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

Year 2018, Volume: 40 Issue: 3, 70 - 78, 01.09.2018

Ebru Özden Yılmaz Döndü Üsküdar Cansu , Cengiz Korkmaz

https://doi.org/10.20515/otd.415387

Cited By: 1

Abstract

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

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

Abstract

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.

 

Keywords

Skleroderma, prognoz, malignite

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