Pulmonary function abnormalities in children with Henoch-Schonlein purpura

Henoch-Schonlein purpura (HSP) is a widespread necrotizing vasculitis affec ting small vessels characterized by nonthrombocytopenic purpura, Pulmonary involvement is a rare fatal complication with diffuse alveolar haemorrhage. The objective of this study was to evaluate possible early lung function a bnormalities and to establish any relationship with the clinical activity o f the disease. Fifteen children with HSP and without clinical or radiological evidence of lung involvement underwent pulmonary function study at the onset of the dis ease, A sample of 28 subjects matched by age, height, and weight was chosen as a control group. After a mean of 21 months (range 12-43) lung function tests were repeated in 10 of the previously studied children. During the acute phase of the disease the transfer factor for carbon monoxi de, measured by steady-state (TL,COss) and single-breath (TL,COsb) methods, was found to be significantly lower in children with HSP than control subj ects. There was no significant relationship between pulmonary function test s with symptoms and signs at onset, nor was there any correlation between v ariables and serum immunoglobulin A (IgA) concentration. In all but two pat ients, clinical recovery was observed within 6 weeks from the onset of the disease, In one case relapses of purpuric skin lesions were observed during the first 3 months of follow-up, The second case had relapses of purpuric skin lesions and microscopical haematuria during the 12 months following th e onset of the disease with characteristic IgA mesangial deposition on rena l biopsy, Although the overall mean value of TL,COsb improved from baseline to the second investigation, in both patients the recurrences of clinical signs were associated,vith a slight impairment of TL,COsb at the second eva luation. These data suggest an early subclinical Lung impairment in children with He noch-Schonlein purpura during the active phase of the disease. The presence of isolated pulmonary function abnormalities was not associated with the s ubsequent development of lung disease.