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Çocuklarda Raynaud Fenomeni ve Birincil ve İkincil Raynaud Hastalarının Karşılaştırılması

Yıl 2018, Cilt: 12 Sayı: 1, 39 - 43, 01.04.2018

Öz

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.

Kaynakça

  • Wigley FM, Flavahan NA. Raynaud’s Phenomenon. N Engl J Med 2016;375:556-65.
  • Fuhlbrigge R. In: Textbook of Pediatric Rheumotology. Petty RE (ed). Philadelphia: Elsevier, 2016;436-47.
  • Pavlov-Dolijanovic S, Damjanov NS, Stojanovic RM, Vujasinovic Stupar NZ, Stanisavljevic DM. Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: A follow-up study of 3,029 patients with primary Raynaud’s phenomenon. Rheumatol Int 2012;32:3039-45.
  • Duffy CM, Laxer RM, Lee P, Ramsay C, Fritzler M, Silverman ED. Raynaud syndrome in childhood. J Pediatr 1989;114:73-8.
  • Cakır N, Pamuk ÖN, Derviş E, Imeryüz N, Uslu H, Benian Ö, et al. The prevalences of some rheumatic diseases in western Turkey: Havsa study. Rheumatol Int 2012;32:895-908.
  • Ingegnoli F, Boracchi P, Gualtierotti R, Lubatti C, Meani L, Zahalkova L, et al. Prognostic model based on nailfold capillaroscopy for identifying Raynaud’s phenomenon patients at high risk for the development of a scleroderma spectrum disorder: PRINCE (prognostic index for nailfold capillaroscopic examination). Arthritis Rheum 2008;58:2174-82.
  • Cutolo M, Herrick AL, Distler O, Becker MO, Beltran E, Carpentier P, et al. Nailfold videocapillaroscopic features and other clinical risk factors for digital ulcers in systemic sclerosis: A multicenter, prospective cohort study. Arthritis Rheum 2016;68:2527-39.
  • Plissonneau Duquene P, Pistorius MA, Pottier P, Aymard B, Planchon B. Cold climate could be an etiologic factor involved in Raynaud’s phenomenon physiopathology. Epidemiological investigation from 954 consultations in general practic. Int Angiol 2015;34:467-74.
  • Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud’s phenomenon in children: A retrospective review of 123 patients. Pediatrics 2003;111:715-21.
  • Maricq HR, Carpentier PH, Weinrich MC, Keil JE, Palesch Y, Biro C, et al. Geographic variation in the prevalence of Raynaud’s phenomenon: A 5 region comparison. J Rheumatol 1997;24:879- 89.
  • Overbury R, Murtaugh MA, Fischer A, Frech TM. Primary care assessment of capillaroscopy abnormalities in patients with Raynaud’s phenomenon. Clin Rheumatol 2015;34:2135-40.
  • Koenig M, Joyal F, Fritzler MJ, Roussin A, Abrahamowicz M, Boire G, et al. Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud’s phenomenon to systemic sclerosis: A twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum 2008;58:3902-12.
  • Pavlov-Dolijanovic S, Damjanov N, Ostojic P, Susic G, Stojanovic R, Gacic D, et al. The prognostic value of nailfold capillary changes for the development of connective tissue disease in children and adolescents with primary raynaud phenomenon: A follow-up study of 250 patients. Pediatr Dermatol 2006;23:437-42.
  • Pain CE, Constantin T, Toplak N, Moll M, Iking-Konert C, Piotto DP, et al. Raynaud’s syndrome in children: Systematic review and development of recommendations for assessment and monitoring. Clin Exp Rheumatol 2016;34:200-6.
  • Bakst R, Merola JF, Franks AG Jr, Sanchez M. Raynaud’s phenomenon: Pathogenesis and management. J Am Acad Dermatol 2008,59:633-53.
  • Thompson AE, Pope JE. Calcium channel blockers for primary Raynaud’s phenomenon: A meta-analysis. Rheumatology (Oxford) 2005;44:145-50.
  • Thompson AE, Shea B, Welch V, Fenlon D, Pope JE. Calcium- channel blockers for Raynaud’s phenomenon in systemic sclerosis. Arthritis Rheum 2001;44:1841-7. olacaktır.

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

Yıl 2018, Cilt: 12 Sayı: 1, 39 - 43, 01.04.2018

Öz

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

Kaynakça

  • Wigley FM, Flavahan NA. Raynaud’s Phenomenon. N Engl J Med 2016;375:556-65.
  • Fuhlbrigge R. In: Textbook of Pediatric Rheumotology. Petty RE (ed). Philadelphia: Elsevier, 2016;436-47.
  • Pavlov-Dolijanovic S, Damjanov NS, Stojanovic RM, Vujasinovic Stupar NZ, Stanisavljevic DM. Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: A follow-up study of 3,029 patients with primary Raynaud’s phenomenon. Rheumatol Int 2012;32:3039-45.
  • Duffy CM, Laxer RM, Lee P, Ramsay C, Fritzler M, Silverman ED. Raynaud syndrome in childhood. J Pediatr 1989;114:73-8.
  • Cakır N, Pamuk ÖN, Derviş E, Imeryüz N, Uslu H, Benian Ö, et al. The prevalences of some rheumatic diseases in western Turkey: Havsa study. Rheumatol Int 2012;32:895-908.
  • Ingegnoli F, Boracchi P, Gualtierotti R, Lubatti C, Meani L, Zahalkova L, et al. Prognostic model based on nailfold capillaroscopy for identifying Raynaud’s phenomenon patients at high risk for the development of a scleroderma spectrum disorder: PRINCE (prognostic index for nailfold capillaroscopic examination). Arthritis Rheum 2008;58:2174-82.
  • Cutolo M, Herrick AL, Distler O, Becker MO, Beltran E, Carpentier P, et al. Nailfold videocapillaroscopic features and other clinical risk factors for digital ulcers in systemic sclerosis: A multicenter, prospective cohort study. Arthritis Rheum 2016;68:2527-39.
  • Plissonneau Duquene P, Pistorius MA, Pottier P, Aymard B, Planchon B. Cold climate could be an etiologic factor involved in Raynaud’s phenomenon physiopathology. Epidemiological investigation from 954 consultations in general practic. Int Angiol 2015;34:467-74.
  • Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud’s phenomenon in children: A retrospective review of 123 patients. Pediatrics 2003;111:715-21.
  • Maricq HR, Carpentier PH, Weinrich MC, Keil JE, Palesch Y, Biro C, et al. Geographic variation in the prevalence of Raynaud’s phenomenon: A 5 region comparison. J Rheumatol 1997;24:879- 89.
  • Overbury R, Murtaugh MA, Fischer A, Frech TM. Primary care assessment of capillaroscopy abnormalities in patients with Raynaud’s phenomenon. Clin Rheumatol 2015;34:2135-40.
  • Koenig M, Joyal F, Fritzler MJ, Roussin A, Abrahamowicz M, Boire G, et al. Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud’s phenomenon to systemic sclerosis: A twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum 2008;58:3902-12.
  • Pavlov-Dolijanovic S, Damjanov N, Ostojic P, Susic G, Stojanovic R, Gacic D, et al. The prognostic value of nailfold capillary changes for the development of connective tissue disease in children and adolescents with primary raynaud phenomenon: A follow-up study of 250 patients. Pediatr Dermatol 2006;23:437-42.
  • Pain CE, Constantin T, Toplak N, Moll M, Iking-Konert C, Piotto DP, et al. Raynaud’s syndrome in children: Systematic review and development of recommendations for assessment and monitoring. Clin Exp Rheumatol 2016;34:200-6.
  • Bakst R, Merola JF, Franks AG Jr, Sanchez M. Raynaud’s phenomenon: Pathogenesis and management. J Am Acad Dermatol 2008,59:633-53.
  • Thompson AE, Pope JE. Calcium channel blockers for primary Raynaud’s phenomenon: A meta-analysis. Rheumatology (Oxford) 2005;44:145-50.
  • Thompson AE, Shea B, Welch V, Fenlon D, Pope JE. Calcium- channel blockers for Raynaud’s phenomenon in systemic sclerosis. Arthritis Rheum 2001;44:1841-7. olacaktır.
Toplam 17 adet kaynakça vardır.

Ayrıntılar

Diğer ID JA37UE25TG
Bölüm Research Article
Yazarlar

Ezgi Deniz Batu Bu kişi benim

Hafize Emine Sönmez Bu kişi benim

Yelda Bilginer Bu kişi benim

Yayımlanma Tarihi 1 Nisan 2018
Gönderilme Tarihi 1 Nisan 2018
Yayımlandığı Sayı Yıl 2018 Cilt: 12 Sayı: 1

Kaynak Göster

Vancouver Batu ED, Sönmez HE, Bilginer Y. Comparing the Characteristics in Children with Primary and Secondary Raynaud’s Disease. Türkiye Çocuk Hast Derg. 2018;12(1):39-43.

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