The observation data of 22 children with systemic vasculitis (SV) and morphologically verified nephritis is presented. Emphasis was put on extrarenal manifestations SV in childhood, analysis clinical, laboratory, immunological, morphological changes factors influencing the course prognosis. It has been established that often manifests general non-specific constitutional symptoms, involvement EN...

We describe a case of Wegener's disease with massive painful bilateral submandibular gland enlargement as the presenting symptom. The diagnosis was based on histologically documented nasal mucosa involvement, focal necrotizing glomerulonephritis, episcleritis and anti-neutrophil cytoplasmic antibody.

Integrin activation is required for neutrophil functions. Impaired integrin activation on neutrophils is the hallmark of leukocyte adhesion deficiency (LAD) syndrome in humans, characterized by impaired leukocyte recruitment and recurrent infections. The Src kinase-associated phosphoprotein 2 (Skap2) is involved in integrin functions in different leukocyte subtypes. However, the role of Skap2 i...

background: the prevalence of anti-neutrophil cytoplasmic antibodies (ancas) and anti-cardiolipin antibodies (anti-cl ab) in behcet’s disease (bd) and also their roles in vascular involvement is controversial. objective: to assess the prevalence of ancas and anti-cl ab as well as their correlations with clinical manifestations in iranian patients with bd. methods: in this case/control study, th...

Introduction: Granulomatosis with polyangiitis (GPA; also known as Wegener’s granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otolo...

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic necrotizing vasculitis, which affects small and medium sized blood vessels and is often associated with cytoplasmic anti-neutrophil cytoplasmic antibodies (ANCA). Inflammatory pseudotumor is a rare condition characterized by the appearance of a mass lesion that mimics a malignant tumor both clinica...

Proteinase 3 (Pr3), the main target of anti-neutrophil cytoplasmic antibodies, is a neutrophil serine protease that may be constitutively expressed at the surface of quiescent circulating neutrophils. This raises the question of the simultaneous presence in the circulation of constitutive membrane-bound Pr3 (mPr3) and its plasma inhibitor alpha1-protease inhibitor (alpha1-Pi). We have looked at...

New therapeutic approaches that can accelerate neutrophil apoptosis under inflammatory conditions to enhance the resolution of inflammation are now under study. Neutrophils are deprived of proliferative capacity and have a tightly controlled lifespan to avoid their persistence at the site of injury. We have recently described that the proliferating cell nuclear antigen (PCNA), a nuclear factor ...

1. Csernok E, Lamprecht P, Gross WL. Diagnostic significance of ANCA in vasculitis. Nat Clin Pract Rheumatol 2006;2:174–5. 2. Radice A, Sinico RA. Antineutrophil cytoplasmic antibodies (ANCA). Autoimmunity 2005;38:93– 103. 3. Savige J, Davies D, Falk RJ, Jennette JC, Wiik A. Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney I...