Organ Fibrosis: Emerging diagnostic and therapeutic strategies

Organ fibrosis is a very common condition affecting different organs and tissues following chronic tissue injury. Organ fibrosis can be defined as an excess deposition of fibrillar extracellular matrix (ECM) components which is, in turn, associated with impaired matrix degradation and remodelling. It represents a common pathological outcome of different causative agents leading to persistent tissue injury associated with a sustained activation of inflammatory response and deregulated wound healing. The progression of organ fibrosis, which significantly affects both the architectural structure and the function of the specific organ or tissue, represents a leading cause of morbidity and mortality worldwide. In addition, in specific tissues (e.g., the liver), the progression of tissue fibrogenesis represents the ideal background for aberrant tissue regeneration leading to cancer. Although in the last two decades several pre-clinical and clinical studies have significantly increased our knowledge concerning the basic mechanisms involved in the pathogenesis and progression of these diseases, we still essentially lack validated and effective anti-fibrotic therapies. The present special issue, in addition to recapitulate and update relevant basic pro-inflammatory and profibrogenic mechanisms (i.e., taking advantage from information emerging from novel methodological approaches and techniques) has been designed to project the current pathophysiology knowledge towards diagnostic and therapeutic applications. In the first review Isabella Lurie, Nadine Gaisa, Ralf Weiskirchen and Frank Tacke offer a general introduction on basic principles and mechanisms that can be considered as essentially common across organs and disease etiologies by involving persistent injury to parenchymal cells and triggering activation and deregulation of healing response. This includes an analysis of major pro-inflammatory and pro-fibrogenic mediators as well as of the intense cross-talk between the cells involved which are critical for disease progression. The following reviews are dedicated to fibrosis occurring in those organs or tissues in which disease progression will deeply affect structure and function, resulting in a major health impact on the general population, including: 2) Cardiac fibrosis (by Arantxa Gonzalez ´ Miqueo and colleguaes), 3) Kidney fibrosis (by Barbara Mara Klinkhammer and Peter Boor), 4) Pulmonary fibrosis (by Brintha Selvarajah, Manuela Platè and Rachel Chambers), 5) Liver fibrosis (by Maurizio Parola and Massimo Pinzani), 6) Skin fibrosis in systemic sclerosis (by David Abraham, Alain Lescoat and Richard Stratton), 7) intestinal fibrosis (by Antonio Di Sabatino and colleagues). We really hope that the readers will appreciate this concerted effort to provide an update on organ fibrosis and that the concepts brought up in the special edition of MAM will inspire younger researcher to pursue this area of research and contribute to effective cures in the not too far future.