Document Detail

Characteristics and outcome of connective tissue diseases in patients with sickle-cell disease: report of 30 cases.
MedLine Citation:
PMID:  18177923     Owner:  NLM     Status:  MEDLINE    
OBJECTIVES: 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.
M Michel; A Habibi; B Godeau; D Bachir; A Lahary; F Galacteros; A Fifi-Mah; S Arfi
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Publication Detail:
Type:  Journal Article     Date:  2008-01-04
Journal Detail:
Title:  Seminars in arthritis and rheumatism     Volume:  38     ISSN:  1532-866X     ISO Abbreviation:  Semin. Arthritis Rheum.     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2008-11-25     Completed Date:  2009-02-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  1306053     Medline TA:  Semin Arthritis Rheum     Country:  United States    
Other Details:
Languages:  eng     Pagination:  228-40     Citation Subset:  IM    
Department of Internal Medicine, Henri-Mondor University Hospital, Créteil, France.
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MeSH Terms
Anemia, Sickle Cell / complications*,  diagnosis,  mortality,  therapy
Antisickling Agents / therapeutic use
Arthritis, Rheumatoid / complications*,  diagnosis,  drug therapy,  mortality
Blood Transfusion
Drug Therapy, Combination
France / epidemiology
Glucocorticoids / therapeutic use
Hydroxyurea / therapeutic use
Immunosuppressive Agents / therapeutic use
Lupus Erythematosus, Systemic / complications*,  diagnosis,  drug therapy,  mortality
Methotrexate / therapeutic use
Retrospective Studies
Risk Assessment
Severity of Illness Index
Survival Rate
Treatment Outcome
Reg. No./Substance:
0/Antisickling Agents; 0/Glucocorticoids; 0/Immunosuppressive Agents; 127-07-1/Hydroxyurea; 59-05-2/Methotrexate

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