Artikel
Demographic and clinical differences between idiopathic (IPAH) and scleroderma-related pulmonary arterial hypertension (SSc-PAH):Russian National Register analysis
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Autoren
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Ekaterina Nikolaeva
- V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation
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Natalia Yudkina
- V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation
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Olga Arkhipova
- Myasnikov Institute of Clinical Cardiology, Russian Cardiology Research-and-Production Complex, Moscow, Russian Federation
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Ildar Kurmukov
- V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation
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Tamila Martynyuk
- Myasnikov Institute of Clinical Cardiology, Russian Cardiology Research-and-Production Complex, Moscow, Russian Federation
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Aleksandr Volkov
- V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation
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Eugene Nasonov
- V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation
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Irina Chazova
- Myasnikov Institute of Clinical Cardiology, Russian Cardiology Research-and-Production Complex, Moscow, Russian Federation
| Veröffentlicht: | 29. August 2016 |
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Gliederung
Text
Background: SSc-PAH and IPAH belongs to group I for classification of PH, but there is evidence of significant differences in their survival.
The aim was to compare patients with SSc-PAH and IPAH based on Russian Register of Pulmonary hypertension.
Methods: 52 pts with IPAH and 50 with SSc-PAH. Diagnosis was based on RHC.
Results: At the moment of diagnosis patients with SSc-PAH was 15 year older (p<0.0001). 6MWD was somewhat more in IPAH group. Borg index was higher in SSc-PAH (4.2±2.1 vs 3.3±1.5, p<0.049). There were no differences in functional class. Dyspnea was the most common among the first symptoms (94% in SSc-PAH and 73% in IPAH). Syncope was observed only in IPAH. The diagnosis was done late (in SSc-PAH group in 33 (13; 56.5) mts, IPAH – in 30.5 (13.3; 76.3) mts. Heart failure were detected with equal frequency (76% and 65% respectively). Advanced stage PAH including symptoms of HF were detected with equal frequency. Pericardial effusion more frequently in SSc-PAH (56% vs 35%, p<0.05). Also they had more frequently weight lost (14% vs 2%, p<0.05). Hemodynamics estimation revealed significant differences: sRVP (73.4±19.1 mm Hg) and mPAP (49.1±11.5 mm Hg) in SSc-PAH were significantly lower, than in IPAH (85.9±25.6 mm Hg and 57.5±15.3 mm Hg). dRVP and mRVP were significantly higher in SSc (7.02±5.59 and 28.5±12.7 mm Hg), compared to 0.95±7.6 and 11.7±6.0 mm Hg respectively. Whereas CO were equal, the average PVR was higher in IPAH (13.2 vs 10.6 WU, p<0.005). RAP were equal at both groups. DLCO was lower in SSc-PAH group (46.9±13.5 vs 68.5±12.8%). Hb in SSc-PAH was significantly lower (136±22 g/l vs 149±19 g/l), erythrocyte count was almost same is both groups. C-reactive protein in patients with SSc-PAH was higher (9.4±15.1 vs 1.1±1.0 mg/l, p<0.001). Significant decrease in survival during the second and third years of observation was revealed in SSc-PAH. Difference in 5 years survival between the groups was 15% (p=0.06).
Conclusion: Although general clinical appearance, SSc-PAH and IPAH looks similar, there are significant differences in hemodynamic indexes and laboratory signs. This factor leads to difference in progress and outcome of the disease.
