IGA VASCULITIS (HENOCH-SCHONLEIN PURPURA) NEPHRITIS AND PSORIASIS IN A CHILD: IS THERE A RELATIONSHIP?

被引:0
作者
Vaz, Ana Sofia [1 ]
Penteado, Raquel [1 ]
Cordinha, Carolina [1 ]
Carmo, Carmen [1 ]
Gomes, Clara [1 ]
机构
[1] Ctr Hosp & Univ Coimbra, Dept Pediat, Serv Pediat Ambulatoria, Unidade Nefrol Pediat, Coimbra, Portugal
来源
JORNAL BRASILEIRO DE NEFROLOGIA | 2021年 / 43卷 / 04期
关键词
Psoriasis; Purpura de Schoenlein-Henoch; Glomerulonephritis; IGA; KIDNEY-DISEASE; GLOMERULONEPHRITIS;
D O I
10.1590/2175-8239-JBN-2020-0101
中图分类号
R5 [内科学]; R69 [泌尿科学(泌尿生殖系疾病)];
学科分类号
1002 ; 100201 ;
摘要
Background: Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schonlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report: A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions: We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.
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页码:603 / 607
页数:5
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