Henoch-Schönlein Purpura in Children from Northwestern Spain: A 20-Year Epidemiologic and Clinical Study

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Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by purpuric skin lesions unrelated to any underlying coagulopathy, gastrointestinal manifestations, arthritis, and renal involvement (25,51). Infiltration of small blood vessels with polymorphonuclear leukocytes and the presence of leukocytoclasia are typical pathologic findings in this vasculitis. In addition, immunofluorescence staining of tissues usually discloses the presence of immunoglobulin A (IgA)-dominant immune deposits in the wall of the small vessels and in the renal glomeruli (21,30).
Henoch-Schönlein purpura is mainly a childhood disease: it is the most common type of vasculitis in children and an infrequent condition in adults (26). The first description of this syndrome was given by Heberden (27), who reported a child with joint pain and painful subcutaneous edema, abdominal pain, vomiting, bloody stools and urine, and “bloody points” over the skin of his legs. However, the names of Schönlein and Henoch are most commonly used as the designation of the syndrome. Initially, Schönlein (52) described the association between arthralgia and purpuric cutaneous lesions in a child using the term “purpura rheumatica.” Some years later, his pupil Henoch (28) described a syndrome of purpura, severe abdominal colic, and melena. A few years later, Henoch referred to nephritis as a complication of this syndrome (29).
As recently pointed out by Stone (56), there has been a relative paucity of clinical and epidemiologic studies on HSP in children over the past few years (50). Of note, the long-term morbidity and mortality of HSP are predominantly attributable to renal involvement. In some studies HSP in children has been reported to account for 5%–15% of patients entering end-stage renal failure (10,37). Most of these studies were done on series of selected patient populations with kidney dysfunction attended in reference centers. Two unselected series (33,55), however, suggested a good prognosis for HSP nephritis in children—a more optimistic outcome than most published series based on more selected groups of patients. To investigate this syndrome further, we examined the incidence, clinical spectrum, and prognosis of HSP in an unselected population of children diagnosed at the single reference hospital for a defined population in northwestern Spain over a 20-year period. Special interest was focused on the outcome of these patients and, in particular, on the risk factors implicated in the development of permanent renal damage.
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