Background/Purpose:
The severity of clinical features and the outcome in the different series of Henoch-Schönlein Purpura (HSP) shows great variability, probably due to selection-bias. 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:
Study of all consecutive patients classified as having HSP at a single center according to the criteria proposed by Michel et al (J Rheumatol 1992; 19: 721-8). HSP was pathologically confirmed in 110 cases by a skin biopsy showing the characteristic histological findings consistent with leukocytoclastic vasculitis. The remaining 307 patients without skin biopsy (mainly children) had typical non thrombocytopenic symmetric palpable purpura. In addition, all of them fulfilled the criteria proposed by Michel et al.
Results:
We performed a retrospective review of 417 patients (240 men/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 (n=315) were children or young patients (age <20 years) 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 HSP. 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%). When the disease was fully established, the main clinical features were skin involvement (100%), mainly palpable purpura, gastrointestinal (64.5%), joint (63.1%), renal involvement (41.2%), fever (20.4%), constitutional syndrome (2.9%) and peripheral neuropathy (1.9%). The main gastrointestinal features were the typical colicky abdominal pain (64.5%), nausea and vomiting (14.4%), melena and or rectorrhagia (12.9%), and positive stool guaiac test (10.3%). Renal involvement (41.2%), usually behaving as a mild nephropathy; nephrotic syndrome (4.8%), nephritic syndrome (2.9%) and renal insufficiency (4.8%). The main laboratory data were leukocytosis (36.7%), anemia (8.9%) and increased of serum IgA levels (31.7%). The most frequent treatments 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 346 patients (83.2%) and persistent, usually mild, nephropathy was observed in 32 cases (7.7%). Relapses were observed in 133 patients (31.9%).
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.
Disclosure:
F. Ortiz-Sanjuán,
None;
V. Calvo-Río,
None;
J. Loricera,
None;
C. Mata,
None;
L. Martín Penagos,
None;
L. Alvarez,
None;
M. C. González-Vela,
None;
D. González-Lamuño,
None;
J. Rueda-Gotor,
None;
H. Fernández-Llaca,
None;
M. A. González-López,
None;
S. Armesto,
None;
M. E. Peiró,
None;
M. Arias,
None;
M. A. Gonzalez-Gay,
None;
R. Blanco,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/henoch-schonlein-purpura-clinical-spectrum-of-the-disease-in-417-patients-from-a-single-center/