PMID- 18240255
OWN - NLM
STAT- MEDLINE
DCOM- 20080407
LR  - 20131121
IS  - 0004-3591 (Print)
IS  - 0004-3591 (Linking)
VI  - 58
IP  - 2
DP  - 2008 Feb
TI  - Immunosuppressive therapy in lupus- and mixed connective tissue 
      disease-associated pulmonary arterial hypertension: a retrospective analysis of 
      twenty-three cases.
PG  - 521-31
LID - 10.1002/art.23303 [doi]
AB  - OBJECTIVE: To describe the response to first-line immunosuppressive therapy with 
      or without pulmonary vasodilators in pulmonary arterial hypertension (PAH) 
      associated with systemic lupus erythematosus (SLE) or mixed connective tissue 
      disease (MCTD). METHODS: Twenty-three consecutive patients with SLE- or 
      MCTD-associated PAH treated with first-line immunosuppressive therapy either 
      alone (n = 16) or in combination with pulmonary vasodilators (n = 7) were 
      evaluated according to clinical and hemodynamic criteria before and after 
      immunosuppressive therapy. Responders were defined as patients in New York Heart 
      Association (NYHA) functional class I or II with hemodynamic improvement after 
      the last pulse of cyclophosphamide. RESULTS: Among the 16 patients treated with 
      first-line immunosuppressive therapy alone, 8 (50%) were responders. These 
      patients had a significantly improved NYHA functional class, 6-minute walking 
      distance, and mean pulmonary artery pressure. Patients in NYHA functional class I 
      or II and/or a cardiac index >3.1 liters/minute/m(2) at baseline were more likely 
      to benefit from immunosuppressive therapy. Six of the 8 nonresponders 
      subsequently improved with pulmonary vasodilators. Among the 7 patients who were 
      initially treated with immunosuppressive therapy and pulmonary vasodilators, 4 
      (57.1%) were responders. CONCLUSION: PAH associated with SLE or MCTD may respond 
      to a treatment combining cyclophosphamide and glucocorticoids. Patients who could 
      benefit from this immunosuppressive therapy could be those who have less severe 
      disease at baseline. For patients with more severe disease, pulmonary 
      vasodilators should be started, possibly in combination with immunosuppressants. 
      In any case, clinical and hemodynamic evaluations are mandatory to monitor the 
      response and adapt the treatment. These retrospective and uncontrolled data need 
      to be confirmed by randomized controlled trials.
FAU - Jais, Xavier
AU  - Jais X
AD  - Hopital Antoine-Beclere, Assistance Publique Hopitaux de Paris, Universite 
      Paris-Sud, Clamart, France.
FAU - Launay, David
AU  - Launay D
FAU - Yaici, Azzedine
AU  - Yaici A
FAU - Le Pavec, Jerome
AU  - Le Pavec J
FAU - Tcherakian, Colas
AU  - Tcherakian C
FAU - Sitbon, Olivier
AU  - Sitbon O
FAU - Simonneau, Gerald
AU  - Simonneau G
FAU - Humbert, Marc
AU  - Humbert M
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Arthritis Rheum
JT  - Arthritis and rheumatism
JID - 0370605
RN  - 0 (Immunosuppressive Agents)
RN  - 0 (Vasodilator Agents)
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
MH  - Adult
MH  - Algorithms
MH  - Cyclophosphamide/*administration & dosage/adverse effects
MH  - Drug Therapy, Combination
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy/etiology/immunology
MH  - Immunosuppressive Agents/*administration & dosage/adverse effects
MH  - Lupus Erythematosus, Systemic/complications/*drug therapy/immunology
MH  - Male
MH  - Middle Aged
MH  - Mixed Connective Tissue Disease/complications/*drug therapy/immunology
MH  - Predictive Value of Tests
MH  - Pulse Therapy, Drug
MH  - Retrospective Studies
MH  - Survival Rate
MH  - Treatment Outcome
MH  - Vasodilator Agents/therapeutic use
EDAT- 2008/02/02 09:00
MHDA- 2008/04/09 09:00
CRDT- 2008/02/02 09:00
PHST- 2008/02/02 09:00 [pubmed]
PHST- 2008/04/09 09:00 [medline]
PHST- 2008/02/02 09:00 [entrez]
AID - 10.1002/art.23303 [doi]
PST - ppublish
SO  - Arthritis Rheum. 2008 Feb;58(2):521-31. doi: 10.1002/art.23303.