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ISSN 1405-1699 (Print)
Órgano Oficial del Colegio Mexicano de Alergia, Asma e Inmunología Pediátrica y de la Asociación Latinoamericana de Pediatría

2000, Number 1

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Alerg Asma Inmunol Pediatr 2000; 9 (1)

Anti SSA/Ro and SSB/La antibodies in neonatal lupus. Case report and literature review

Rosiles SG, Hernández BV, Correa BY, Almendarez FC, Berrón PR

Language: Spanish
References: 27
Page: 16-20
PDF size: 400.15 Kb.


ABSTRACT

Introduction: The neonatal lupus erythematosus is a disease primarily characterized by cardiac and/or cutaneus involvement. Liver and haematological involvement occur in a few cases and these are normally considered to be mild and transient complications. The disease is suspected to be caused by transplacental passage of maternal autoantibodies, primarily anti- Ro/SSA and anti La/SSB, from a mother with systemic lupus erytematosus. Anti-Ro/SSA antibodies are antinuclear antibodies most commonly found in everyday clinical practice and are closely associated with neonatal lupus. They play a pathogenic role in a variety clinical manifestation, including skin lesions and heart block. Case report: A 15 days old boy he was born with a the discoid erytematous rash located in the face and thorax. He was the second child; familiar history were not important. On examination. Eutrophic patient with discoid lesions that are plaquenlike, elevated areas that when active are erytematous with necrotic center, in face and thorax. Cardiopulmonary and gastrointestinal normal. Immunologic evaluation showed ANA (++), anticardiolipin antibodies (-) anti RO/SSA antibodies 89.9 IU (++), and seric complement 149 IU/mL immune complexes (I.C) 2.4 IU immunoglobulin levels normal. Laboratory studies (only for) showed to thrombocitosis. Hepatic and renal function were normal, with not sign is cardiomyopaty. The diagnosis of neonatal lupus eritematosus was stabilish and star therapy with deflazacort 2 mg/kg/day. The skin biopsy reported epidermal atrophy, interphase injury and necrotic queratinocites, and plasmocitary infiltrate. Twenty eight days later clinical improvement and immunological evaluation ANA (++) Anti nDNA (-) I.C 1.5 µ/mL CH50 259 IU/mL artiRO/SSA 65 IU. Now day the patient is 3 months old, clinical improvement only patch hyperchromic residual, not with neonatal lupus erythematosus with no cardiac involvement. Discussion: congenital heart block and the transient rash of the neonatal lupus are strongly associated with maternal antibodies to SSA/Ro and SSB/LA proteins the precise mechanism by which these antibodies organ specific injury is not yet defined. Thus interaction between the anti SSA/RO antibodies and Ultraviolet Ray irradiated keratinocitos may induced the skin lesions throungh a cytotoxic mechanism.


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