The systemic vasculitides are characterized by inflammation of blood vessel walls. Vessels of any type, in any organ can be affected, resulting in a broad spectrum of signs and symptoms. The heterogenous nature of vasculitides presents a diagnostic challenge. The American College of Rheumatology classification criteria and the Chapel Hill Consensus Conference nomenclature are the most widely us...

Pauci -immune necrotising glomerulonephritis with extracapillary proliferation is a common renal histological manifestation of systemic vasculitis. Although its aetiopathogenesis is not well known, it is possible that environmental factors may have a bearing on genetically predisposed subjects, since there are many familial cases of systemic vasculitis. The frequent association between systemic...

Retinal vasculitis is a poorly characterized, potentially sight-threatening, inflammatory ocular condition that occurs when there is the presence of abnormal blood vessels in the retina. The annual incidence of retinal vasculitis in the United States is estimated at 1-2 per 100,000 with variation between regions. Because it is not well understood, the definition of “retinal vasculitis” is not w...

Although the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis....

The development of immunological abnormalities in various neoplasms is a rather common phenomenon. The prevalence of life-threatening systemic vasculitis in malignancy, however, is much lower. Nonetheless we found an unexpected frequency of several autoimmune manifestations, including systemic vasculitis, in certain myelodysplastic syndromes. We illustrate this finding with the case of a 43-yea...

Systemic vasculitis is a heterogeneous group of disorders characterized by inflammation andnecrosis in the vessel wall. The diagnosis of a systemic vasculitis is challenging, because patients usually present a broad spectrum of manifestations that vary according to the predominant size of vessels affected, organs and systems involved, and the extent of the inflammatory process (1). In systemic ...

Allergic granulomatous angiitis (Churg-Strauss syndrome) is a variant of systemic vasculitis characterized by pulmonary and systemic small vessel vasculitis, extra-vascular granulomas and eosinophilia. Clinical features include presence of asthma and/or allergic rhinitis with constitutional symptoms such as fever and weight loss. Multisystem involvement includes pulmonary infiltrations, m...

Cutaneous vasculitis may present as a significant component of many systemic vasculitic syndromes such as rheumatoid vasculitis or anti-neutrophil cytoplasmic antibody (ANCA) associated primary vasculitic syndromes like microscopic polyangiits. Cutaneous vasculitis manifested most often as palpable purpura or infiltrated erythema indicating dermal small vessel vasculitis and less commonly as no...

Localized gastrointestinal vasculitis is a rare condition. It may be observed as an incidental unexpected pathologic finding at the time of biopsy of an abdominal mass or may present as unexplained abdominal pain with or without unexplained lower gastrointestinal bleeding. In this report we describe a new case of localized polyarteritis nodosa with involvement of peripancreatic middle-sized blo...

OBJECTIVES To establish the clinical significance of isolated nail fold vasculitis in patients with rheumatoid arthritis (RA). METHODS Patients attending hospital with established RA were assessed by a single observer using unaided vision. Isolated nail fold vasculitis (NFV) was diagnosed as small nail edge or nail fold lesions without any evidence of other extra-articular or systemic vasculi...