title: Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center.
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.
authors.affiliation: 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.
authors.forename: Vanesa
authors.initials: V
authors.lastname: Calvo-Río
authors.forename: Javier
authors.initials: J
authors.lastname: Loricera
authors.forename: Cristina
authors.initials: C
authors.lastname: Mata
authors.forename: Luis
authors.initials: L
authors.lastname: Martín
authors.forename: Francisco
authors.initials: F
authors.lastname: Ortiz-Sanjuán
authors.forename: Lino
authors.initials: L
authors.lastname: Alvarez
authors.forename: M Carmen
authors.initials: MC
authors.lastname: González-Vela
authors.forename: Domingo
authors.initials: D
authors.lastname: González-Lamuño
authors.forename: Javier
authors.initials: J
authors.lastname: Rueda-Gotor
authors.forename: Héctor
authors.initials: H
authors.lastname: Fernández-Llaca
authors.forename: Marcos A
authors.initials: MA
authors.lastname: González-López
authors.forename: Susana
authors.initials: S
authors.lastname: Armesto
authors.forename: Enriqueta
authors.initials: E
authors.lastname: Peiró
authors.forename: Manuel
authors.initials: M
authors.lastname: Arias
authors.forename: Miguel A
authors.initials: MA
authors.lastname: González-Gay
authors.forename: Ricardo
authors.initials: R
authors.lastname: Blanco
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones; D017668:Age of Onset; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006403:Hematologic Tests; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D008297:Male; D012189:Retrospective Studies; D012621:Seasons; D013030:Spain
chemical_list: D000305:Adrenal Cortex Hormones