Objective To describe the extrarenal symptoms and clinical course of Henoch–Schönlein purpura (HSP).
Design A prospective national multicentre trial with 6-month follow-up.
Patients A total of 223 newly diagnosed paediatric HSP patients.
Results Purpura was the initial symptom in 73% of the patients and was preceded by joint or gastrointestinal manifestations in the rest by a mean of 4 days. Joint symptoms, abdominal pain, melena, nephritis and recurrences occurred in 90%, 57%, 8%, 46% and 25% of the patients, respectively. Orchitis affected 17/122 (14%) of the boys. Seven patients developed protein-losing enteropathy characterised by abdominal pain, oedema and serum albumin under 30 g/l, and an additional 49 patients had subnormal albumin levels without any proteinuria. Positive fecal occult blood (26/117, 22%) and α1-antitrypsin (7/77, 9%) suggested mucosal injury even in the patients without gastrointestinal symptoms. HSP was often preceded by various bacterial, especially streptococcal (36%) and viral infections. Previous streptococcal infection did not induce changes in the level of complement component C3. Recurrences were more frequent in patients >8 years of age (OR 3.7, CI 2.0 to 7.0, p<0.001) and in patients with nephritis (OR 4.6, CI 2.3 to 8.9, p<0.001). Patients with severe HSP nephritis had more extrarenal symptoms up to 6 months. There was no difference in the clinical course between the prednisone-treated and non-treated patients during the 6-month follow-up.
Conclusions Serum albumin is often low in HSP patients without proteinuria, due to protein loss via the intestine. Although corticosteroids alleviate the symptoms, they seem not to alter the clinical course of HSP during 6 months of follow-up.
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Funding OJ and JR received a grant from the Alma and K A Snellman Foundation, Oulu, Finland and from the Foundation for Paediatric Research for writing this report.
Competing interests None.
Provenance and peer review Not commissioned; externally peer reviewed.
Ethics approval This study was conducted with the approval of the ethics committees of each study centre (the university hospitals of Helsinki, Kuopio, Oulu, Tampere and Turku).
Patient consent Obtained.