Henoch–Schönlein purpura with hypocomplementemia

Lin, Qian; Yue, Min; Li, Yanhong; Zhu, Yun; Song, Xiaoxiang; Xu, Quangang; Wang, Lifeng; Cheng, Jin; Qihua, Feng; Li, Xiaozhong

doi:10.1007/s00467-011-2070-z

Cited by 38 publications

(30 citation statements)

References 18 publications

(42 reference statements)

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“…HSP has also been regarded as an immune complex disease; however, hypocomplementemia with low serum or plasma C3 and/or C4 levels was not commonly shown and usually transient in patients with acute HSP [23,24]. In the present study, the results showed the serum levels of C3 and C4 in all 30 HSP children at both acute and convalescent stages were within normal ranges.…”

Section: Discussionsupporting

confidence: 42%

The Interaction between Circulating Complement Proteins and Cutaneous Microvascular Endothelial Cells in the Development of Childhood Henoch-Schönlein Purpura

et al. 2015

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ObjectiveIn addition to IgA, the deposition of complement (C)3 in dermal vessels is commonly found in Henoch-Schönlein purpura (HSP). The aim of this study is to elucidate the role of circulating complement proteins in the pathogenesis of childhood HSP.MethodsPlasma levels of C3a, C4a, C5a, and Bb in 30 HSP patients and 30 healthy controls were detected by enzyme-linked immunosorbent assay (ELISA). The expression of C3a receptor (C3aR), C5a receptor (CD88), E-selectin, intercellular adhesion molecule 1 (ICAM-1), C3, C5, interleukin (IL)-8, monocyte chemotactic protein (MCP)-1, and RANTES by human dermal microvascular endothelial cells (HMVEC-d) was evaluated either by flow cytometry or by ELISA.ResultsAt the acute stage, HSP patients had higher plasma levels of C3a (359.5 ± 115.3 vs. 183.3 ± 94.1 ng/ml, p < 0.0001), C5a (181.4 ± 86.1 vs. 33.7 ± 26.3 ng/ml, p < 0.0001), and Bb (3.7 ± 2.6 vs. 1.0 ± 0.6 μg/ml, p < 0.0001), but not C4a than healthy controls. Although HSP patient-derived acute phase plasma did not alter the presentation of C3aR and CD88 on HMVEC-d, it enhanced the production of endothelial C3 and C5. Moreover, C5a was shown in vitro to up-regulate the expression of IL-8, MCP-1, E-selectin, and ICAM-1 by HMVEC-d with a dose-dependent manner.ConclusionIn HSP, the activation of the complement system in part through the alternative pathway may have resulted in increased plasma levels of C3a and C5a, which, especially C5a, may play a role in the disease pathogenesis by activating endothelium of cutaneous small vessels.

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Section: Discussionsupporting

confidence: 42%

The Interaction between Circulating Complement Proteins and Cutaneous Microvascular Endothelial Cells in the Development of Childhood Henoch-Schönlein Purpura

et al. 2015

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“…17 13.9% (47/338) of HenochSchönlein purpura patients was reported to present with low C4 levels. 32 In the present study, a high prevalence (19.34%) of low C4 levels was also detected in the IgAN patients. C4-mediated lectin pathway is involved in IgAN providing evidences as follows: in IgAN, polymeric IgA could bind to the mannan-binding lectin domain to activate lectin pathway resulting in renal C4 deposition.…”

Section: Discussionmentioning

confidence: 56%

Clinical characteristics of IgA nephropathy associated with low complement 4 levels

Zhu

Lin

et al. 2014

Renal Failure

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Objective: C4 deficiency is the most commonly inherited immune disorder in human. The present study investigated the characteristics of the IgAN patients with low serum C4 levels.Methods: We performed a prospective observational study. Clinical as well as histopathologic parameters were assessed. A Kaplan-Meier survival analysis was performed concerning the primary outcome defined as the serum creatinine increased 1.5-fold from baseline. The prognostic significances of clinical and histopathologic parameters were determined using Cox proportional hazards models. Results: Five-hundred twelve biopsy proven IgAN cases were available for analysis with a median follow-up of 38.4 months. Ninety-nine cases (19.34%) presented with low C4 levels (LowC4 group) and the other 413 cases did not (NlowC4 group). At the time of renal biopsy, renal injury was lighter in the LowC4 group compared with the NlowC4 group. Renal C4 deposition was significantly decreased while IgM deposition was increased in the LowC4 group. A correlation analysis shows that lower C4 levels were associated with better renal presentations at biopsy. However, the risk of developing the primary outcome was significantly greater in those with low C4 levels. Specifically, during the follow-up period, the risk of developing primary outcome was nearly ten folds higher in those with low C4, compared to those without low C4. Conclusion: There is a high prevalence of low C4 levels in IgAN patients. These patients with low C4 levels exhibited better renal presentations at the time of renal biopsy, whereas might be associated with a poor prognosis.

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“…Some children with HSP present with accompanying hypocomplementaemia, although these children do not necessarily present with nephropathy; thus, serum complement level decrease has no relevance to HSP severity or the development of HSPN . However, the hypocomplementaemia seen in our patient improved with the amelioration of clinical symptoms, including proteinuria and general oedema.…”

Section: Discussionmentioning

confidence: 56%

Local leukocyte proliferation as a target for cyclophosphamide in the treatment of Henoch‐Schönlein purpura nephritis grade VI

et al. 2015

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Henoch-Schönlein purpura nephritis (HSPN) is one of the most common types of chronic glomerulonephritis in children; however, there have been few reports on the pathogenesis and management of grade VI HSPN. We present the case of a 6-year-old boy with grade VI HSPN accompanied by severe nephrotic syndrome and hypocomplementaemia. Immunohistological studies revealed profound glomerular accumulation of CD45- and CD68-positive inflammatory cells. Moreover, some cells expressed the proliferating marker proliferating cell nuclear antigen. His proteinuria and general oedema persisted despite repeated high-dose steroid therapy; however, these clinical symptoms immediately improved after beginning treatment with cyclophosphamide (CyP). Grade VI HSPN was successfully treated with steroids and immunosuppressants. Among immunosuppressive drugs, CyP was considered the most effective.

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Henoch–Schönlein purpura with hypocomplementemia

Abstract: Hypocomplementemia associated with HSP is a transient phenomenon. The incidence of significant sequelae such as HSPN between patients with and without hypocomplementemia does not differ.

Cited by 38 publications

References 18 publications

The Interaction between Circulating Complement Proteins and Cutaneous Microvascular Endothelial Cells in the Development of Childhood Henoch-Schönlein Purpura

The Interaction between Circulating Complement Proteins and Cutaneous Microvascular Endothelial Cells in the Development of Childhood Henoch-Schönlein Purpura

Clinical characteristics of IgA nephropathy associated with low complement 4 levels

Local leukocyte proliferation as a target for cyclophosphamide in the treatment of Henoch‐Schönlein purpura nephritis grade VI

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