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

Öz

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.

  

Anahtar Kelimeler

• Scleroderma,prognosis,malignancy

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

Öz

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

Kaynakça

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