[Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma.]

Autor:	Couto SB; Unidade de Nefrologia Pediátrica do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brasil., Sallum AM; Unidade de Nefrologia Pediátrica do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brasil., Henriques LS; Unidade de Nefrologia Pediátrica do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brasil., Malheiros DM; Departamento de Patologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brasil., Silva CA; Departamento de Patologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brasil., Vaisbich MH; Unidade de Nefrologia Pediátrica do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brasil. Electronic address: vaisbich@terra.com.br.
Jazyk:	portugalština
Zdroj:	Revista brasileira de reumatologia [Rev Bras Reumatol] 2014 Oct 22. Date of Electronic Publication: 2014 Oct 22.
DOI:	10.1016/j.rbr.2014.08.005
Abstrakt:	Renal involvement occurs in 1%-12% in juvenile systemic sclerosis (JSSc) patients, mainly with arterial hypertension, proteinuria and scleroderma renal crisis. We report herein a patient who presented nephrotic syndrome (NS) as the first manifestation of JSSc with focal segmental glomerulosclerosis (FSGS). A female patient presented steroid-sensitive NS at the age of 12 years. At 14 years, she had orbital and lower limbs edema, arterial hypertension, sclerodactyly and proximal skin sclerosis. Moderate capillary dilation and mild focal devascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern). Percutaneous renal biopsy guided by ultrasound revealed focal segmental glomerulosclerosis and direct immunofluorescence were negative. Therefore, she fullfilled the provisional classification criteria for JSSc. Patient was treated with oral 25-hydroxyvitamin D (800 IU/day), methotrexate (0.5mg/kg/week) and amlodipin (0.15 mg/kg). Prednisone (60 mg/m 2 /day) was administered for 4 consecutive weeks, followed by alternate-day (40mg/m 2 ) for 2 consecutive months, with tapering for 4 months and then stopping this medication. Currently she is being treated with methotrexate 15 mg/week, without edema and proteinuria. In conclusion, we reported a rare case of NS with FSGS as the first manifestation of scleroderma. Therefore, renal biopsy is mandatory in JSSc patients with sustained proteinuria or NS. (Copyright © 2014 Elsevier Editora Ltda. All rights reserved.)
Databáze:	MEDLINE
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