منابع مشابه
Recent advances in understanding and treating vasculitis
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are near universally fatal conditions if untreated. Although effective therapeutic options are available for these diseases, treatment regimens are associated with both short- and long-term adverse effects. The recent identification of effective B-cell-targeted therapy with an anti-CD20 monoclonal antibody has transforme...
متن کاملANCA-associated vasculitis in childhood: recent advances
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respir...
متن کاملRecent Advances in Systemic Scleroderma in Childhood
Introduction Systemic sclerosis (SSc; scleroderma) is a systemic autoimmune disease characterised by diverse skin and internal organ involvement. The underlying pathology consists of fibrosis caused by fibroblast dysfunction and small vessel vasculopathy. It is much rarer in childhood, occurring only 1 in 1 million children, and comprises approximately 3-10% of the adult incidence [1,2]. Only o...
متن کاملRecent Advances in Hemophilia Gene Therapy
Heterogeneous loss of function mutations at F8 and F9 genes causes X-linked recessive bleeding disorders, hemophilia A (HA) and hemophilia B (HB), respectively. HA is clinically indistinguishable from HB and accounts for more than 80% of hemophilia cases; the former affects 1/5000 and the latter 1/25000 male births worldwide. In Iran, it is estimated that around 4300 HA and 900 HB patients are ...
متن کاملRecent advances in managing systemic sclerosis
How the main components in systemic sclerosis-namely autoimmunity, vasculopathy, and fibrosis-fit together is still not sufficiently clear. However, vascular treatment options are well established, the body of evidence for the efficacy of immunomodulatory approaches is increasing, and now at least one hopeful substance that may directly interfere with fibrosis is being tested. Although we still...
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ژورنال
عنوان ژورنال: Journal of the Royal Society of Medicine
سال: 1990
ISSN: 0141-0768,1758-1095
DOI: 10.1177/014107689008300627