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More than a third of SS patients may develop CI at some point. Cardiovascular manifestations are now the third leading cause of death in these patients. No effective specific treatment has been reported for CI in SS.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Up to 26% of individuals with CI can develop pericardial effusion (PE), which is usually mild and can manifest early in diffuse cutaneous SS.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> To date, only three cases of SS with constrictive pericarditis have been reported.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> In this manuscript, we report the first case successfully treated with sodium mycophenolate.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 43-year-old Caucasian woman was admitted to our hospital because of persistent dyspnoea and PE. She had previously been a smoker. Her medical history included hypertension, dyslipidaemia, type 2 diabetes, atrial fibrillation and chronic renal failure due to cardiovascular risk factors. Regarding the SS, the diagnosis had been made 19 years ago. Two years earlier, during a hospitalization for diarrhoea and dehydration, she developed for the first time severe PE, with echocardiographic signs of cardiac tamponade. At that time, she was treated with oral methotrexate (15<span class="elsevierStyleHsp" style=""></span>mg/day) and prednisone (7.5<span class="elsevierStyleHsp" style=""></span>mg/day). Pericardiocentesis was performed, draining a total of 600<span class="elsevierStyleHsp" style=""></span>cc of exudative fluid. A cardiac MRI showed mild systolic dysfunction (50%) of the left ventricle (LV), with lower apical hypokinesia associated with a small lower apical subendocardial infarction. She was treated with prednisone (0.5<span class="elsevierStyleHsp" style=""></span>mg/kg/day). Treatment with methotrexate was discontinued. During follow-up, PE was completely resolved. A year later she developed severe PE again, without haemodynamic compromise, and was treated with hydroxychloroquine 400<span class="elsevierStyleHsp" style=""></span>mg/day and prednisone 0.5<span class="elsevierStyleHsp" style=""></span>mg/kg/day, leaving a small amount of pericardial fluid. Nine months later she was admitted again to her local hospital due to a second episode of cardiac tamponade, requiring pericardiocentesis again, obtaining 500<span class="elsevierStyleHsp" style=""></span>cc of pericardial fluid. After a transient increase in the dose of prednisone, treatment was maintained again at 5<span class="elsevierStyleHsp" style=""></span>mg/day of prednisone and 400<span class="elsevierStyleHsp" style=""></span>mg/day of hydroxychloroquine.</p><p id="par0015" class="elsevierStylePara elsevierViewall">A month before being admitted to our hospital, she developed oedema in the lower limbs, palpitations and dyspnoea with a IIIb functional class according to the <span class="elsevierStyleItalic">New York Heart Association</span> (NYHA). Treatment was started with furosemide, with partial improvement, finally being referred to our site.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Vital signs on admission were: blood pressure 96/67<span class="elsevierStyleHsp" style=""></span>mmHg, heart rate 90<span class="elsevierStyleHsp" style=""></span>bpm, oxygen saturation on room air 90%. Some bibasilar moist rales and jugular venous distension were detected on auscultation. The electrocardiogram revealed the presence of atrial fibrillation with complete left bundle branch block. The value of brain natriuretic peptide was 430.2<span class="elsevierStyleHsp" style=""></span>pg/dl (<span class="elsevierStyleItalic">N</span><span class="elsevierStyleHsp" style=""></span><<span class="elsevierStyleHsp" style=""></span>100). Immunological tests showed the presence of antinuclear antibodies with a titre of 1/640. The indirect immunofluorescence test evidenced a speckled pattern. The test for anti-RNA polymerase-III detection was positive. The high-resolution CT scan showed the presence of mild interstitial lung disease, and a thickened pericardium. Echocardiography revealed absence of PE, with the presence of overall dyskinesia (LV ejection fraction was 35%). There were signs suggestive of pericarditis. The left coronary angiography found no significant abnormalities. Right heart catheterization confirmed the diagnosis of pericarditis.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The final diagnosis was chronic constrictive pericarditis associated with SS. Performing a pericardiectomy was considered, but the surgical risk was greater than the potential benefits. Treatment of heart failure was optimized by adding bisoprolol at low doses (2.5<span class="elsevierStyleHsp" style=""></span>mg/day). Sodium mycophenolate (720<span class="elsevierStyleHsp" style=""></span>mg/day) was initiated. Doses of prednisone and furosemide were maintained. She was discharged without signs of heart failure or respiratory compromise.</p><p id="par0030" class="elsevierStylePara elsevierViewall">She has remained stable during follow-up and has been monitored on an outpatient basis. After 9 months of treatment, she achieved a functional class II as per NYHA. Further episodes of cardiac tamponade or heart failure did not occur. At that time, an echocardiogram showed a slight decrease in left ventricular ejection fraction (48% 2D) with the presence of septal <span class="elsevierStyleItalic">notch</span>, and no other signs of constriction. PE or indirect signs of pulmonary arterial hypertension were not detected.</p><p id="par0035" class="elsevierStylePara elsevierViewall">CI is a common complication in patients with SS. Acute pericarditis can be treated with nonsteroidal anti-inflammatory drugs or colchicine. In chronic cases, corticosteroids or disease-modifying antirheumatic drugs may be indicated, although there is risk of developing scleroderma renal crisis with equivalent doses of prednisone >7.5<span class="elsevierStyleHsp" style=""></span>mg/day in diffuse cutaneous SS.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1,4</span></a> Mycophenolate is a safe drug, with immunosuppressive and anti-fibrotic effect, which has proved effective in SS for skin and lung involvement.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Because of this, we decided to initiate mycophenolate sodium in our patient, which proved to be effective for the treatment of chronic pericardial disease. Further studies in order to evaluate alternatives for pericardial involvement related to SS are needed.</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: Fernández-Codina A, Francisco-Pascual J, Fonollosa-Plà V. Tratamiento exitoso de un caso de pericarditis crónica constrictiva utilizando micofenolato sódico en una paciente con esclerosis sistémica. Med Clin (Barc). 2017;148:574–575.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Systemic sclerosis and the heart" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "J.L. 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Hidalgo" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s00296-015-3382-2" "Revista" => array:6 [ "tituloSerie" => "Rheumatol Int" "fecha" => "2017" "volumen" => "37" "paginaInicial" => "75" "paginaFinal" => "84" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26497313" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0040" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:3 [ "comentario" => "pii: bcr2013010254" "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "New development of cardiac tamponade on underlying effusive-constrictive pericarditis: an uncommon initial presentation of scleroderma" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "S.R. Subramanian" 1 => "R. Akram" 2 => "A. Velayati" 3 => "H. Chadow" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:2 [ "tituloSerie" => "BMJ Case Rep" "fecha" => "2013" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0045" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Cardiac tamponade and severe pericardial effusion in systemic sclerosis: report of nine patients and review of the literature" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Fernández Morales" 1 => "N. Iniesta" 2 => "A. Fernández-Codina" 3 => "J. Vaz de Cunha" 4 => "T. Pérez Romero" 5 => "R. Hurtado García" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/1756-185X.12952" "Revista" => array:2 [ "tituloSerie" => "Int J Rheum Dis" "fecha" => "2016" ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0050" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Safety and effectiveness of mycophenolate in systemic sclerosis. A systematic review" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "M.A. Omair" 1 => "A. Alahmadi" 2 => "S.R. Johnson" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1371/journal.pone.0124205" "Revista" => array:5 [ "tituloSerie" => "PLoS One" "fecha" => "2015" "volumen" => "10" "paginaInicial" => "e0124205" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/25933090" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] "agradecimientos" => array:1 [ 0 => array:4 [ "identificador" => "xack288346" "titulo" => "Acknowledgements" "texto" => "<p id="par0040" class="elsevierStylePara elsevierViewall">The authors wish to thank Drs. Carmen Pilar Simeon-Aznar, Paula Suanzes-Díez, Alfredo Guillén-del Castillo, Fernando Martinez-Valle (Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Vall d’Hebron University Hospital) and José Rodríguez-Palomares (Department of Cardiology, Vall d’Hebron University Hospital) for their valuable contributions.</p>" "vista" => "all" ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000014800000012/v1_201707060050/S2387020617303686/v1_201707060050/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000014800000012/v1_201707060050/S2387020617303686/v1_201707060050/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020617303686?idApp=UINPBA00004N" ]
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Vol. 148. Issue 12.
Pages 574-575 (June 2017)
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Vol. 148. Issue 12.
Pages 574-575 (June 2017)
Letter to the Editor
Successful treatment of constrictive chronic pericarditis with mycophenolate sodium in a patient with systemic sclerosis
Tratamiento exitoso de un caso de pericarditis crónica constrictiva utilizando micofenolato sódico en una paciente con esclerosis sistémica
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