J Rheum Dis.  2017 Apr;24(2):114-118. 10.4078/jrd.2017.24.2.114.

Pulmonary Arterial Hypertension is Normalized Following Six Years of Inhaled Iloprost Treatment in a Patient with Systemic Sclerosis

• Yoon SY
• Yoo ES
• Yoo EJ
• Jung JY
• Kim HA
• Suh CH

• KMID: 2378090
• DOI: http://doi.org/10.4078/jrd.2017.24.2.114

Abstract

Pulmonary arterial hypertension is a critical manifestation of systemic sclerosis (SSc) and is a main cause of death. Several treatment modalities for SSc have been identified, with effects that improve quality of life and mortality rates. However, whether these drugs can also normalize pulmonary arterial pressure, remains unclear. Here, we report the case of a woman with diffuse SSc with pulmonary arterial hypertension, who had a functional status equivalent to the New York Heart Association class III. The patient was treated with inhaled iloprost. After six years of inhaled iloprost therapy, echocardiography showed that pulmonary arterial pressure normalized, accompanied by improvement in functional capacity. Inhaled iloprost might not only normalize pulmonary arterial pressure, but also improve the functional status of patients with SSc with pulmonary arterial hypertension.

Keyword

Systemic sclerosis; Pulmonary hypertension; Iloprost

MeSH Terms

Arterial Pressure
Cause of Death
Echocardiography
Female
Heart
Humans
Hypertension*
Hypertension, Pulmonary
Iloprost*
Mortality
Quality of Life
Scleroderma, Systemic*
Iloprost

Figure

• 

  Figure 1. Chest computed tomography. Interstitial pneumonitis and bilateral pleural effusion.

• 

  Figure 2. Improvement in pulmonary arterial hypertension. (A) Reduction in pulmonary arterial pressure (PAP) on echocardiography. (B) Improvement in New York Heart Association (NYHA) class.

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  Figure 3. No significant change in interstitial lung disease during the treatment. (A) Chest radiography at diagnosis. (B) Chest radiography 6 years later.

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