Mortality and Associated Factors in Patients with Systemic Sclerosis Associated Pulmonary Hypertension with and without Interstitial Lung Disease: A Long-Term Follow-up Study.
Shirkhan Amikishiyev, Yasemin Yalçınkaya, Konul Mammadova, Numune Aliyeva, Gorkem Durak, Bahar Artim-Esen, Ahmet Gül, Ahmet Kaya Bilge, Gulfer Okumuş, Murat Inanc
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Abstract
Objectives: We aimed to analyze the prevalence, mortality, and prognostic factors in systemic sclerosis (SSc) patients with pulmonary hypertension (PH) with or without interstitial lung disease (ILD).
Methods: The associations between mortality and demographics, transthoracic echocardiography, right heart catheterization (RHC), pulmonary functional parameters at baseline, and treatment modalities in two groups; patients with pulmonary arterial hypertension (PAH, PH without significant ILD) and PH + ILD were evaluated.
Results: The mean age of the patients was 56.6±13.5 (range: 34-82, 44 women/ 2 men), and 23 (52.3%) were deceased during a median follow-up of 45 months. The survival rates of PH-SSc patients were 91% for the first year, 75% for 2 years, 68% for 3 years, and 43.1% for 5 years. The majority of deceased patients were in the PH + ILD group (p=0.007). The PH + ILD group had more diffuse skin involvement, anti-Scl-70 positivity, high C-reactive protein, low FVC, and lower DLCO values. The deceased patients had higher ePASP, low CO, and FVC values compared to surviving patients. Median survival time was significantly better in patients on combined therapy (44 vs. 61 months, p=0.01). The mortality-related factors in the PH + ILD group were decreased initial FVC, high ePASP on echocardiography, low cardiac output on RHC, deteriorated functional class, and monotherapy.
Conclusion: This is the first reported SSc-PH cohort from Turkey by a multidisciplinary team after the implementation of PAH-specific drugs. SSc-PH is a severe complication of SSc with high mortality especially in patients with accompanying severe ILD.
期刊介绍:
Modern Rheumatology publishes original papers in English on research pertinent to rheumatology and associated areas such as pathology, physiology, clinical immunology, microbiology, biochemistry, experimental animal models, pharmacology, and orthopedic surgery.
Occasional reviews of topics which may be of wide interest to the readership will be accepted. In addition, concise papers of special scientific importance that represent definitive and original studies will be considered.
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