Hypersensitivity vasculitis group (HVG)

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Since the original clinical and pathologic description of Kussmaul and Maier1 over a century ago, vasculitis has remained a formidable problem. Pre-mortem diagnosis remains notoriously difficult, treatment is still fraught with significant morbidity and mortality, and its classification remains controversial at best.

Despite these difficulties, significant advances have been made in the area of pathogenesis. Three observations are largely responsible for this added insight: (1) the elucidation of the pathogenic role of immune complexes in serum sickness;2 (2) the recognition that many connective tissue diseases, e.g., rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), which are frequently accompanied by vasculitis, are characterized by immune complexes;3 and (3) the identification of a few putative antigens, e.g., HbsAg, responsible for certain forms of arteritis.4 All of these observations have given credence to the immune complex theory of vascular inflammation. Furthermore, recent advances in the clinical laboratory have enabled clinicians to monitor immune complex levels, aiding both in diagnosis and treatment.5

This discussion deals with a subgroup of the vasculitic syndromes that have been variously termed allergic vasculitis, allergic angiitis, and leukocytoclastic vasculitis. Furthermore, other well-defined syndromes such as Henoch-Schönlein purpura and essential cryoglobulinemia have frequently been included in this classification because of certain clinical and pathologic similarities, especially the nearly universal involvement of the skin, and the presence of small vessel inflammation with varying amounts of nuclear debris (leukocytoclasis). We believe that the vascular damage induced by immune complexes is a dynamic phenomenon that frequently, however, assumes characteristic anatomic distributions with resultant clinical . . .



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