of SLE and has been regarded as an important target for biologic drugs. Several B cell-targeted drugs have been developed and although the mechanisms seem promising, most of the studies published to date have failed to achieve their primary endpoints, leading to an ongoing debate regarding the role of B cell therapy in SLE. The present report discusses the pros and cons of B cell-targeted therapy in SLE, reviews the clinical studies, and offers possible explanations for the discrepancies between randomized control studies and real-life experience.
Shirish R. Sangle MBBS MD and David P. D’Cruz MD FRCP
Albert Selva-O’Callaghan MD PhD and Ricard Cervera MD PhD FRCP
Carlo Selmi MD PhD, Elena Generali MD and Luca Cantarini MD PhD
Attila Kovacs MD PhD, Adelina G. Siminischi MD, Beáta Baksay MD, Andras Gall MD, Maria Takacs MD and Zoltan Szekanecz MD PhD
Siniša Roginic MD, Alan Jelic MD, Asja Stipic-Markovic MD PhD, Artukovic Marinko MD, Irena N. Artukovic MD and Martinovic-Kaliterna Dusanka MD PhD
Adam Austin MD, Angela Tincani MD, Shaye Kivity MD, María-Teresa Arango MSc and Yehuda Shoenfeld MD FRCP MaACR
Nurit Katz-Agranov MD, Amir Tanay MD, Daniel Bachar MD and Gisele Zandman-Goddard MD
Itzhak Sharabi MD, Amir Tanay MD and Gisele Zandman-Goddard MD
Abdulla Watad MD, Alessandra Soriano MD, Hananya Vaknine MD, Yehuda Shoenfeld MD FRCP MaACR and Howard Amital MD MHA
Abdulla Watad MD, Marina Perelman MD, Ribhi Mansour MD, Yehuda Shoenfeld MD FRCP MaACR and Howard Amital MD MHA