title: Cardiac tamponade and severe pericardial effusion in systemic sclerosis: report of nine patients and review of the literature.
abstract: OBJECTIVE
  To describe the clinical characteristics, treatment and outcome of patients with systemic sclerosis (SSc) developing severe pericardial effusion or cardiac tamponade.
  
  
  METHODS
  SSc patients with severe pericardial effusion or cardiac tamponade from three Spanish hospitals were collected. In addition, a computer-assisted (PubMed, MEDLINE) search of the literature to identify all cases of cardiac tamponade or severe pericardial effusion associated with SSc reported in English, French and Spanish from 1987 through September 2015 was performed.
  
  
  RESULTS
  We included 40 patients (nine cases from the Spanish hospitals and 31 cases from the literature review). Most patients (87%) were female with a mean age at pericardial involvement of 49.3 ± 15.2 years (range: 18-80 years), and 22 (55%) patients had the diffuse cutaneous subtype. Twenty-five (63%) patients presented with cardiac tamponade and the remaining 15 (37%) as severe or massive pericardial effusion. Pericardial involvement was previous or simultaneous to SSc diagnosis in 13 (32.5%) cases. In most cases (88.9%) pericardial fluid analysis disclosed an exudate. Half the patients received steroids and 35% needed surgical treatment. Five (12.5%) patients died due to cardiac tamponade, three of them during the acute phase and the remaining two, 2 and 9 months later, respectively.
  
  
  CONCLUSIONS
  Although cardiac tamponade or severe pericardial effusion is an infrequent complication in SSc patients, it can be the first manifestation of disease associated with the diffuse cutaneous subset. No specific treatment for this complication is known.
authors.affiliation: Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
authors.forename: Alejandro
authors.initials: A
authors.lastname: Fernández Morales
authors.affiliation: Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
authors.forename: Nerea
authors.initials: N
authors.lastname: Iniesta
authors.affiliation: Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Catalonia, Spain.
authors.forename: Andreu
authors.identifier: http://orcid.org/0000-0001-7939-7837
authors.initials: A
authors.lastname: Fernández-Codina
authors.affiliation: Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
authors.forename: Joana
authors.initials: J
authors.lastname: Vaz de Cunha
authors.affiliation: Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
authors.forename: Teresa
authors.initials: T
authors.lastname: Pérez Romero
authors.affiliation: Department of Internal Medicine, Hospital de la Vega Baja, Orihuela, Alacant, Spain.
authors.forename: Robert
authors.initials: R
authors.lastname: Hurtado García
authors.affiliation: Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Catalonia, Spain.
authors.forename: Carmen Pilar
authors.initials: CP
authors.lastname: Simeón-Aznar
authors.affiliation: Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Catalonia, Spain.
authors.forename: Vicent
authors.initials: V
authors.lastname: Fonollosa
authors.affiliation: Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
authors.affiliation: Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002305:Cardiac Tamponade / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010490:Pericardial Effusion / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D010491:Pericardial Window Techniques; D020519:Pericardiocentesis; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D012720:Severity of Illness Index; D013030:Spain; D013256:Steroids / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D013256:Steroids
keywords: cardiac tamponade; scleroderma; severe pericardial effusion; systemic sclerosis