title: Characteristics and outcome of connective tissue diseases in patients with sickle-cell disease: report of 30 cases.
abstract: OBJECTIVE
  To analyze the main characteristics of adults with sickle cell disease (SCD) and concurrent connective tissue disease (CTD).
  
  
  METHODS
  A retrospective investigational study was performed. CTD was diagnosed according to standard international criteria. Severity of SCD was assessed by a clinical severity score.
  
  
  RESULTS
  Thirty patients, 23 women (76%) and 7 men, with hemoglobin S/S (n = 25) or S/C (n = 5) SCD were included. The subtypes of CTD were rheumatoid arthritis (RA) (n = 15), definite systemic lupus erythematosus or "incomplete lupus" requiring treatment (n = 13), primary Sjögren's syndrome with central nervous system involvement (n = 1), and systemic sclerosis (n = 1). Twenty-five of the 30 patients (83%) received steroid treatment, and 15 (50%) received at least 1 immunosuppressive agent (methotrexate in 14 cases) to control CTD. Four RA patients were given antitumor necrosis factor (TNF)alpha and 1 was treated with rituximab without SCD exacerbation. After a median follow-up of 4.5 years [range: 6 months to 30 years] from CTD diagnosis, 11 of the 25 (44%) patients receiving steroids had at least 1 episode of severe infection (mostly due to Staphylococcus aureus or Escherichia coli). SCD exacerbated in 13 of the 30 (43%) patients after CTD onset; 12 of these patients were receiving prednisone and/or methotrexate. Six patients (20%) had died from sepsis (n = 2), stroke (n = 2), or acute chest syndrome (n = 2).
  
  
  CONCLUSIONS
  CTD-related clinical manifestations and outcome were not particularly severe in patients with SCD. However, those with active CTD and undergoing steroid +/- methotrexate treatment had more serious SCD-related manifestations, a higher rate of severe infections, and an overall patient mortality rate of 20%. Thus, the management of patients with CTD and underlying SCD should consider the risk/benefit ratio of each treatment and steroid-sparing strategies should be implemented.
authors.affiliation: Department of Internal Medicine, Henri-Mondor University Hospital, Créteil, France. marc.michel@hmn.aphp.fr.
authors.forename: M
authors.initials: M
authors.lastname: Michel
authors.forename: A
authors.initials: A
authors.lastname: Habibi
authors.forename: B
authors.initials: B
authors.lastname: Godeau
authors.forename: D
authors.initials: D
authors.lastname: Bachir
authors.forename: A
authors.initials: A
authors.lastname: Lahary
authors.forename: F
authors.initials: F
authors.lastname: Galacteros
authors.forename: A
authors.initials: A
authors.lastname: Fifi-Mah
authors.forename: S
authors.initials: S
authors.lastname: Arfi
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D000986:Antisickling Agents / Q000627:therapeutic use; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D001803:Blood Transfusion; D004359:Drug Therapy, Combination; D005260:Female; D005602:France / Q000453:epidemiology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000986:Antisickling Agents; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D006918:Hydroxyurea; D008727:Methotrexate