Letter to the Editor
Neonatal lupus syndrome induced by anti-ribonucleoprotein antibodies
Síndrome de lupus neonatal inducido por anticuerpos antirribonucleoproteína
Ana Mercedes Victoria-Martínez
, Altea Esteve-Martínez, Violeta Zaragoza-Ninet, Isabel Febrer-Bosch
Corresponding author
Consorcio Hospital General Universitario de Valencia, Valencia, Spain
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Clinically, NLE may present itself with cutaneous, cardiac, hepatobiliary and/or haematologic involvement. Most cases are associated with anti-Ro (SSA) and anti-La (SSB) antibodies, and less frequently cases have been described associated with antirribonucleoproteina antibodies (anti-RNP).</p><p id="par0010" class="elsevierStylePara elsevierViewall">Here we expose the clinical case of a 2-month-old male who came to our consultation because of a generalised rash that had been developing over one month, and had not improved despite treatment with topical corticosteroids. There were no other findings of interest. The physical examination showed erythematous annular and polycyclic lesions predominantly in the scalp, face and trunk, some with necrotic core with a tendency to leave a depressed central scar (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). No injuries were photoconditioned.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The pregnancy had been monitored closely because the mother was diagnosed with a mixed connective tissue disease (MCTD).</p><p id="par0020" class="elsevierStylePara elsevierViewall">Given the cutaneous symptoms presented by the child and the family history cited, suspected NLE was diagnosed. A complete analytical test was requested that included an autoimmunity profile, which showed an increase in transaminases and ANA at titres of 1:1280, positive ENA with positive anti-U1 RNP (same autoantibodies profile as the mother). An abdominal ultrasound was conducted in addition to an electrocardiogram and an echocardiogram, which were all normal.</p><p id="par0025" class="elsevierStylePara elsevierViewall">With all these data, the NLE diagnosis associated with anti-RNP antibodies with anti-Ro and anti-LA negatives was confirmed.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Given the lack of response to treatment with topical corticosteroids another treatment was applied with prednisolone and photoprotection. Clinically his cutaneous lesions presented an improvement at 2 weeks. At 5 months, virtually all lesions had disappeared, although residual scar lesions on the scalp and trunk persisted, but there was a normalisation of liver enzymes and autoantibodies.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The NLE is a rare autoimmune disorder that affects one in every 12,000–20,000 live births. It is due to the transplacental passage of maternal IgG autoantibodies, anti-Ro (SSA) and anti-La (SSB) antibodies, and more rarely anti-U1-RNP. Clinically, NLE may present itself with cutaneous, cardiac, hepatobiliary and/or haematologic involvement. There may be a single alteration or a combination of several of them.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2</span></a> 50% of mothers with children who develop NLE are diagnosed with a connective tissue disease, most frequently Sjögren's syndrome, systemic lupus erythematosus or MCTD. The other 50% are asymptomatic but do eventually develop an autoimmune disease in the future.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,4</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Skin lesions are 3 times more common in women, are usually asymptomatic and have been linked to sun exposure. They are characterised by macules or annular or polycyclic erythematous plaques that typically affect the face, and may have a periorbital location, giving an appearance of a mask. Lesions are resolved spontaneously at 6–8 months, coinciding with the clearance of maternal antibodies. The NLE diagnosis is mainly clinical and serological. The histopathological study of the skin has a confirmatory function. NLE skin treatment is simple and conservative. Photoprotection and low potency topical corticosteroids are recommended.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Cardiac involvement consists of a complete heart block that can be permanent, requiring the placement of a pacemaker. This complication marks the prognosis, associating a mortality of up to 20%.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Hepatobiliary disorders occur in 10% of cases and the most common is an increase of transient transaminase or a pattern of cholestasis. The predominant haematologic disorder is a transient and benign thrombocytopenia observed in 40% of cases.</p><p id="par0055" class="elsevierStylePara elsevierViewall">Ultrasound monitoring should be performed for pregnant women with positive anti-Ro antibodies and preventive treatment should be considered with oral corticosteroids or hydroxychloroquine.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,6</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Prior to our patient, 16 cases of anti-RNP positive and anti-Ro negative NLE had been reported in the medical literature.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> No differences were observed in terms of sexes, unlike classic NLE, which is more common in women. Furthermore, no cases with cardiac involvement had been described. Skin symptoms are the same in both types with the anti-RNP subtype less prone to photosensitivity and having a greater frequency of atrophic scars.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> In all reported cases of the anti-RNP subtype, except one,<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">9,10</span></a> the mother was diagnosed with a connective tissue disease, with the MCTD and systemic lupus erythematosus predominating.</p><p id="par0065" class="elsevierStylePara elsevierViewall">The NLE with anti-RNP positive antibodies and anti-Ro/La negatives, is a subtype that has been differentiated from the classic NLE, especially in that cutaneous involvement has been observed, but, in no cases, until now, has cardiac involvement, such that it seems to have a more benign behaviour.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Victoria-Martínez AM, Esteve-Martínez A, Zaragoza-Ninet V, Febrer-Bosch I. Síndrome de lupus neonatal inducido por anticuerpos antirribonucleoproteína. Med Clin (Barc). 2016;146:e21–e22.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 662 "Ancho" => 995 "Tamanyo" => 76356 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Erythematous annular lesions on the trunk are observed, with atrophic central area.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The clinical spectrum of neonatal lupus" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "L.A. 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