The relationship between skin findings and capillaroscopic evaluation due to peripheral vascular involvement and lung disease in patient with scleroderma

Introduction: Scleroderma is a connective tissue disease characterized by inflammatory, vascular and fibrotic changes in the skin and various organs. Lung involvement is estimated to develop in 70-85% of patients and is an important cause of mortality. Objective: To investigate the relationship between skin involvement, physical examination and capillaroscopic evaluation findings and serological subtypes and lung involvement in patients with systemic sclerosis. Materials and Methods: In this study, 78 patients with systemic sclerosis, who are being followed up in the Division of Rheumatology, Department of Internal Medicine of Akdeniz University Faculty of Medicine, were evaluated retrospectively. Patients aged 18 years and older, followed in the Division of Rheumatology, Department of Internal Medicine, Akdeniz University Medical Faculty Hospital, diagnosed with systemic sclerosis according to the 2013 American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) classification criteria were included in the study. Information on demographic characteristics, comorbidities, clinical and examination findings, capillaroscopy evaluations, laboratory and radiology results, pulmonary function tests, transthoracic echocardiography reports and treatments of the patients were obtained from patient files and hospital information management system. During the follow-up period, skin involvement and vasculopathy findings, capillaroscopic evaluations, respiratory parameters, transthoracic echocardiographic findings and the treatments they received were examined. It was examined whether there was a radiological and functional difference in lung involvement with skin involvement and capillaroscopy evaluations. The examination of the patients was based on Raynaud's phenomenon, sclerodactyly, presence of digital ulcer, capillaroscopy and simultaneous lung evaluation. Results: Of the 78 scleroderma patients included in the study, 74 (94.9%) were female and 4 (5.1%) were male. The mean age of the patients was 52.7 and 80.8% of the patients were more than 5 years old disease age. According to the results of capillaroscopy; 44.9% (35 patients) of the patients had early scleroderma pattern, 29.5% (23 patients) had active scleroderma pattern, 25.6% (20 patients) had late scleroderma pattern. There was sclerodactyly in 72 of the patients (92.3%). Raynaud's phenomenon was present in 68 patients (87.2%). Digital ulcer was observed in 25 patients (52.5%). In serological evaluation, ANA was positive in 67 patients (87%). In patients with ANA positivity, as subtypes, centromeric staining was observed in 24 patients (34.8%), nucleolar staining in 34 patients (49.3), and granular staining in 8 patients (11.6%). In 55 patients (70.5%), lung findings related to the disease were seen on HRCT. The FVC value (Mean: 69.2) in patients with HRCT was lower than those without (Mean:80.13) (p:0.009). Although the relationship between HRTC and Capillaroscopy variables was found to be statistically insignificant, the effect size was moderate (Φ: 0.252). A statistically significant relationship was found between digital ulcer and capillaroscopy variables (p:0.001). In paired comparisons (with Bonferroni correction), the rate of capillaroscopy 1 was found (67.3%) in patients without digital ulcer. In those with digital ulcers, the rate of capillaroscopy 3 (64%) was higher than those without digital ulcers (7.7%). The FVC value (Median: 62) and DLCO value (Mean: 51) in those with digital ulcers were lower than the FVC (Median: 83) and DLCO values (Mean: 65) in those without digital ulcers (p:0.05). The rate of ANA subtype centromeric staining in patients with HRCT findings (76.9%) was found to be higher than in other involvements (21.1%) (p:0.001). The rate of having HRTC findings in patients with six types of nucleolar (88.2%) was found to be statistically significantly higher than those with subtype centromeric (37.5%) (p:0.001). Although the incidence of digital ulcers in patients with nucleolar staining (41.2%) was not statistically significantly higher than in those with centromeric involvement (17.4%), the effect size was moderate (Φ: 0.251). FVC values (Median: 73) of those with ANA nucleolar involvement were significantly lower than those with centromeric (Median: 83.5) (p=0.002). When the treatments received by the patients were grouped according to the presence of pulmonary findings in HRCT, the use of mycomenolate mofetil (MMF) (p:0.004), bosentan use (p:0.037), and nintedanib (p:0.028) were found to be significantly higher. Conclusion: When evaluated retrospectively in this study, a statistically significant deterioration in capillaroscopy and respiratory tests such as FVC and DLCO was observed in patients with scleroderma lung involvement demonstrated by HRCT compared to patients without lung involvement. More lung involvement was observed with HRCT, especially in people with advanced disease age, and capillaroscopy findings were found to be advanced in these people. These findings show that lung involvement is more common as the disease progresses and that capillaroscopy findings and lung functions worsen in these patients. The presence of a digital ulcer was also seen to be associated with advanced capillaroscopy findings, and lung function tests were found to be low in the presence of a digital ulcer. The relationship between capillaroscopy and HRCT -which was not affected by pulmonary artery pressure- suggests that capillaroscopy may be an independent predictive factor for lung progression. Key Words: Scleroderma, Capillaroscopy, Interstitial Lung Disease