Three Diseases Mediated by Different Immunopathologic Mechanisms—ANCA-Associated Vasculitis, Anti-Glomerular Basement Membrane Disease, and Immune Complex-Mediated Glomerulonephritis—A Common Clinical and Histopathologic Picture: Rapidly Progressive Crescentic Glomerulonephritis

Immune mechanisms play an important role in the pathogenesis of glomerulonephritis (GN), with autoimmunity being the main underlying pathogenetic process of both primary and secondary GN. We present three autoimmune diseases mediated by different autoimmune mechanisms: glomerulonephritis in vasculitis mediated by anti-neutrophil cytoplasmic antibodies (ANCAs), glomerulonephritis mediated by anti-glomerular basement membrane antibodies (anti-GBM antibodies), and immune complex-mediated glomerulonephritis. Some of these diseases represent a common clinical and histopathologic scenario, namely rapidly progressive crescentic glomerulonephritis. This is a severe illness requiring complex therapy, with the main role being played by therapy aimed at targeting immune mechanisms. In the absence of immune therapy, the crescents, the characteristic histopathologic lesions of this common presentation, progress toward fibrosis, which is accompanied by end-stage renal disease (ESRD). The fact that three diseases mediated by different immunopathologic mechanisms have a common clinical and histopathologic picture reveals the complexity of the relationship between immunopathologic mechanisms and their clinical expression. Whereas most glomerular diseases progress by a slow process of sclerosis and fibrosis, the glomerular diseases accompanied by glomerular crescent formation can progress, if untreated, in a couple of months into whole-nephron glomerulosclerosis and fibrosis. The outcome of different immune processes in a common clinical and histopathologic phenotype reveals the complexity of the relationship of the kidney with the immune system. The aim of this review is to present different immune processes that lead to a common clinical and histopathologic phenotype, such as rapidly progressive crescentic glomerulonephritis

ORAL MANIFESTATIONS IN PRIMARY PEDIATRIC VASCULITIS

Vasculitis are disorders characterized by the presence of an inflammatory process in the blood vessel wall, resulting in damage or necroses of certain tissues or organs. Numerous clinical symptoms, ranging from acute localized hypersensitivity reactions to severe auto-immune systemic disorders that are incurable and life-threatening, can be attributed to the types and locations of affected arteries as well as the level of inflammation. Typical oral or facial symptoms of several forms of vasculitis can help in an early diagnosis of vasculitis. IgA vasculitis and Kawasaki disease (KD) are the two predominant types of pediatric vasculitis that involve the mouth, followed by ANCA vasculitis. Furthermore, SLE, a connective tissue disease, is one of the most prevalent autoimmune illnesses in children and can proceed rapidly across multiple organs or start mildly and gradually. Numerous more systemic conditions, such as infection, autoinflammatory disorders and neoplasia, can also primary or secondary localize in the oral cavity, infections being more frequent. Many various professionals, including dentists, family physicians, pediatricians, rheumatologists, hematologists, gastroenterologists, and otorhinolaryngologists, examine and treat children with oral symptoms. In 87.7% of patients overall and over 90% of patients with KD and IgA vasculitis (formerly known as Henoch-Schönlein purpura), cutaneous involvement was observed. Recurrent oral aphthous ulcers were present in all Behçet syndrome patients (1)