Background The severity of clinical features and the outcome in the different series of patients with Henoch-Schönlein Purpura (HSP) shows great variability, probably due to selection-bias.
Objectives Our aim was 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.
Methods For this purpose, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al.1
Results From this series of 417 patients, 240 were men and 177 women with a median age at the time of disease diagnosis of 7.5 years (interquartile range-IQR: 5.3-20.1). Three-quarters of them were children or young people 20 years old and younger (n=315) and a quarter (n=102) were adults. 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 the cases, mainly purpuric skin lesion, was the most common manifestations when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%).The main laboratory data were leukocytosis (36.7%), anemia (8.9%) and increased of serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal anti-inflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 (IQR: 2-38) months, complete recovery was observed in most cases (n=346; 83.2%) while persistent, usually mild, nephropathy was observed in 32 (7.7%) cases. Relapses were observed in almost a third of patients (n=133; 31.9%).
Conclusions 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 mostly on renal involvement.
Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensiviti vasculitis and Henoch-Schönlein purpura: a comparison between the two disorders. J Rheumatol. 1992; 19:721-728.
Acknowledgements This study was supported by a grant from “Fondo de Investigaciones Sanitarias” PI12/00193 (Spain). This work was also partially supported by RETICS Programs, RD08/0075 (RIER) and RD12/0009/0013 from “Instituto de Salud Carlos III” (ISCIII) (Spain).
Disclosure of Interest None declared