Abstract
We report the case of a 40-year-old patient with systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap syndrome with pulmonary arterial hypertension (overlap-PAH) that was successfully treated with a combination of immunosuppressive therapy and the soluble guanylate cyclase stimulator riociguat. She was diagnosed with mixed connective tissue disease (MCTD) two years prior to admission. She was admitted to our hospital with dyspnea on exertion and progressive skin sclerosis. She fulfilled both SLE and SSc classification criteria and was re-diagnosed with overlap syndrome. The tricuspid valve pressure gradient (TRPG) on echocardiography was 64 mmHg at admission. On right heart catheterization, mean pulmonary arterial pressure (mPAP) was 43 mmHg and pulmonary capillary wedge pressure was 15 mmHg. We diagnosed her with SSc-SLE overlap-PAH and started treatment with corticosteroids and intravenous cyclophosphamide. We also started treatment with riociguat because we speculated she had a component of SSc-PAH and that immunosuppressive therapy alone may be insufficient. We chose riociguat because of its favorable treatment effect on SSc-PAH. Two months after treatment, her TRPG improved to 33 mmHg and the skin sclerosis improved dramatically, suggesting the efficacy of multi-drug treatment and the importance of early intervention.
Highlights
Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), categorized as group I pulmonary hypertension (PH) [1], is often life-threatening
A previous study demonstrated that patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) have a lower survival rate, while those with systemic lupus erythematosus-associated PH (SLE-PAH) have a similar survival rate as those with idiopathic PAH [6]
This is possibly because SSc-PAH is unresponsive to immunosuppressive therapy, is less responsive to PAH-specific therapy, and has other factors that affect prognosis, such as the component of interstitial lung disease and/or left heart disease-associated PH (LHD-PH)
Summary
Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), categorized as group I pulmonary hypertension (PH) [1], is often life-threatening. We started riociguat two weeks after admission because she fulfilled SSc classification criteria [10] and we speculated that immunosuppressive therapy alone is insufficient because her PAH was derived from components of both SLE and SSc. After two months of treatment, her symptoms and laboratory data improved: CPK declined to 31 IU/L, C3 increased to 104 mg/dL, NT-pro BNP declined to 109 ng/dL, urinary total protein to urinary creatinine declined to 0.43 g/g creatinine, CTR on the chest radiograph declined to 37%, TRPG declined to 33 mmHg on follow-up echocardiogram, 6MWT improved to 400 m, and WHO-FC improved to class I; after this time, she was discharged. WBC, white blood cell; Neu, neutrophil; Ly, lymphocyte; RBC, red blood cell; Hb, hemoglobin; MCV, mean corpuscular volume; PLT, platelet; LAC, lupus anticoagulant; Alb, albumin; T-Bil, total bilirubin; LDH, lactose dehydrogenase; AST, aspartate transaminase; ALT, alanine transaminase; BUN, blood urea nitrogen; Cre, creatinine; UA, uric acid; Na, serum sodium; K, serum potassium; Cl, serum chloride; APTT, activated partial thromboplastin time; PT-INR, international normalized ratio of prothrombin time; CRP, Creactive protein; NT-proBNP, N-terminal pro-brain natriuretic peptide; TSH, thyroid stimulating hormone; FT4, free thyroxine; C, complement; CH50, hemolytic complement activity; C1q, immune complex; Ig, immunoglobulin; ANA, anti nuclear antibody; H. pylori, helicobacter pylori; DRVVT, Russell's viper venom time; KCT, kaolin clotting time; U1RNP, U1 ribonucleoprotein; Ab, antibody; dsDNA, double-stranded DNA; Sm, Smith; Scl, scleroderma; RNApIII, RNA polymerase 3; MDA-5, melanoma differentiation associated gene 5; ARS, aminoacyl transfer RNA synthetase; PA-IgG, platelet associated antibody IgG; UTP/UCre, urine protein to creatinine ratio
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