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      Henoch-Schönlein Purpura in Northern Spain : Clinical Spectrum of the Disease in 417 Patients From a Single Center

      research-article
      , MD, , MD, , MD, , MD, , MD, , MD, , MD, PhD, , MD, PhD, , MD, , MD, , MD, PhD, , MD, PhD, , MD, , MD, PhD, , MD, PhD * , , MD, PhD *
      Medicine
      Lippincott Williams & Wilkins
      ACR = American College of Rheumatology, ANA = antinuclear antibodies, ANCAs = antineutrophil cytoplasmic antibodies, ESR = erythrocyte sedimentation rate, HIV = human immunodeficiency virus, HSP = Henoch-Schönlein purpura, IQR = interquartile range, RF = rheumatoid factor, SD = standard deviation, URTI = upper respiratory tract infection

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          Abstract

          The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3–20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2–38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).

          In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement.

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          Most cited references34

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          Henoch-Schönlein Purpura in adults: outcome and prognostic factors.

          Henoch-Schönlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. A cohort of 250 adults suffering HSP was retrospectively analyzed for a median follow-up period of 14.8 yr. All patients had biopsies consistent with HSP (predominant IgA mesangial deposits) associated with purpura, bowel angina, and/or abdominal pain. At presentation, palpable purpura was present in 96% of patients, and arthritis was reported in 61%, and gastrointestinal involvement in 48%. Thirty-two percent of the patients showed renal insufficiency (Creatinine clearance [CrCl] <50 ml/min), usually associated with proteinuria (99%) and/or hematuria (93%). Endocapillary glomerulonephritis was the most frequent lesion on renal biopsy (61%). At the end of follow-up, patient survival was only 74%. The first cause of death was carcinoma (most of them of respiratory or digestive tract). Regarding renal function, 11% of patients reached end-stage renal failure, 13% exhibited severe renal failure (CrCl <30 ml/min), and 14% moderate renal insufficiency (CrCl <50 ml/min). Clinical remission defined as the absence of proteinuria, hematuria, and a normal renal function was achieved in only 20%. This is a retrospective study; therefore, it is not possible to demonstrate any steroid and/or cyclophosphamide efficacy in diminishing the incidence of renal insufficiency. Multivariate analysis demonstrated that renal function impairment and proteinuria level at presentation and, on renal biopsy, the degree of interstitial fibrosis, percentage of sclerotic glomeruli, and presence of glomeruli with fibrinoid necrosis were associated with a poor renal prognosis. The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.
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            The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura.

            Criteria for identifying Henoch-Schönlein Purpura (HSP) and distinguishing HSP from other forms of systemic arteritis were developed by comparing the manifestations in 85 patients who had HSP with those of 722 control patients with other forms of vasculitis. By the traditional format of choosing different combinations of candidate criteria and comparing the combinations for their ability to separate HSP cases from controls, 4 criteria were identified: age less than or equal to 20 years at disease onset, palpable purpura, acute abdominal pain, and biopsy showing granulocytes in the walls of small arterioles or venules. The presence of any 2 or more of these criteria distinguish HSP from other forms of vasculitis with a sensitivity of 87.1% and a specificity of 87.7%. The criteria selected by a classification tree method were similar: palpable purpura, age less than or equal to 20 years at disease onset, biopsy showing granulocytes around arterioles or venules, and gastrointestinal bleeding. These were able to distinguish HSP from other forms of vasculitis with a sensitivity of 89.4% and a specificity of 88.1%.
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              Clinical update: Henoch-Schönlein purpura.

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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MD
                Medicine
                Lippincott Williams & Wilkins
                0025-7974
                1536-5964
                March 2014
                04 March 2014
                : 93
                : 2
                : 106-113
                Affiliations
                [1]From Divisions of Rheumatology (VC-R, JL, CM, FO-S, JR-G, EP, MAG-G, RB), Nephrology (LM, MA), Pediatrics (LA, DG-L), Pathology (MCG-V), and Dermatology (HF-L, MAG-L, SA), Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
                Author notes
                Correspondence: Miguel A. González-Gay or Ricardo Blanco, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, Avda. Valdecilla s/n., ES-39008, Santander, Spain (e-mail: miguelaggay@ 123456hotmail.com or rblanco@ 123456humv.es ).
                Article
                MD130059 00006
                10.1097/MD.0000000000000019
                4616305
                24646467
                38e307db-1ec7-4aba-b7db-8c4af751ca61
                Copyright © 2014 by Lippincott Williams & Wilkins
                History
                Categories
                Original Study

                acr = american college of rheumatology,ana = antinuclear antibodies,ancas = antineutrophil cytoplasmic antibodies,esr = erythrocyte sedimentation rate,hiv = human immunodeficiency virus,hsp = henoch-schönlein purpura,iqr = interquartile range,rf = rheumatoid factor,sd = standard deviation,urti = upper respiratory tract infection

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