Case report
Severe and life-threatening onset of systemic lupus erythematosus
Una presentación grave y potencialmente fatal de lupus eritematoso sistémico
Ana Martinsa,b,
, Sofia Pimentaa,b, Daniela Oliveiraa,b, Frederico Martinsc, Beatriz Samõesd, Lúcia Costaa
Autor para correspondencia
a Department of Rheumatology, Centro Hospitalar Universitário de São João, Porto, Portugal
b Department of Medicine, Faculdade de Medicina da Universidade do Porto, Porto, Portugal
c Department of Rheumatology, Hospital de Faro, CHA, Faro, Portugal
d Department of Rheumatology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
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array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:4 [ 0 => array:3 [ "entidad" => "Department of Rheumatology, Centro Hospitalar Universitário de São João, Porto, Portugal" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Medicine, Faculdade de Medicina da Universidade do Porto, Porto, Portugal" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Department of Rheumatology, Hospital de Faro, CHA, Faro, Portugal" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Department of Rheumatology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal" "etiqueta" => "d" "identificador" => "aff0020" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Una presentación grave y potencialmente fatal de lupus eritematoso sistémico" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Macrophage activation syndrome (MAS) is a underrecognized and potentially life-threatening complication of rheumatic diseases characterized by fever, pancytopenia, liver insufficiency, coagulopathy, and neurologic symptoms<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> and is thought to be caused by uncontrolled activation and proliferation of T lymphocytes and macrophages, leading to widespread hemophagocytosis and cytokine overproduction.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical observation</span><p id="par0010" class="elsevierStylePara elsevierViewall">A previously healthy 20-year-old female presented with daily fever in the last 2 weeks, fatigue, anorexia, weight loss, polyarthralgia of the hands and dry mouth. She denied symptoms suggestive of infection, night sweats or swelling of the lymph nodes. No family history for rheumatic disease. Physical examination revealed normal vital signs except high temperature (38.8<span class="elsevierStyleHsp" style=""></span>°C), pallor of the skin, mild tenderness at proximal interphalangeal joints without swelling or deformities. Cardiopulmonary, abdominal and neurological examination was normal. Small red and painful patches were observed on her fingers. No axillary, cervical and inguinal adenopathy or peripheral edema were noticed. Laboratory workup showed pancytopenia (Hb 8.4<span class="elsevierStyleHsp" style=""></span>g/dL, WBC 1630/μL, platelet 108,000/μL), normal ESR and CRP, normal renal function, mild hypoalbuminemia (36.1<span class="elsevierStyleHsp" style=""></span>g/L), high AST and ALT (98 and 81<span class="elsevierStyleHsp" style=""></span>U/L, respectively), high ferritin (5075<span class="elsevierStyleHsp" style=""></span>ng/mL) and hypertriglyceridemia (357<span class="elsevierStyleHsp" style=""></span>mg/dL). High lactate dehydrogenase (LDH 1095<span class="elsevierStyleHsp" style=""></span>U/L), low haptoglobin (<8<span class="elsevierStyleHsp" style=""></span>mg/dL), positive direct coombs test, absence of schistocytes on the peripheral blood smear and reticulocytopenia were also observed. Coagulation tests showed normal prothrombin and activated partial thromboplastin time and hypofibrinogenemia (150<span class="elsevierStyleHsp" style=""></span>mg/dL). Urine analysis was normal. Blood cultures and serological tests for infectious diseases, namely for cytomegalovirus and epstein barr virus, were negative. Autoimmune workup showed positive ANA (1:1280, homogenous pattern), anti-dsDNA (>800<span class="elsevierStyleHsp" style=""></span>IU/mL), anti-Sm, anti-SS-A and anti-nucleosome antibodies and low complement levels (C3 54<span class="elsevierStyleHsp" style=""></span>mg/dL, C4 8<span class="elsevierStyleHsp" style=""></span>mg/dL). CT of chest, abdomen and pelvis showed cervical, axillary and retroperitoneal lymphadenopathy, hepatosplenomegaly with no focal lesions. No signs of infection or malignancy. Biopsy of axillary node showed <span class="elsevierStyleItalic">non-specific reactive lymphadenitis.</span> Bone marrow aspiration and biopsy were performed but the sample was inappropriate. Soluble CD25 in serum was only collected after 10 days of therapy. Even though, the level remained high (1187<span class="elsevierStyleHsp" style=""></span>U/mL, <span class="elsevierStyleItalic">N</span> 158–623).</p><p id="par0015" class="elsevierStylePara elsevierViewall">New-onset of systemic lupus erythematosus (SLE) associated with MAS was diagnosed. MAS was diagnosed according to the HLH-2004 criteria and the <span class="elsevierStyleItalic">H</span>-score.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5</span></a> The patient started hydroxychloroquine 400<span class="elsevierStyleHsp" style=""></span>mg daily, intravenous methylprednisolone pulse (1<span class="elsevierStyleHsp" style=""></span>g daily for 3 days), followed by 1<span class="elsevierStyleHsp" style=""></span>mg/kg daily of oral prednisolone and cyclosporine 3<span class="elsevierStyleHsp" style=""></span>mg/kg daily. <span class="elsevierStyleItalic">Prophylaxis</span> for <span class="elsevierStyleItalic">Pneumocystis jiroveci</span>, calcium and vitamin D supplementation were also started.</p><p id="par0020" class="elsevierStylePara elsevierViewall">A significant clinical and laboratory improvement was noticed with resolution of fever and constitutional symptoms, improvement of pancytopenia, hyperferritinemia, hypertriglyceridemia and hypofibrinogenemia. The patient was discharged after 3 weeks, with steroid tapering. At one year follow-up, a sustained remission was observed. Steroid was stopped and hydroxychloroquine 400<span class="elsevierStyleHsp" style=""></span>mg daily and cyclosporine 2.5<span class="elsevierStyleHsp" style=""></span>mg/kg daily were maintained.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0025" class="elsevierStylePara elsevierViewall">MAS is rarely associated with SLE and the incidence is 0.9–4.6%.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,6</span></a> The mainstay of treatment is steroids.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,7</span></a> Concomitant use of other agents, such as etoposide, cyclosporine, high-dose IV immunoglobulin and anakinra is useful in patients with severe, corticosteroid-resistant or refractory MAS.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">7</span></a> Cyclosporine is a cyclic polypeptide immunosuppressant that suppresses the production of IL-2, IFN-γ TNF-<span class="elsevierStyleItalic">α</span>, IL-1 and IL-6, and also inhibits T lymphocyte activation.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> This agent is effective for the induction and maintenance of remission in patients with MAS associated with rheumatic diseases, even in the cases of severe and corticosteroid-resistant disease.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Early diagnosis is crucial since mortality remains high, even in patients undergoing treatment.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> Here, we present an unusual, severe and life-threatening onset manifestation of SLE, MAS. Remission of the disease was achieved with corticosteroids and ciclosporine, with no adverse events reported, which corroborate the effectiveness, and also the safety, of this therapy.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conclusion</span><p id="par0035" class="elsevierStylePara elsevierViewall">Fever with constitutional symptoms and cytopenia can be clues to MAS and the level of suspicion should be high, to guarantee an early diagnosis. Looking for secondary causes of MAS, such as rheumatic diseases, is essential to ensure an accurate diagnosis and to select the appropriate treatment. However, a genetic predisposition can also exist and be related to dysregulated inflammasome activity (e.g., NLRC4 gene), and especially in situations with a positive family history of MAS, this should be investigated.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflict of interest</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare they have no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:10 [ 0 => array:3 [ "identificador" => "xres1937906" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1669998" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1937905" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1669999" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Clinical observation" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conclusion" ] 8 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflict of interest" ] 9 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2022-07-11" "fechaAceptado" => "2022-09-12" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1669998" "palabras" => array:3 [ 0 => "Systemic lupus erythematosus" 1 => "Macrophagic activation syndrome" 2 => "Cyclosporin" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1669999" "palabras" => array:3 [ 0 => "Lupus eritematoso sistémico" 1 => "Síndrome de activación macrofágica" 2 => "Ciclosporina" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic diseases. We report a unique case of a previously healthy 20-year-old female presenting with MAS as first presentation of systemic lupus erythematosus. Remission was achieved with hydroxychloroquine, intravenous methylprednisolone pulse followed by oral prednisolone and cyclosporine. However, the management of MAS is still challenging, and the mortality rate remains high.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El síndrome de activación macrofágica (SAM) es una complicación potencialmente letal de algunas enfermedades reumáticas. Presentamos un caso único de una mujer de 20 años previamente sana que se presentó con SAM como primera manifestación de lupus eritematoso sistémico. Se logró una remisión completa con hidroxicloroquina, pulsos intravenosos de metilprednisolona seguido de prednisolona oral y ciclosporina. Sin embargo, el manejo del SAM sigue siendo un desafío y la tasa de mortalidad sigue siendo alta.</p></span>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "S. Fukaya" 1 => "S. Yasuda" 2 => "T. Hashimoto" 3 => "K. Oku" 4 => "H. Kataoka" 5 => "T. 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