Ebru OZDEN YİLMAZ, DÖNDÜ ÜSKÜDAR CANSU, CENGİZ KORKMAZ

Sklerodermalı Hastalarımızın Klinik ve Laboratuvar Sonuçları ve Prognozu: Tek Merkez Deneyimi

Sistemik skleroderma (SSk) progresif fibrozisle giden nadir görüln bir bağ dokusu hastalığıdır. Hastalıkta özellikle akciğer, kalp ve böbrek tutulumu önemli morbidite ve mortalite nedeni iken cilt tutulumu, Raynaud fenomeni (RF) gibi tutulumlar da morbiditeyi arttırarak yaşam kalitesini arttırabilmektedir. Bu çalışma ile amacımız, Romatoloji bölümünde takip edilen SSk’lı hastalarımızn demografik özellikleri yanında organ tutulumları ve prognozlarını belirleyerek literatür verileri ile kıyaslamaktı. Bunun için bölümümüzde takip edilen 79 hastanın verileri retrospektif olarak değerlendirildi. 79 hastanın 74 (%93.7)’ü kadın, hastaların yaş ortalaması 54.3 ± 12.7 (22-76) yıl, hastalık süreleri ise ortalama 7.29±7.02 yıl idi. 71 (%89.8)’inde sınırlı kutanöz SSk (skSSk), 6 (%7.6)’sında diffüz kutanöz SSk (dkSSk) ve 2 (%2.5)’sinde ise sine-skleroderma mevcuttu. Hastaların kümülatif organ tutulumu değerlendirildiğinde 73 (%92.4)’ünde RF, 63 (%79.7)’sinde sklerodaktili, 14 (%17.7)’sinde dijital ülser ve 22 (%27.8)’inde telenjiektazi mevcut idi. Hastaların 8 (%10.1)’inde eklem şikayeti mevcut idi. Hastaların 49 (%62)’unda antinükleer antikor (ANA) (+), 31 (%39.2)’inde anti-Scl-70 (+) idi. 74 hasta (%93.7) halen yaşamakta olup 5 (%6.3)’i ölmüştü. Mortalite üzerine etkili olabilecek hastalıkla ilişkili bir faktör saptanmadı (p>0.05). Hastaların 4 (%5.1)’ünde malignite geliştiği görüldü. Kanser gelişimi üzerine etkili olabilecek faktör olarak sadece yüksek rezolusyonlu bilgisayarlı akciğer tomografisi (HCRT) çekim sayısı saptandı (OR 1.74, p=0.023 (CI 1.081-2.829). Sonuç olarak SSk ile takip ettiğimiz hastalarda mortalite ve malignite nedenleri literatüre benzer şekilde iken literatürden farklı olarak sadece akciğer tomografi çekim sıklığı ile malignite gelişimi arasında ilişki saptadık. Bu sonucun daha büyük hasta sayılarını içeren çalışmalarla desteklenmesi gerekmektedir.

Anahtar Kelimeler:

Skleroderma, prognoz, malignite

Clinical and Laboratory Results and Prognosis Patients with Scleroderma: A Single Center Experience

Systemic scleroderma (SSc) is a rare connective tissue disease accompanied by progressive fibrosis. Especially pulmonary, cardiac and renal involvement is a major cause of morbidity and mortality in the disease while skin involvement and Raynaud's phenomenon (RP) can also decrease the quality of life by increasing morbidity. In this study, our aim was to determine the demographics, organ involvement and prognosis of our SSc patients followed up in Rheumatology department and compare the data with the literature. For this purpose, the data of 79 patients who were followed up in our department were evaluated retrospectively. 74 (93.7%) of the 79 patients were female, the mean age of the patients was 54.3 ± 12.7 (22-76) years and the mean duration of disease was 7.29 ± 7.02 years. 71 (89.8%) had limited cutaneous SSc (lcSSc), 6 (7.6%) had diffuse cutaneous SSc (dcSSc) and 2 (2.5%) had sine scleroderma. When the cumulative organ involvement of the patients was evaluated, it was seen that 73 (92.4%) had RP, 63 (79.7%) sclerodactyly, 14 (17.7%) digital ulcer and 22 (27.8%) telangiectasia. Eight (10.1%) patients had articular complaints. Antinuclear antibody (ANA) was (+) in 49 (62%) of the patients and anti-Scl-70 was (+) in 31 (39.2%). 74 patients (93.7%) were still alive and 5 (6.3%) died. No disease-related factor was found to be effective on mortality (p> 0.05). Malignancy was developed in 4 (5.1%) patients. Only high resolution computed chest tomography (HRCT) shot count were found to be an effective factor for cancer development (OR 1.74, p = 0.023 (CI 1.081-2.829). In conclusion, causes of mortality and malignancy were similar with the literature in patients we followed up with SSc. We found a correlation between the incidence of chest CT and the malignancy development unlike the literature. This result should be supported by studies involving larger patient numbers.

Keywords:

Scleroderma, prognosis, malignancy,

PDF

___

1.Bhattacharyya S, Wei J, Varga J. Understanding fibrosis in systemic sclerosis: shifting paradigms, emerging opportunities. Nat Rev Rheumatol. 2012;8:42-54.2.Liakouli V, Cipriani P, Marrelli A, Alvaro S, Ruscitti P, Giacomelli R. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmun Rev. 2011;10:590-4.3.LeRoy EC. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202-6.4.Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809-155.Kaşifoğlu T, Bilge ŞY, Yıldız F, Özen G, Pehlivan Y et al. Risk factors for malignancy in systemic sclerosis patients. Clin Rheum. 2016;35:1529-33.6.Khanna D, Denton CP, Lin CJ, van Laar JM, Frech TM, Anderson ME et al. Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis. 2018;77:212-220.7.Arandia NI, Pilar C, Castillo M, Argüelles DC, Martínez LT, Hernández G et al. Influence of antibody profile in clinical features and prognosis in a cohort of Spanish patients with systemic sclerosis. Clin Exp Rheumatol. 2017;35:98-105.8.Talotta R, Bongiovanni S, Letizia T, Rigamonti F, Ditto MC, Atzeni F et al. Measurement of Serum Klotho in Systemic Sclerosis. Send to Dis Markers. 2017;2017:9545930.9.Radić M, Kaliterna DM, Fabijanić D, Radić J. Prevalence of systemic sclerosis in Split–Dalmatia county in Southern Croatia. Clin Rheum. 2010;29:419-21.10.Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol. 2012;8:469-79.11.Denton C, Korn J. Digital ulceration and critical digital ischemia in scleroderma. Scleroderma Care Res. 2003;1:12-6.12.Walker U, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis. 2007;66:754-63.13.Mukerjee D, St George D, Coleiro B, Knight C, Denton C, Davar J et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62:1088-93.14.Steen V, Medsger TA. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum. 2003;48:516-22.15.Varga J, King Jr TE, Axford JS, Hollingsworth H. Overview of pulmonary complications of systemic sclerosis (scleroderma). Up to date.com Literature review current through: Oct 2017.16.Arakkal G, Chintagunta SR, Chandika V, Damarla SV, Manchala S, Kumar BU. Cardio-pulmonary involvement in systemic sclerosis: A study at a tertiary care center. Indian J Dermatol Venereol Leprol.. 2017;83:677.17.Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE, Clements PJ et al. High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 2008;134:358-67.18.Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, et al. A multicenter, prospective, randomized, double‐blind, placebo‐controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum.. 2006;54:3962-70.19.Al-Dhaher FF, Pope JE, Ouimet JM. Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum. 2010;39:269-277.20.Rubio-Rivas M, Royo C, Simeón CP, Corbella X, Fonollosa V. Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum. 2014;44:208-219. 21.Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66:940-4.22.Elhai M, Meune C, Avouac J, Kahan A, Allanore Y. Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology. 2011;51:1017-26.23.Monaco AL, Bruschi M, La Corte R, Volpinari S, Trotta F. Epidemiology of systemic sclerosis in a district of northern Italy. Clin Exp Rheumatol. 2011;29:10.24.Rosenthal AK, McLaughlin JK, Linet MS, Persson I. Scleroderma and malignancy: an epidemiological study. Ann Rheum Dis. 1993;52:531-3.25.Zeineddine N, El Khoury L, Mosak J. Systemic sclerosis and malignancy: a review of current data. J Clin Med Res. 2016;8:625.26.Olesen AB, Svaerke C, Farkas D, Sørensen HT. Systemic sclerosis and the risk of cancer: a nationwide population‐based cohort study. Br J Dermatol. 2010;163:800-6.27.Colaci M, Giuggioli D, Sebastiani M, Manfredi A, Vacchi C, Spagnolo P et al. Lung cancer in scleroderma: results from an Italian rheumatologic center and review of the literature. Autoimmun Rev. 2013;12:374-9.28.de González AB, Mahesh M, Kim K-P, Bhargavan M, Lewis R, Mettler F et al. Projected cancer risks from computed tomographic scans performed in the United States in 2007. Arch Intern Med. 2009;169:2071-7.