Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension.

Abstract

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.

• Authors: Olivier Sanchez, Olivier Sitbon, Xavier Jaïs, Gérald Simonneau, Marc Humbert
• Journal: Chest (Chest) Vol. 130 Issue 1 Pg. 182-9 (Jul 2006) ISSN: 0012-3692 [Print] United States
• PMID: 16840400 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Glucocorticoids
• Immunosuppressive Agents
• Cyclophosphamide

Topics

• Adult
• Aged
• Blood Pressure (drug effects)
• Connective Tissue Diseases (classification, complications, drug therapy)
• Cyclophosphamide (therapeutic use)
• Exercise Test (methods)
• Female
• Glucocorticoids (therapeutic use)
• Humans
• Hypertension, Pulmonary (drug therapy, etiology, physiopathology)
• Immunosuppressive Agents (therapeutic use)
• Male
• Middle Aged
• Retrospective Studies
• Severity of Illness Index