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Communications to the Editor |

IV Epoprostenol for Systemic Sclerosis FREE TO VIEW

Elizabeth S. Klings, MD; Harrison W. Farber, MD
Author and Funding Information

Affiliations: The Pulmonary Center Boston University School of Medicine Boston, MA,  Director, New York Presbyterian Pulmonary Hypertension Center Columbia University College of Physicians and Surgeons New York, NY

Correspondence to: Elizabeth S. Klings, MD, Pulmonary Center R-304, Boston University, 715 Albany St, Boston, MA 02118; e-mail: bchklingon@aol.com



Chest. 2000;118(3):881-882. doi:10.1378/chest.118.3.881
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Published online

To the Editor:

We read with interest the editorial of Dr. Barst (January 2000)1on the use of IV epoprostenol for treatment of pulmonary hypertension related to connective tissue diseases. We would like to refer her and your readers to our study evaluating both short- and long-term effects of IV epoprostenol for the treatment of systemic sclerosis (SSc)-associated pulmonary hypertension.2 In our study of 16 patients, 15 of 16 patients had a diagnosis of limited SSc; none had significant interstitial lung disease, suggesting that they all had a vasculopathy similar pathologically to primary pulmonary hypertension (PPH). Although only 3 of 16 patients (18.8%) demonstrated an acute response to either nitric oxide, calcium channel blockers, or adenosine, 13 of 16 patients (81.3%) had a therapeutic response to epoprostenol (defined as a reduction in pulmonary vascular resistance [PVR] by ≥ 25%). These patients have been followed as outpatients for up to 3 years. All of the initial responders have had improvement in symptoms and exercise tolerance as defined by New York Heart Association class. Three of the initial responders (18.8%) have subsequently died of progressive right heart failure while on epoprostenol. Repeat right heart catheterizations have been performed annually in six patients; all continue to exhibit persistent reductions in PVR.23 Although concern was raised regarding a higher rate of infection and other complications in this patient group,4 this has not been the case, as only 1 of 16 patients (6.3%) has developed bacteremia secondary to the presence of an indwelling catheter.

Several studies have alluded to survival differences in the PPH and SSc populations with epoprostenol treatment5; our experience although limited, does not support this. We agree that a larger group of patients studied for a longer period of time is necessary to make this statement more definitive. In conclusion, we concur that pulmonary hypertension is a relatively common complication of SSc, particularly the limited cutaneous variant, with a lack of viable treatment options and a high mortality rate. In light of these factors and the results of our study, we are optimistic that the use of IV epoprostenol will have a significant impact on the natural history of pulmonary hypertension related to SSc.

References

Barst, RJ (2000) Experience and reason [editorial].Chest117,2-5. [CrossRef] [PubMed]
 
Klings, ES, Hill, NS, Ieong, MH, et al Systemic sclerosis-associated pulmonary hypertension: short- and long-term effects of epoprostenol (prostacyclin).Arthritis Rheum1999;42,2638-2645. [CrossRef] [PubMed]
 
Klings, ES, Ieong, MH, Simms, RW, et al Long-term intravenous epoprostenol therapy for pulmonary hypertension associated with systemic sclerosis [abstract]. Am J Respir Crit Care Med. 2000;;161 ,.:A459
 
Humbert, M, Sanchez, O, Fartoukh, M, et al Treatment of severe pulmonary hypertension secondary to connective tissue disease with continuous IV epoprostenol (prostacyclin).Chest1998;114(suppl),80S-82S
 
Sitbon, O, Humbert, M, Sanchez, O, et al Survival in pulmonary hypertension associated with connective tissue diseases (PH-CTD) treated with long-term epoprostenol (PGI2): comparison with primary pulmonary hypertension (PPH) [abstract].Am J Respir Crit Care Med1999;159,A158
 
To the Editor:

Thank you for reporting your experience with chronic IV epoprostenol in patients with pulmonary hypertension associated with systemic sclerosis. I apologize if my editorial was misinterpreted to imply that chronic IV epoprostenol therapy should not be considered as a therapeutic option for patients with pulmonary hypertension associated with various connective tissue disorders. My comments were only meant to alert physicians to our not currently having the same experience with these patients as we have with patients who have primary pulmonary hypertension. We obviously are cautiously optimistic and hopeful that chronic IV epoprostenol will be efficacious for various groups of patients with pulmonary hypertension associated with other disorders, including collagen vascular diseases, particularly in light of many of these patients not being candidates for transplantation due to their associated collagen vascular diseases. Your experience appears similar to previously published experiences with these patients, eg, chronic IV epoprostenol does appear to be efficacious, although whether or not its efficacy is as good as we and others have seen with primary pulmonary hypertension remains to be determined. In the meantime, consideration of looking at these patients in a registry may allow us to evaluate the relative efficacy of epoprostenol and rank the efficacy of chronic IV epoprostenol for various groups of patients with pulmonary vascular disease, including primary pulmonary hypertension, pulmonary hypertension associated with the various collagen vascular disorders (subdivided into the specific collagen vascular disorders, since their responses may be different), as well as pulmonary hypertension associated with portal hypertension, HIV, and appetite suppressant-induced primary pulmonary hypertension.


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References

Barst, RJ (2000) Experience and reason [editorial].Chest117,2-5. [CrossRef] [PubMed]
 
Klings, ES, Hill, NS, Ieong, MH, et al Systemic sclerosis-associated pulmonary hypertension: short- and long-term effects of epoprostenol (prostacyclin).Arthritis Rheum1999;42,2638-2645. [CrossRef] [PubMed]
 
Klings, ES, Ieong, MH, Simms, RW, et al Long-term intravenous epoprostenol therapy for pulmonary hypertension associated with systemic sclerosis [abstract]. Am J Respir Crit Care Med. 2000;;161 ,.:A459
 
Humbert, M, Sanchez, O, Fartoukh, M, et al Treatment of severe pulmonary hypertension secondary to connective tissue disease with continuous IV epoprostenol (prostacyclin).Chest1998;114(suppl),80S-82S
 
Sitbon, O, Humbert, M, Sanchez, O, et al Survival in pulmonary hypertension associated with connective tissue diseases (PH-CTD) treated with long-term epoprostenol (PGI2): comparison with primary pulmonary hypertension (PPH) [abstract].Am J Respir Crit Care Med1999;159,A158
 
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