Nailfold capillaroscopic pattern and modified Rodnan skin score associated with deterioration of right ventricle functions in systemic sclerosis patients without overt pulmonary hypertension

Yıl 2022, Cilt: 3 Sayı: 3, 188 - 194, 26.09.2022

Bekir Çalapkorur , Erkan Demirci , Samet Karahan , Kemal Erol , Tayfun Akalın

https://doi.org/10.47582/jompac.1147325

Öz

Objective: Although nailfold capillaroscopy (NC) and modified Rodnan skin score (mRSS) have already been studied in a variety of contexts related to Systemic Sclerosis (SSc) progression, there is limited data about the relationships between NC, mRSS, and right ventricle (RV) function in SSc patients without overt pulmonary arterial hypertension (PAH). In this study, we examined the relationship between RV function and clinical SSc parameters such as NC pattern and mRSS.
Material and Method: Thirty two patients with SSc and twenty healthy participants as a control group were enrolled in this study. Patients with SSc were assessed for digital ulcers, Raynaud’s phenomenon, and severity of skin involvement by a rheumatology specialist. Also, all participants underwent echocardiographic examinations by cardiology specialists. The echo parameters were measured considering the criteria of the American Society of Echocardiography guidelines.
Results: Systolic pulmonary arterial pressure (sPAP) was statistically higher in the SSc group (26.4±3.2 vs 30.8±3.6 mmHg, p<0.001). Tricuspid annular plane systolic movement (TAPSE), pulmonary accerelation time and mean RV free wall strain were found to be lower in the SSc group (p=0.003, p<0.001, p<0.001 for all). Patients with capillaroscopic active and late pattern and late pattern had significantly lower TAPSE, ventricular isovolumic acceleration (IVA) and RV free wall mean strain (p=0.002, p=0.005, and p<0.001, respectively) compared to patients with capillaroscopic early pattern and active and early pattern In the univariant linear regression analisys, in the SSc group, mRSS was significantly associated with RV free wall mean strain (R2=0.192; p=0.007).
Conclusion: In this study, early deterioration of sPAP and RV functions were shown in SSc patients without overt PAH. Also, high scores of mRSS and abnormal capilloroscopic pattern were associated with worse RV function.

Anahtar Kelimeler

Systemic sclerosis, nailfold capillaroscopy, modified Rodnan skin score, right ventricle strain imaging

Aşikâr pulmoner hipertansiyonu olmayan sistemik sklerozlu hastalarda tırnak kapilaroskopi bulguları ve modifiye Rodnan skoru sağ ventrikül fonksiyonlarında bozulma ile ilişkilidir

Öz

Amaç: Tırnak kapilloskopisi ve modifiye Rodnan skoru sistemik sklerozda (SS) çeşitli durumlarda klinik progresyon ile ilişkili bulunmuş olmasına rağmen sağ ventrikül fonksiyonları ile ilişkileri yeterince çalışılmamıştır. Bu çalışmada aşikar pulmoner hipertansiyonu olmayan sistemik sklerozlu hastalarda sağ ventrikül fonksiyonları ile modifiye Rodnan skoru, tırnak kapillaroskopisi gibi klinik SS klinik parametreleri ile arasındaki ilişki araştırılmıştır.
Gereç ve Yöntem: Otuz iki SS’li hasta ve yirmi sağlıklı birey kontrol grubu için çalışmaya alınmıştır. Hastalar romatoloji uzmanı tarafından dijital ülserler, tırnak kapillaroskopisi ve deri tutulumu için değerlendirilmiştir. Tüm katılımcılara ekokardiyografik değerlendirme kardiyoloji uzmanı tarafından yapılmıştır. Ekokardiyografik parametreler Amerikan Ekokardiyografi Birliğinin kılavuzlarına göre hesaplanmıştır.
Bulgular: Sistolik pulmoner arteriyel SS grubunda istatiksel olarak anlamlı şekilde yüksekti (26,4±3,2 vs 30,8±3,6 mmHg, p=0,001). Triküspit anüler plan sistolik hareketi (TAPSE), pulmoner akselerasyon zamanı ve ortalama sağ ventrikül strain değeri SS grubunda anlamlı olarak daha düşüktü (p=0,003, p=0,001, p=0,001). Kapillaroskopik olarak aktif ve geç patern ve geç paterne sahip hastalarda erken ve aktif paterne ve erken paterne sahip hastalara göre TAPSE, ventriküler isovolumik akselerasyon ve sağ ventrikül ortalama serbest duvar straini anlamlı olarak daha düşüktü (p=0,002, p=0,005, and p=0,001, sırasıyla) Univariat doğrusal regresyon analizde, SS grubunda modifiye Rodnan cilt skoru ile sağ ventrikül serbest duvar ortalama strain arasında anlamlı ilişki vardı (R2=0,192; p=0,007).
Sonuç: Bu çalışmada SS’li hastalarda sistolik pulmoner arteriyel basınçta ve sağ ventrikül fonksiyonlarında erken dönemde bozulma olduğu gösterilmiştir. Ayrıca yüksek modifiye Rodnan cilt skoru ve geç dönem kapillaroskopik değişikliklerin sağ ventrikül fonksiyonlarındaki bozulmayla ilişkili olduğu bulunmuştur.

Anahtar Kelimeler

Sistemik skleroz, tırnak kapillaroskopi, modifiye Rodnan cilt skoru, sağ ventrikül strain görüntüleme

Kaynakça

• Clements PJ, Hurwitz EL, Wong WK, et al. Skin thickness score as a predictor and correlate of outcome in systemic sclerosis: high-dose versus low-dose penicillamine trial. Arthritis Rheum 2000; 43: 2445-54.
• Shand L, Lunt M, Nihtyanova S, et al. Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model. Arthritis Rheum 2007; 56: 2422-31.
• Hesselstrand R, Wildt M, Ekmehag B, et al. Survival in patients with pulmonary arterial hypertension associated with systemic sclerosis from a Swedish single centre: prognosis still poor and prediction difficult. Scand J Rheumatol 2011; 40: 127-32.
• Pirat B, McCulloch ML, Zoghbi WA. Evaluation of global and regional right ventricular systolic function in patients with pulmonary hypertension using a novel speckle tracking method. Am J Cardiol 2006; 98: 699-704.
• Durmus E, Sunbul M, Tigen K, et al. Right ventricular and atrial functions in systemic sclerosis patients without pulmonary hypertension. Herz 2015; 40: 709-15.
• Ingegnoli F, Ardoino I, Boracchi P, et al. EUSTAR co-authors. Nailfold capillaroscopy in systemic sclerosis: data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Microvasc Res 2013; 89: 122-8.
• Minopoulou I, Theodorakopoulou M, Boutou A, et al. Nailfold capillaroscopy in systemic sclerosis patients with and without pulmonary arterial hypertension: a systematic review and meta-analysis. J Clin Med 2021; 10: 1528.
• Lambova S, Hermann W, Müller-Ladner U. Capillaroscopic pattern at the toes of systemic sclerosis patients: does it “tell” more than those of fingers? J Clin Rheumatol 2011; 17: 311-4.
• Low AHL, Ng SA, Berrocal V, et al. Evaluation of Scleroderma Clinical Trials Consortium training recommendations on modified Rodnan skin score assessment in scleroderma. Int J Rheum Dis 2019; 22: 1036-40.
• Erol K, Gok K, Cengiz G, et al. Hand functions in systemic sclerosis and rheumatoid arthritis and influence on clinical variables. Int J Rheum Dis 2018; 21: 249-52
• Khanna D, Furst DE, Clements PJ, et al. Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis. J Scleroderma Relat Disord 2017; 2: 11-8.
• LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988; 15: 202-5.
• Smith V, Vanhaecke A, Herrick AL, et al. Fast track algorithm: How to differentiate a “scleroderma pattern” from a “non-scleroderma pattern. Autoimmun Rev 2019; 18: 102394.
• Quinones MA, Otto CM, Stoddard M, et al. Doppler Quantification Task Force of the Nomenclature and Standards Committee of the American Society of Echocardiography. Recommendations for quantification of Doppler echocardiography: A report from the Doppler Quantification Task force of the Nomenclature and Standards Committee of the American Society of Echocardiography. J Am Soc Echocardiogr 2002; 15: 167–84.
• Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016; 37: 67-119.
• Guerra F, Stronati G, Fischietti C, et al. Global longitudinal strain measured by speckle tracking identifies subclinical heart involvement in patients with systemic sclerosis. Eur J Prev Cardiol 2018; 25: 1598-606.
• Vandecasteele EH, De Pauw M, Brusselle G et al. The heart and pulmonary arterial hypertension in systemic sclerosis. Acta Clin Belg 2016; 71: 1-18. 
• Lindqvist P, Waldenstrom A, Wikstrom G, et al. The use of isovolumic contraction velocity to determine right ventricular state of contractility and filling pressures: a pulsed Doppler tissue imaging study. Eur J Echocardiogr 2005; 6: 264-70.
• Ernande L, Cottin V, Leroux PY, et al. Right isovolumic contraction velocity predicts survival in pulmonary hypertension. J Am Soc Echocardiogr 2013; 26: 297-306.
• Bratis K, Lindholm A, Hesselstrand R, et al. CMR feature tracking in cardiac asymptomatic systemic sclerosis: Clinical implications. PLoS One 2019; 14: e0221021. 
• Felekos I, Aggeli C, Gialafos, et al. Global longitudinal strain and long-term outcomes in asymptomatic extracardiac sarcoid patients with no apparent cardiovascular disease. Echocardiography 2018; 35: 804-8.
• Kemal HS, Kayikcioglu M, Kultursay H, et al. Right ventricular free-wall longitudinal speckle tracking strain in patients with pulmonary arterial hypertension under specific treatment. Echocardiography 2017; 34: 530-6. 
• Kusunose K, Fujiwara M, Yamada H, et al. Deterioration of biventricular strain is an early marker of cardiac involvement in confirmed sarcoidosis. Eur Heart J Cardiovasc Imaging 2020; 21: 796-804. 
• Steen VD, Medsger TA Jr. Improvement in skin thickening in systemic sclerosis associated with improved survival. Arthritis Rheum 2001; 44: 2828-35.
• Foocharoen C, Mahakkanukrauh A, Suwannaroj S, et al. Pattern of skin thickness progression and clinical correlation in Thai scleroderma patients. Int J Rheum Dis 2012; 15: e90-5.
• Riccieri V, Vasile M, Iannace N, et al. Systemic sclerosis patients with and without pulmonary arterial hypertension: a nailfold capillaroscopy study. Rheumatology 2013; 52: 1525-8.