Complement activation in Henoch-Schönlein purpura

Smith, Graham C.; Davidson, Joyce; Hughes, David; Holme, E; Beattie, Tom F.

doi:10.1007/s004670050320

Cited by 32 publications

(29 citation statements)

References 15 publications

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“…Finally, serum SC5b-9 has been shown to correlate with the disease activity in patients with HSP [11]. In contrast to these reports, a study of 64 children with acute HSP failed to demonstrate any signi®cant dierence in the levels of C1r:C1s:C1 esterase inhibitor, C3bP and C5b-9 complexes between the patients and the controls [20]. Even though several studies have demonstrated activation of C3 and C4 in HSP, they failed to ®nd a corresponding reduction in serum concentrations of C3 and C4, which remained normal or elevated.…”

Section: Discussionmentioning

confidence: 77%

Low serum C3, leukopenia, and thrombocytopenia: unusual features of Henoch-Schonlein purpura

Krause

Davidovits

et al. 1999

European Journal of Pediatrics

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Henoch-Schonlein purpura (HSP) affects predominantly the skin, joints, gastrointestinal tract and kidney. Although the pathogenesis is probably of immune origin and complement activation is thought to play a role, laboratory findings including the serum level of the complement components are usually normal. We present a patient with a severe form of HSP nephritis who had unusual laboratory findings of a low level of C3, mild leukopenia and thrombocytopenia. These findings may further support the importance of complement activation in the pathogenesis of HSP.

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Section: Discussionmentioning

confidence: 77%

Low serum C3, leukopenia, and thrombocytopenia: unusual features of Henoch-Schonlein purpura

Krause

Davidovits

et al. 1999

European Journal of Pediatrics

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“…However, an activation of the complement occurs since degradation products have been demonstrated in plasma and glomeruli [20, 22]. However, the role of this activation remains unclear.…”

Section: Discussionmentioning

confidence: 99%

Serum IgA/C3 Ratio May Be a Useful Marker of Disease Activity in Severe Henoch-Schönlein Nephritis

et al. 2005

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Background/Aims: This study was designed to investigate whether the serum IgA/C3 ratio can be a serologic marker of disease activity in children with severe Henoch-Schönlein nephritis (HSN). Methods: Twelve HSN patients who were treated with steroids and cyclosporine were examined. The levels of serum IgA and C3 were measured using an international reference preparation (IFCC/CRM470) and a renal biopsy was performed in all patients before and after therapy. After therapy, patients were divided into 3 groups: complete remission (n = 6, group I), mild urinary abnormalities (n = 3, group II), and active renal disease (n = 3, group III). Results: The serum IgA/C3 ratio decreased significantly in groups I and II after therapy (2.62 ± 0.82 vs. 2.02 ± 0.52, p = 0.02), whereas the ratio in group III increased, although it was not statistically significant (2.13 ± 0.93 vs. 4.67 ± 1.71, p = 0.25). A follow-up renal biopsy revealed that the activity index was reduced in groups I and II (7.0 ± 2.4 vs. 3.6 ± 1.6, p = 0.016), and not changed in group III (7.3 ± 2.1 vs. 9.3 ± 2.5, p = 0.25). The activity index at a follow-up renal biopsy correlated positively with the changes of the serum IgA/C3 ratio: posttherapy activity index = 1.20 × ΔIgA/C3 + 4.78 (r = 0.635, p = 0.027); where ΔIgA/C3 is posttherapy IgA/C3 – pretherapy IgA/C3. Conclusion: These findings suggest that the serum IgA/C3 ratio may be a useful marker to predict disease activity and histologic severity in HSN.

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“…However, an activation of the complement cascade does occur since degradation products have been demonstrated in plasma and glomeruli [15,40,76]. The role of this activation remains however unclear.…”

Section: Complementmentioning

confidence: 98%

“…The role of this activation remains however unclear. Results of studies attempting to correlate the level of complement split products in the blood with clinical signs [15,40,76] or the glomerular deposition of various complement factors and histological lesions are contradictory [26].…”

Section: Complementmentioning

confidence: 99%