Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time....

Background Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis characterized by asthma, hypereosinophilia, cardiac failure, renal damage and peripheral neuropathy. CSS typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally in a systemic small-vessel vasculitis. During the late phase, coronary vessels and myocardium are ...

Polyarteritis nodosa (PAN) is a systemic vasculitis whose severe forms are treated with glucocorticoids and cyclophosphamide. Refractory patients are exposed to many complications, notably accelerated atherosclerosis. We report a case report of 71-year-old man followed for polyarteritis nodosa refractory to glucocorticoids and cyclosphosphamide. Systemic vasculitis relapses are followed to acce...

Hypocomplementemic urticarial vasculitis (HUV) is the rare immune complex vasculitis, affecting small vessels and associated with anti-C1q antibodies, presenting with urticaria and hypocomplementemia. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation are common in HUV. HUV may present as an idiopathic disease or be a part of systemic autoimmune or autoinflamm...

The association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA), has been described in a number of case reports and case series reports in the last 2 decades. In addition, patients with pulmonary fibrosis and ANCA positivity but without other manifestations of systemic vasculitis hav...

The pathogenesis of systemic vasculitis is complex and is likely to involve many mechanisms. There is a growing body of evidence that T cells may contribute to the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Predominantly, T cells and monocytes are found in inflammatory infiltrates in patients with Wegener's granulomatosis (WG). The production of ANCA ap...

Microscopic polyangiitis (MPA) is a rare systemic vasculitis with an incidence of about 1–3/100,000/year [1]. MPA is defined as a necrotizing vasculitis, with few or no immune deposits, primarily affecting small vessels including capillaries, venules or arterioles [2]. MPA mainly affects kidneys and lungs, nevertheless cutaneous vasculitis, musculoskeletal symptoms, gastrointestinal involvement...