Document Detail

Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension.
MedLine Citation:
PMID:  16840400     Owner:  NLM     Status:  MEDLINE    
STUDY OBJECTIVE: Immune and inflammatory mechanisms could play a significant role in pulmonary arterial hypertension (PAH) genesis or progression, especially in patients with connective tissue diseases. Immunosuppressive therapy should be better evaluated in this setting.
STUDY DESIGN: Monocentric retrospective study.
PATIENTS: We reviewed the clinical and hemodynamic effects of immunosuppressants administered as first-line monotherapy to 28 consecutive patients with connective tissue disease-associated PAH.
INTERVENTIONS: All patients received a monthly IV bolus of cyclophosphamide, 600 mg/m2, for at least 3 months, and 22 of 28 patients received systemic glucocorticosteroids. Responders to immunosuppressive therapy were defined as patients who remained in New York Heart Association (NYHA) functional class I or II with sustained hemodynamic improvement after at least 1 year of immunosuppressive therapy without addition of prostanoids, phosphodiesterase type 5 inhibitors, or endothelin receptor antagonists.
RESULTS: Eight of 28 patients (systemic lupus erythematosus [SLE], n = 5; mixed connective tissue disease [MCTD], n = 3) [29%] were responders. These patients had a significantly improved 6-min walking distance (available in five patients) and a significant improvement in hemodynamic function. No patients with systemic sclerosis responded, while 5 of 12 patients with SLE and 3 of 8 patients with MCTD did respond. Survival analysis indicated that responders had a better survival than nonresponders. Patients with a lower baseline NYHA functional class and better baseline pulmonary hemodynamics (p < 0.05) were more likely to benefit from immunosuppressive therapy.
CONCLUSION: PAH associated with SLE or MCTD might respond to a treatment combining glucocorticosteroids and cyclophosphamide.
Olivier Sanchez; Olivier Sitbon; Xavier Jaïs; Gérald Simonneau; Marc Humbert
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Chest     Volume:  130     ISSN:  0012-3692     ISO Abbreviation:  Chest     Publication Date:  2006 Jul 
Date Detail:
Created Date:  2006-07-14     Completed Date:  2006-08-10     Revised Date:  2014-07-29    
Medline Journal Info:
Nlm Unique ID:  0231335     Medline TA:  Chest     Country:  United States    
Other Details:
Languages:  eng     Pagination:  182-9     Citation Subset:  AIM; IM    
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MeSH Terms
Blood Pressure / drug effects
Connective Tissue Diseases / classification,  complications,  drug therapy*
Cyclophosphamide / therapeutic use*
Exercise Test / methods
Glucocorticoids / therapeutic use*
Hypertension, Pulmonary / drug therapy,  etiology*,  physiopathology
Immunosuppressive Agents / therapeutic use*
Middle Aged
Retrospective Studies
Severity of Illness Index
Reg. No./Substance:
0/Glucocorticoids; 0/Immunosuppressive Agents; 8N3DW7272P/Cyclophosphamide

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