title: [Childhood-onset systemic polyarteritis nodosa and systemic lupus erythematosus: an overlap syndrome?]
abstract: We described herein a patient who presented an overlap syndrome of childhood-onset systemic polyarteritis nodosa (c-PAN) and childhood-onset systemic lupus erythematosus (c-SLE). A 9-year-old girl presented tender subcutaneous nodules on feet, arterial hypertension, right hemiplegia and dysarthric speech. She was hospitalized due to stroke and left foot drop. Brain computer tomography showed ischemic stroke. Magnetic resonance angiography revealed stenosis in the middle cerebral and internal carotid arteries. Electroneuromyography identified a mononeuropathy of left posterior tibial nerve and she fulfilled the c-PAN validated criteria. She was treated with intravenous methylprednisolone pulse therapy followed by prednisone, that was progressively tapered, six months of intravenous cyclophosphamide and after that she received azathioprine for 19 months. At the age of 14 years and 9 months, she presented malar rash, photosensitivity, edema in lower limbs and arterial hypertension. The proteinuria was 1.7g/day. Antinuclear antibodies (ANA) were 1/1280 (homogeneous nuclear pattern) and anti-dsDNA antibodies were positive. Renal biopsy showed focal proliferative and membranous glomerulonephritis. Therefore, she fulfilled the American College of Rheumatology classification criteria for SLE and she was treated with prednisone, hydroxychloroquine and mycophenolate mofetil. In conclusion, we described herein a possible overlap syndrome of two autoimmune diseases, where c-PAN occurred five years before the c-SLE diagnosis.
authors.affiliation: Unidade de Reumatologia Pediátrica do Departamento de Pediatria da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brasil.
authors.forename: Victor L S
authors.initials: VL
authors.lastname: Marques
authors.affiliation: Unidade de Reumatologia Pediátrica do Departamento de Pediatria da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brasil; Unidade de Reumatologia Pediátrica do Departamento de Pediatria da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brasil.
authors.forename: Andressa
authors.initials: A
authors.lastname: Guariento
authors.affiliation: Unidade de Reumatologia Pediátrica do Departamento de Pediatria da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brasil.
authors.forename: Marlise S M
authors.initials: MS
authors.lastname: Simões
authors.affiliation: Unidade de Reumatologia Pediátrica do Departamento de Pediatria da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brasil.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Blay
authors.affiliation: Unidade de Reumatologia Pediátrica do Departamento de Pediatria da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brasil.
authors.forename: Ana Paola N
authors.initials: AP
authors.lastname: Lotito
authors.affiliation: Unidade de Reumatologia Pediátrica do Departamento de Pediatria da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brasil. Electronic address: clovisaasilva@gmail.com.
authors.forename: Clovis A
authors.initials: CA
authors.lastname: Silva
keywords: Childhood‐onset systemic polyarteritis nodosa; Childhood‐systemic lupus erythematosus; Lúpus eritematoso sistêmico de início na infância; Overlap syndrome; Poliarterite nodosa sistêmica de início na infância; Síndrome de sobreposição; Vasculite; Vasculitis