Clinical Research Directory

AutoInflammatory Disease Alliance Registry (AIDA)

Autoinflammatory diseases (AID) are clinical entities characterized by recurrent inflammatory attacks in absence of infection, neoplasm or deregulation of the adaptive immune system. Among them, hereditary periodic syndromes, also known as monogenic AID, represent the prototype of this disease group, caused by mutations in genes involved in the regulation of innate immunity, inflammation and cell death. Based on recent experimental acquisitions in the field of monogenic AID, several immunologic disorders have been reclassified as polygenic/multifactorial AID, sharing pathogenetic and clinical features with hereditary periodic fevers. This has paved the way to new treatment targets for patients suffering from rare diseases of unknown origin, including Behçet's disease, Still disease, Schnitzler's disease, PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome, chronic recurrent multifocal osteomyelitis (CRMO), non-infectious uveitis and scleritis. Gathering information on such rare conditions is made difficult by the small number of patients, along with the difficulty of obtaining an accurate diagnosis in non-specialized clinical settings. In this context, the AIDA project promotes international collaboration among clinical centres to develop a permanent registry aimed at collecting demographic, genetic, clinical and therapeutic data of patients affected by monogenic and polygenic AID, in order to expand the current knowledge of these rare conditions.

Randomized Controlled Multicenter Study Comparing Steroid Therapy Plus Anticoagulants to Steroid Therapy Alone in Deep Venous Thrombosis of Behçet's Syndrome

In patients with Behçet's syndrome (BS), deep venous thrombosis (DVT) is thought to result from inflammation of the vessel wall rather than hyper coagulability. Post Thrombotic Syndrome (PTS) is frequent especially with recurrent episodes of deep vein thrombosis and may result in leg ulcers that are very difficult to treat. Vascular involvement is a major cause of morbidity and mortality among BS patients. However, one of the most controversial issues regarding the management of BS is whether DVT should be treated with anticoagulants. Moreover, use of anticoagulants exposes patients to serious bleeding, especially in those who presents simultaneous arterial aneurysms. However, many physicians are still using anticoagulants. This is the first prospective, randomized study assessing benefits of corticosteroids associated with anticoagulant compared to that of corticosteroids alone in DVT in BS patients. It will validate or not the use of anticoagulants in those situations. It will allow a direct comparison of the safety profile of those two schemes of treatment.

Evaluation of Serum Follistatin-Like Protein 1 Levels in Behcet's Disease and Its Association With Disease Activity

Behçet's disease (BD) is a multi-system auto inflammatory disorder with vasculitic features. The exact etiological of BD is still obscure, although environmental and genetics factors have been found to be involved in disease pathogenesis.FSTL-1 can act as diagnostic and prognostic biomarkers for Some inflammatory autoimmune diseases including BD.

Pan-immune-inflammation Value (PIN) in Behçet's Disease and Its Correlation With Disease Activity

Behçet's disease (BD) is a systemic vasculitis affecting both small and large blood vessels in the venous and arterial systems. It is a multiorgan disease with various manifestations, including mucocutaneous, articular, ocular, gastrointestinal, vascular, and neurological involvements. The aim of present study is to assess pan immune inflammation value (PIN) value in BD in comparison to healthy control ,assess the PIN value in association with demographics and clinical characteristics of Behcet patients and to assess the PIN value in association with disease activity.