PMID- 16840400
OWN - NLM
STAT- MEDLINE
DCOM- 20060810
LR  - 20140729
IS  - 0012-3692 (Print)
IS  - 0012-3692 (Linking)
VI  - 130
IP  - 1
DP  - 2006 Jul
TI  - Immunosuppressive therapy in connective tissue diseases-associated pulmonary 
      arterial hypertension.
PG  - 182-9
AB  - 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.
FAU - Sanchez, Olivier
AU  - Sanchez O
AD  - Centre National de Reference de l'Hypertension Arterielle Pulmonaire, UPRES 
      EA2705, Service de Pneumologie et Reanimation Respiratoire, Hopital Antoine 
      Beclere, Assistance Publique Hopitaux de Paris, Universite Paris-Sud, Clamart, 
      France.
FAU - Sitbon, Olivier
AU  - Sitbon O
FAU - Jais, Xavier
AU  - Jais X
FAU - Simonneau, Gerald
AU  - Simonneau G
FAU - Humbert, Marc
AU  - Humbert M
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Chest
JT  - Chest
JID - 0231335
RN  - 0 (Glucocorticoids)
RN  - 0 (Immunosuppressive Agents)
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Blood Pressure/drug effects
MH  - Connective Tissue Diseases/classification/complications/*drug therapy
MH  - Cyclophosphamide/*therapeutic use
MH  - Exercise Test/methods
MH  - Female
MH  - Glucocorticoids/*therapeutic use
MH  - Humans
MH  - Hypertension, Pulmonary/drug therapy/*etiology/physiopathology
MH  - Immunosuppressive Agents/*therapeutic use
MH  - Male
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Severity of Illness Index
EDAT- 2006/07/15 09:00
MHDA- 2006/08/11 09:00
CRDT- 2006/07/15 09:00
PHST- 2006/07/15 09:00 [pubmed]
PHST- 2006/08/11 09:00 [medline]
PHST- 2006/07/15 09:00 [entrez]
AID - S0012-3692(15)50970-7 [pii]
AID - 10.1378/chest.130.1.182 [doi]
PST - ppublish
SO  - Chest. 2006 Jul;130(1):182-9. doi: 10.1378/chest.130.1.182.