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ADAMTS13&#44; 0&#37; and negative anti-ADAMTS13 and genetic tests&#46; The initial treatment consisted of IV methylprednisolone &#40;3 boluses of 500&#8239;mg&#41; followed by a step-down protocol of oral prednisone &#40;1&#8239;mg&#47;kg&#41; and plasmapheresis&#46; The patient exhibited a favourable haematological and renal response after 1 week of treatment&#44; so she was discharged home&#46; A month later&#44; after withdrawal of steroid therapy&#44; the patient presented with epistaxis and purpura in the lower extremities and blood tests results evincing worsening&#58; Hb&#44; 10&#8239;g&#47;dL&#59; platelets&#44; 10 000&#47;&#956;l&#59; Pr&#58;Cr 5&#8239;mg&#47;mg&#46; The test for detection of anti-Ro52 antibodies was positive&#46; Based on the suspicion of reactivation of thrombotic thrombocytopenic purpura &#40;TTP&#41; in the context SLE&#44; the patient once again underwent plasmapheresis and received treatment with methylprednisolone boluses and oral prednisone at a dose of 2&#8239;mg&#47;kg&#46; The patient exhibited a favourable haematologic response &#40;Hb&#44; 11&#8239;g&#47;dL&#59; platelets&#44; 178 000&#47;&#956;l&#41;&#44; and the subsequent examination of a renal biopsy specimen revealed features suggestive of class V lupus nephritis&#44; prompting initiation of maintenance therapy with mycophenolate &#40;1&#8239;g&#47;12&#8239;h&#41; that achieved normalization of the protein levels in urine and ADAMTS13 activity &#40;95&#37;&#41;&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">A Dominican boy aged 11 years presented with illness of 1 week&#8217;s duration characterised by fever&#44; aphthae&#44; a butterfly rash&#44; erythematous oedematous plaques in the trunk &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#41; and polyarthralgia&#46; The salient findings of blood tests were&#58; white blood cell count of 2000 cells&#47;&#956;L&#59; Hb&#44; 10&#8239;g&#47;dL&#59; positive direct Coombs test&#59; ANA antibodies&#44; 1&#47;1280&#59; anti-dsDNA greater than 1&#47;320&#59; C3&#44; 18&#8239;mg&#47;dL and C4&#44; 1&#46;8&#8239;mg&#47;dL&#59; a triple positive antiphospholipid IgE antibody profile in absence of thrombotic features &#40;strongly positive anticoagulant lupus test&#59; anti-B2GPI IgG&#44; 231&#46;7&#8239;U&#47;mL&#59; ACA IgG&#44; 70&#8239;U&#47;mL&#41;&#59; activated partial thromboplastin time &#40;APTT&#41;&#44; 33&#8239;s&#59; prothrombin percent activity &#40;PT&#37;&#41;&#44; 52&#37;&#59; factor II&#44; 33&#37;&#59; mixing study PT&#37;&#44; 85&#37; indicative of factor II deficiency with no evidence of bleeding&#59; mild haematuria&#59; Pr&#58;Cr&#44; 1&#8239;mg&#47;mg with normal renal function&#46; The biopsy findings were compatible with acute cutaneous lupus&#44; and the renal biopsy was postponed due to risk of haemorrhage&#44; but it remained necessary because prognosis and treatment depend on the pathological classification of affected important organs&#46; Treatment consisted of a step-down protocol of oral prednisone at an initial dose of 1&#8239;mg&#47;kg and hydroxychloroquine at a dose of 200&#8239;mg&#47;day&#44; with clinical improvement of the skin and mucosae observed at 5 days &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>B&#41; and normalization of coagulation &#40;negative lupus antigoaculant test&#59; anti-B2GPI IgG&#44; 48&#8239;U&#47;mL and ACA IgG&#44; 26&#8239;U&#47;mL&#59; mixing study PT&#37;&#44; 77&#37;&#59; factor II&#44; 60&#37;&#41; that was maintained throughout the follow-up&#46; This allowed obtention of a renal biopsy sample the features of which were compatible with class III lupus nephritis&#46; The patient started treatment with mycophenolate &#40;500&#8239;mg&#47;12&#8239;h&#41; with normalization of urine sediment findings at 1 month&#46; Three months later&#44; rituximab was added &#40;2 doses of 750&#8239;mg&#47;m<span class="elsevierStyleSup">2</span> 14 days apart&#41; to manage refractory cutaneous and articular activity&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Thrombotic microangiopathies are characterised by haemolytic anaemia and thrombocytopenia with variable organ involvement&#46; The most frequent type is HUS secondary to infection by Shiga toxin-producing <span class="elsevierStyleItalic">E&#46; coli</span>&#59; atypical SHU results from a dysregulation of the alternative complement pathway of genetic aetiology that is treated with eculizumab&#44; which blocks C5 and thus inhibits formation of the membrane attack complex<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a>&#59; TTP results from a congenital or acquired deficiency of ADAMTS13 activity&#46; Secondary MATs develop in the context of various diseases&#44; such as disorders of connective tissues &#40;chiefly SLE and antiphospholipid syndrome&#41;&#46; Systemic lupus erythematosus usually predates the onset of MAT &#40;73&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The histological classification of lupus nephritis is an important prognostic factor&#46; In addition to the classic subtypes&#44; it is important to assess for the presence of other lesions&#44; among which MAT is most relevant due to having the least favourable outcomes and being an independent risk factor for kidney injury&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Forms associated with SLE used to be managed with immunosuppressive therapy and plasmapheresis&#44;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> but since complement activation plays a role in both disorders&#44; eculizumab has been used in recent series of patients with lupus nephritis refractory to conventional immunosuppressive therapy&#44; achieving good responses&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Other studies have reported clinical improvement and a lower cumulative steroid dose with the addition of rituximab to induction therapy&#44; especially in black patients&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The presence of TTP is associated with long duration of SLE&#44; nephritis and high disease activity&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Clinical manifestations and monitoring of enzymatic activity &#40;which is normal in remission&#41; can guide the diagnosis in case of negative antibody test results&#46; Patients are managed with plasmapheresis until blood work findings improve or with immunoglobulin therapy combined with high-dose steroid therapy&#46; The addition of rituximab may be helpful in refractory cases&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Lupus anticoagulant hypoprothrombinaemia syndrome &#40;LAHS&#41; is associated with an acquired factor II deficiency and positive lupus anticoagulant results&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> It is most frequent in childhood<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and predominantly associated to connective tissue disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> It is suspected in the presence of prolonged aPTT and PTT with full correction of PTT and minimal correction of the aPTT in the mixing studies&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> It manifests with bleeding &#40;mainly of the skin and mucosae&#41; but may be associated with thrombosis in patients with tissue disorders &#40;10&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Treatment is based on plasma transfusions and steroid therapy alone or combined with an immunosuppressive agent depending on the systemic manifestations&#44; which usually achieves normalization of coagulation and resolution of bleeding&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a> At this point&#44; the patients should be monitored for the potential development of thrombosis with initiation of platelet antiaggregation or anticoagulation therapy if necessary&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p></span>"
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Scientific Letter
Systemic lupus erythematosus of atypical onset: a presentation of 3 cases
Lupus eritematoso sistémico de debut atípico: a propósito de tres casos
Laura Barrio Nogala,
Corresponding author
lbarrio@torrejonsalud.com

Corresponding author.
, Daniel Clemente Garulob, Carmen de Lucas Collantesc, Cristina Aparicio Lópezc, Juan Carlos López Robledillob
a Servicio de Enfermedades del Sistema Inmune y Reumatología, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain
b Servicio de Reumatología Pediátrica, Hospital Universitario Niño Jesús, Madrid, Spain
c Servicio de Nefrología, Hospital Universitario Niño Jesús, Madrid, Spain
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we initiated treatment with 3 intravenous &#40;IV&#41; methylprednisolone pulses &#40;500&#8239;mg&#41; followed by oral prednisone &#40;1&#8239;mg&#47;kg&#41;&#44; IV cyclophosphamide &#40;500&#8239;mg&#47;15 days&#41;&#44; IV eculizumab &#40;900&#8239;mg&#47;week&#41; and sessions of haemofiltration to manage the progressive deterioration of renal function and uraemic encephalopathy&#46; Examination of a renal biopsy specimen revealed features compatible with class IV lupus nephritis with active and chronic &#40;A&#47;C&#41; lesions and thrombotic microangiopathy &#40;TMA&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; After 2 weeks&#44; given the persistence of primarily haematologic activity&#44; we added immunoglobulins &#40;1&#8239;mg&#47;kg&#41; and rituximab &#40;375&#8239;mg&#47;m<span class="elsevierStyleSup">2</span>&#47;week for 4 weeks&#44; alternating every other week with cyclophosphamide&#41;&#46; After a month&#8217;s stay in hospital&#44; the patient exhibited progressive and sustained improvement of renal function in absence of proteinuria &#40;Cr&#44; 0&#46;82&#8239;mg&#47;dL&#59; Pr&#58;Cr&#44; 0&#46;75&#8239;mg&#47;mg&#41; and of TMA &#40;Hb&#44; 11&#8239;g&#47;dL&#59; platelets&#44; 378 000&#47;&#956;l&#41;&#46; After completing the course of cyclophosphamide&#44; the patient started maintenance treatment with mycophenolate &#40;500&#8239;mg&#47;12&#8239;h&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">An Asian girl aged 8 years presented with poor general health&#46; The salient findings of blood tests were&#58; Hb&#44; 8&#46;4&#8239;g&#47;dL&#59; schistocytes&#59; platelets&#44; 11 000&#47;&#956;l&#44; negative direct Coombs test&#59; haematuria and Pr&#58;Cr of 11&#8239;mg&#47;mg with normal renal function&#59; ANA&#44; 1&#47;640&#59; negative anti-dsDNA test&#59; C3&#44; 60&#46;9&#8239;mg&#47;dL and C4&#44; 12&#46;8&#8239;mg&#47;dL&#59; ADAMTS13&#44; 0&#37; and negative anti-ADAMTS13 and genetic tests&#46; The initial treatment consisted of IV methylprednisolone &#40;3 boluses of 500&#8239;mg&#41; followed by a step-down protocol of oral prednisone &#40;1&#8239;mg&#47;kg&#41; and plasmapheresis&#46; The patient exhibited a favourable haematological and renal response after 1 week of treatment&#44; so she was discharged home&#46; A month later&#44; after withdrawal of steroid therapy&#44; the patient presented with epistaxis and purpura in the lower extremities and blood tests results evincing worsening&#58; Hb&#44; 10&#8239;g&#47;dL&#59; platelets&#44; 10 000&#47;&#956;l&#59; Pr&#58;Cr 5&#8239;mg&#47;mg&#46; The test for detection of anti-Ro52 antibodies was positive&#46; Based on the suspicion of reactivation of thrombotic thrombocytopenic purpura &#40;TTP&#41; in the context SLE&#44; the patient once again underwent plasmapheresis and received treatment with methylprednisolone boluses and oral prednisone at a dose of 2&#8239;mg&#47;kg&#46; The patient exhibited a favourable haematologic response &#40;Hb&#44; 11&#8239;g&#47;dL&#59; platelets&#44; 178 000&#47;&#956;l&#41;&#44; and the subsequent examination of a renal biopsy specimen revealed features suggestive of class V lupus nephritis&#44; prompting initiation of maintenance therapy with mycophenolate &#40;1&#8239;g&#47;12&#8239;h&#41; that achieved normalization of the protein levels in urine and ADAMTS13 activity &#40;95&#37;&#41;&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">A Dominican boy aged 11 years presented with illness of 1 week&#8217;s duration characterised by fever&#44; aphthae&#44; a butterfly rash&#44; erythematous oedematous plaques in the trunk &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#41; and polyarthralgia&#46; The salient findings of blood tests were&#58; white blood cell count of 2000 cells&#47;&#956;L&#59; Hb&#44; 10&#8239;g&#47;dL&#59; positive direct Coombs test&#59; ANA antibodies&#44; 1&#47;1280&#59; anti-dsDNA greater than 1&#47;320&#59; C3&#44; 18&#8239;mg&#47;dL and C4&#44; 1&#46;8&#8239;mg&#47;dL&#59; a triple positive antiphospholipid IgE antibody profile in absence of thrombotic features &#40;strongly positive anticoagulant lupus test&#59; anti-B2GPI IgG&#44; 231&#46;7&#8239;U&#47;mL&#59; ACA IgG&#44; 70&#8239;U&#47;mL&#41;&#59; activated partial thromboplastin time &#40;APTT&#41;&#44; 33&#8239;s&#59; prothrombin percent activity &#40;PT&#37;&#41;&#44; 52&#37;&#59; factor II&#44; 33&#37;&#59; mixing study PT&#37;&#44; 85&#37; indicative of factor II deficiency with no evidence of bleeding&#59; mild haematuria&#59; Pr&#58;Cr&#44; 1&#8239;mg&#47;mg with normal renal function&#46; The biopsy findings were compatible with acute cutaneous lupus&#44; and the renal biopsy was postponed due to risk of haemorrhage&#44; but it remained necessary because prognosis and treatment depend on the pathological classification of affected important organs&#46; Treatment consisted of a step-down protocol of oral prednisone at an initial dose of 1&#8239;mg&#47;kg and hydroxychloroquine at a dose of 200&#8239;mg&#47;day&#44; with clinical improvement of the skin and mucosae observed at 5 days &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>B&#41; and normalization of coagulation &#40;negative lupus antigoaculant test&#59; anti-B2GPI IgG&#44; 48&#8239;U&#47;mL and ACA IgG&#44; 26&#8239;U&#47;mL&#59; mixing study PT&#37;&#44; 77&#37;&#59; factor II&#44; 60&#37;&#41; that was maintained throughout the follow-up&#46; This allowed obtention of a renal biopsy sample the features of which were compatible with class III lupus nephritis&#46; The patient started treatment with mycophenolate &#40;500&#8239;mg&#47;12&#8239;h&#41; with normalization of urine sediment findings at 1 month&#46; Three months later&#44; rituximab was added &#40;2 doses of 750&#8239;mg&#47;m<span class="elsevierStyleSup">2</span> 14 days apart&#41; to manage refractory cutaneous and articular activity&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Thrombotic microangiopathies are characterised by haemolytic anaemia and thrombocytopenia with variable organ involvement&#46; The most frequent type is HUS secondary to infection by Shiga toxin-producing <span class="elsevierStyleItalic">E&#46; coli</span>&#59; atypical SHU results from a dysregulation of the alternative complement pathway of genetic aetiology that is treated with eculizumab&#44; which blocks C5 and thus inhibits formation of the membrane attack complex<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a>&#59; TTP results from a congenital or acquired deficiency of ADAMTS13 activity&#46; Secondary MATs develop in the context of various diseases&#44; such as disorders of connective tissues &#40;chiefly SLE and antiphospholipid syndrome&#41;&#46; Systemic lupus erythematosus usually predates the onset of MAT &#40;73&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The histological classification of lupus nephritis is an important prognostic factor&#46; In addition to the classic subtypes&#44; it is important to assess for the presence of other lesions&#44; among which MAT is most relevant due to having the least favourable outcomes and being an independent risk factor for kidney injury&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Forms associated with SLE used to be managed with immunosuppressive therapy and plasmapheresis&#44;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> but since complement activation plays a role in both disorders&#44; eculizumab has been used in recent series of patients with lupus nephritis refractory to conventional immunosuppressive therapy&#44; achieving good responses&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Other studies have reported clinical improvement and a lower cumulative steroid dose with the addition of rituximab to induction therapy&#44; especially in black patients&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The presence of TTP is associated with long duration of SLE&#44; nephritis and high disease activity&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Clinical manifestations and monitoring of enzymatic activity &#40;which is normal in remission&#41; can guide the diagnosis in case of negative antibody test results&#46; Patients are managed with plasmapheresis until blood work findings improve or with immunoglobulin therapy combined with high-dose steroid therapy&#46; The addition of rituximab may be helpful in refractory cases&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Lupus anticoagulant hypoprothrombinaemia syndrome &#40;LAHS&#41; is associated with an acquired factor II deficiency and positive lupus anticoagulant results&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> It is most frequent in childhood<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and predominantly associated to connective tissue disorders&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> It is suspected in the presence of prolonged aPTT and PTT with full correction of PTT and minimal correction of the aPTT in the mixing studies&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> It manifests with bleeding &#40;mainly of the skin and mucosae&#41; but may be associated with thrombosis in patients with tissue disorders &#40;10&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Treatment is based on plasma transfusions and steroid therapy alone or combined with an immunosuppressive agent depending on the systemic manifestations&#44; which usually achieves normalization of coagulation and resolution of bleeding&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;6</span></a> At this point&#44; the patients should be monitored for the potential development of thrombosis with initiation of platelet antiaggregation or anticoagulation therapy if necessary&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p></span>"
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