Çocuklarda Raynaud Fenomeni ve Birincil ve İkincil Raynaud Hastalarının Karşılaştırılması

Abstract

Amaç: Raynaud fenomeni (RF), vücudun uç kısımlarında solukluk, siyanoz ve kızarıklıkla karakterize vazospastik bir durumdur. Altta yatan yapısal bir vasküler hastalık veya kolajen doku hastalığı olup olmamasına göre birincil ve ikincil RF olarak sınıflanır. Çalışmada, RF tanısı ile takipli çocuk olgularımızın klinik, laboratuvar ve kapilleroskopik özelliklerini özetlemeyi amaçladık.Gereç ve Yöntemler: Çalışmaya 2014-2016 yılları arasında Hacettepe Üniversitesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Romatoloji Bilim Dalı’nda RF tanısı ile takip edilen 0-18 yaş arası 35 olgu dahil edildi.Bulgular: Hastaların 26’sında (%74.3) birincil; 9’unda (%25.7) ikincil RF saptandı. Kız/erkek oranı 1.7’di. Hastaların medyan (minimum-maksimum) tanı yaşı 13 (5.5-16)’tü. 19 hastada (%54.3) bifazik, 16 hastada ise (%45.7) trifazik Raynaud saptandı. İkincil Raynaud’lu olguların semptom başlangıç ve tanı yaşlarının daha erken olduğu (10 ve 10.5 yaşa karşı 13 ve 13.75 yaş), anti nükleer antikor ve ekstrakte edilebilir nükleer antijene karşı antikor pozitifliğinin daha sık olduğu (ANA için %55.6’ya karşı %15.4; ENA için %55.6’ya karşı %0), eritrosit sedimantasyon hızı yüksekliğinin daha sık (%77.8’e karşı %0), kapilleroskopide geniş kapil görülmesi durumunun daha sık (%66.7’ye karşı %19.2), olduğu görüldü (p<0.05).Sonuç: Bu çalışmada, RF’li olgularımızda daha çok birincil RF olduğunu gördük. İkincil RF’si olan olgularda ise hastalık daha erken dönemde belirti vermekteydi ve kapilleroskopide anormal bulgu oranı daha yüksekti. Ayrıca eritrosit sedimentasyon hızı ve bazı otoantikorların birincil ve ikincil RF’li olguları ayırmada yardımcı laboratuvar parametreler olduğu sonucuna ulaştık. Daha sonra yapılacak ileri dönük, çok merkezli çalışmalarla, bu hastalarda hastalığın gidişi ile ilgili daha çok bilgi edinilebilir ve hastaların daha etkin bir biçimde tedavi edilmesi mümkün olabilir.

Keywords

• Çocuk, Raynaud fenomeni, Romatizmal hastalıklar

Comparing the Characteristics in Children with Primary and Secondary Raynaud’s Disease

Abstract

Objective: Raynaud phenomenon (RP) is a vasospastic condition characterized by pallor, cyanosis, and erythema in the acral parts of body. It is classified as primary and secondary RP according to the presence or absence of underlying vascular or connective tissue disease. We aimed to summarize the clinical, and capillaroscopic characteristics of our patients. Material and Methods: Thirty-five pediatric patients with RP followed at the Departments of Pediatrics, Division of Rheumatology, Hacettepe University, between 2014-2016 were included in the study.Results: Twenty-six patients had primary and nine had secondary RP. The female/male ratio was 1.7. The median (min-max) age at diagnosis was 13 years. The pattern of RP was biphasic in 19 and triphasic in 16 patients. In patients with secondary RP, the age values at symptom onset and diagnosis were younger (10 vs. 10.5 years and 13 vs. 13.75 years, respectively) and antinuclear antibody, extracted nuclear antigen antibody positivity (55.6% vs 15.4% and 55.6% vs 0%, respectively), high erythrocyte sedimentation rate (ESR) (77.8% vs 0%), and dilated capillaries on capillaroscopy were seen more frequently (66.7% vs 19.2%) when compared to primary RP patients.Conclusion: In this study, we found that primary RP was more common. The disease onset was earlier and abnormal findings on capillaroscopy was more common in secondary RP patients. In addition, we demonstrated that ESR and some autoantibodies could be useful laboratory parameters to distinguish between primary and secondary RP. Future multicenter studies could provide more information about the disease course and aid in treating these cases more effectively

Keywords

• Child, Raynaud phenomenon, Rheumatic diseases

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