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+A 34-year-old Japanese woman had a diagnosis of MCTD with the presence of Raynaud's phenomenon, pancytopenia, elevated plasma creatinine kinase, and antibodies against U1-ribonucleoprotein in 2005 and received 10 mg of prednisolone daily.
+Pulmonary arterial systolic pressure estimated by echocardiography was slightly elevated (48 mmHg) in 2006.
+She felt progressive shortness of breath on physical effort in 2011.
+Pulmonary arterial hypertension was diagnosed with 74 mmHg of mean pulmonary arterial pressure (mPAP) evaluated by right heart catheterization (RHC) in 2012.
+Cyclophosphamide-based immunosuppressive therapy was proposed but refused because of possible early menopausal symptoms.
+Treatment with 250 mg/day of bosentan and 120 μg/day of beraprost was initiated.
+However, in January 2014, her dyspnea deteriorated (WHO functional class IV), and she was admitted to our hospital.
+A physical examination showed low systemic blood pressure (89/61 mmHg), tachycardia (104/min), low blood oxygen level (percutaneous oxygen saturation (SpO2) 95%), jugular venous distension, and severe systemic edema.
+Coarse crackles and loud pulmonic valve closure sounds were detected.
+She was unable to undergo the 6-minute walk test due to severe dyspnea.
+Laboratory examinations showed elevated plasma brain natriuretic peptide (BNP) level (929.4 pg/mL), hypoxemia (PaO2 62.5 mmHg, PaCO2 27.1 mmHg), and the presence of antibodies against U1-ribonucleoprotein (97.9 U/mL) as well as antinuclear antibodies (1:640, speckled pattern).
+A chest X-ray showed enlarged pulmonary arteries and cardiomegaly (Figure).
+Electrocardiogram showed P wave elevation in the V1-V2 leads.
+RHC demonstrated elevated mPAP (65 mmHg) with normal pulmonary arterial wedge pressure (12 mmHg), high pulmonary vascular resistance (1,547 dyne・sec・cm-5), and decreased cardiac output (cardiac index 1.69 L/min/m2).
+A ventilation/perfusion scan showed no signs of pulmonary thromboembolism.
+No signs of chronic obstructive pulmonary disease or interstitial lung diseases were found with computed tomography.
+Her dyspnea was therefore considered to be due to MCTD-associated PAH which was refractory to bosentan and beraprost therapy.
+In addition to supportive therapy with oxygen supplementation and diuretics (40 mg/day of furosemide), initiation of 60 mg/day of sildenafil as well as gradual increment in the dose of beraprost was chosen as an additional treatment.
+However, her dyspnea remained unchanged, and her plasma BNP level increased.
+IVCY (750 mg/m2, every 4 weeks) was initiated, and the dose of prednisolone was increased to 60 mg daily.
+Her dyspnea then ameliorated, and she became able to undergo the 6-minute walk test (260 m, minimum SpO2 95 %).
+Her plasma BNP level also decreased after the initiation of IVCY (Figure).
+Six months after the admission, a follow-up RHC revealed improved mPAP (65 to 53 mmHg), pulmonary vascular resistance (1,547 to 1,116 dyne・sec・cm-5), and cardiac index (1.69 to 1.83 L/min/m2) (Table).
+Since she remained stable but did not obtain further improvements 18 months after the admission (Table), we consider parenteral prostanoids or lung transplantation as a subsequent treatment.