Evaluation of Primary and Secondary Raynaud’s Phenomenon in Childhood

Year 2023, Volume: 17 Issue: 1, 7 - 12, 30.01.2023

Müge Sezer Elif Çelikel Fatma Aydın Zahide Ekici Tekin Tuba Kurt Nilüfer Tekgöz Cüneyt Karagöl Serkan Coşkun Melike Kaplan Banu Acar

https://doi.org/10.12956/tchd.1054799

Abstract

Objective: To evaluate the clinical, laboratory and capillaroscopic findings of pediatric patients with Raynaud’s phenomenon.

Material and Methods: Ninety-five pediatric patients who were diagnosed with Raynaud’s phenomenon between January 2014 and January 2021, were retrospectively examined. The demographic data, laboratory parameters and capillaroscopic findings of the patients were recorded. The capillaroscopic findings of the patients were classified as normal, nonspecific abnormalities and scleroderma pattern.

Results: Primary Raynaud’s phenomenon was present in 84 (88.5%) patients, and secondary Raynaud’s phenomenon was present in 11 (11.5%). Arthralgia, arthritis, rash and recurrent fever were significantly more common in secondary Raynaud’s phenomenon (p=0.001, p=<0.001, p=0.01, p=0.035, respectively). Antinuclear antibody positivity >1/320 was significantly higher in the patients with secondary Raynaud’s phenomenon (p=0.01). Of the 40 patients who had capillaroscopy performed, 2 had a scleroderma pattern, 19 had nonspecific changes, and 19 had normal nailfold capillaroscopic findings. Capillary irregularity, tortuous capillaries and increased branching were significantly higher in the secondary Raynaud’s phenomenon cases (p=0.015, p=0.015, p=0.003, respectively).

Conclusion: Having antinuclear antibody titer >1/320 and detection of capillary irregularity, tortuous capillaries and increased branching may be useful in distinguishing primary and secondary Raynaud’s phenomenon. 

Keywords

Antinuclear antibody, capillaroscopy, pediatric, Raynaud’s Phenomenon, rheumatology

Çocukluk Çağı Birincil ve İkincil Raynaud Fenomeni Olgularının Değerlendirilmesi

Abstract

Amaç: Raynaud fenomeni ile takip edilen pediatrik hastaların klinik, laboratuvar ve kapilleroskopik bulgularını değerlendirmek.

Gereç ve Yöntemler: Ocak 2014 ile Ocak 2021 tarihleri arasında Raynaud fenomeni tanısı ile takip edilen 95 çocuk hasta geriye dönük olarak incelendi. Hastaların demografik verileri, laboratuvar parametreleri ve kapilleroskopik bulguları kaydedildi. Hastaların kapilleroskopik bulguları normal, nonspesifik anormallikler ve skleroderma paterni olarak sınıflandırıldı.

Bulgular: Birincil Raynaud fenomeni 84 hastada (%88.5), ikincil Raynaud fenomeni 11 hastada (%11.5) mevcuttu. Artralji, artrit, döküntü ve tekrarlayan ateş, sekonder Raynaud fenomeninde anlamlı olarak daha sıktı (sırasıyla p=0.001, p=<0.001, p=0.01, p=0.035). Sekonder Raynaud fenomeni olan hastalarda 1/320 titre ve üzerinde antinükleer antikor pozitifliği anlamlı olarak daha sıktı (p=0.01). Kapilleroskopi yapılan 40 hastanın 2’sinde skleroderma paterni, 19’unda nonspesifik değişiklik ve 19’unda normal kapilleroskopi bulguları vardı. İkincil Raynaud fenomeni olgularında kapiller düzensizlik, tortuyoz kapiller ve dallanma artışı anlamlı olarak daha sık saptandı (sırasıyla p=0.015, p=0.015, p=0.003).

Sonuç: Antinükleer antikor titresinin >1/320 olması ve kapiller düzensizlik, tortuyoz kapiller ve dallanma artışının saptanması, birincil ve ikincil Raynaud fenomenini ayırt etmede faydalı olabilir.

Keywords

Antinükleer antikor, kapilleroskopi, pediatri, Raynaud Fenomeni, Romatoloji

References

• Rigante D, Fastiggi M, Ricci F, D’Errico F, Bracci B, Guerriero C. Handy Hints About Raynaud’s Phenomenon in Children: A Critical Review. Pediatr Dermatol 2017;34:235-9.
• Goldman RD. Raynaud phenomenon in children. Can Fam Physician 2019;65:264-5.
• Turan E, Kilic SS. Retrospective view of primary Raynaud’s phenomenon in childhood. Reumatol Clín (English Edition) 2019;15:e92-e5.
• Planchon B, Pistorius M-A, Beurrier P, De Faucal P. Primary Raynaud’s phenomenon: age of onset and pathogenesis in a prospective study of 424 patients. Angiology 1994;45:677-86.
• Haque A, Hughes M. Raynaud’s phenomenon. Clin Med 2020;20:580.
• Herrick AL, Wigley FM. Raynaud’s phenomenon. Best Pract Res Clin Rheumatol 2020;34:101474.
• Esteireiro AS, Bicho A. Raynaud’s phenomenon in paediatric age. BMJ Case Rep 2020;13.
• Vicente EO, Redondo MG. Raynaud’s phenomenon in children. Reumatol Clín (English Edition) 2016;12:342-4.
• Pauling JD, Hughes M, Pope JE. Raynaud’s phenomenon—an update on diagnosis, classification and management. Clin Rheumatol 2019;38:3317-30.
• Adrovic A, Şahin S, Barut K, Kasapçopur O. Juvenile scleroderma: a referral center experience. Arch Rheumatol 2018;33:344.
• Nigrovic PA, Fuhlbrigge RC, Sundel RP. Raynaud’s phenomenon in children: a retrospective review of 123 patients. Pediatrics 2003;111:715-21.
• Pavlov‐Dolijanović S, Damjanov N, Ostojić P, Sušić G, Stojanović R, Gacić 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.
• Ingegnoli F, Herrick AL. Nailfold capillaroscopy in pediatrics. Arthritis Care Res 2013;65:1393-400.
• Petty RE, Laxer RM, Lindsley CB, Wedderburn L, Fuhlbriggezzz RC, Mellins ED. Textbook of Pediatric Rheumatology E-Book:8th ed. USA. Elsevier Health Sciences; 2020.
• Fleck DE, Hoeltzel MF. Hand and foot color change: diagnosis and management. Pediatr Rev 2017;38:511-9.
• Batu ED, Sönmez HE, Bilginer Y. Çocuklarda Raynaud Fenomeni ve Birincil ve İkincil Raynaud Hastalarının Karşılaştırılması. Turkish J Pediatr Dis 2018;12:39-43.
• Maverakis E, Patel F, Kronenberg DG, Chung L, Fiorentino D, Allanore Y, et al. International consensus criteria for the diagnosis of Raynaud’s phenomenon. J Autoimmun 2014;48:60-5.
• Schonenberg-Meinema D, Bergkamp SC, Nassar-Sheikh Rashid A, van der Aa LB, de Bree GJ, Ten Cate R, et al. Nailfold capillary abnormalities in childhood-onset systemic lupus erythematosus: a cross-sectional study compared with healthy controls. Lupus 2021;30:818-27.
• Smith V, Herrick AL, Ingegnoli F, Damjanov N, De Angelis R, Denton CP, et al. Standardisation of nailfold capillaroscopy for the assessment of patients with Raynaud’s phenomenon and systemic sclerosis. Autoimmun Rev 2020;19:102458.
• Pain CE, Constantine 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.
• Shapira Y, PoratKatz B-S, Gilburd B, Barzilai O, Ram M, Blank M, et al. Geographical differences in autoantibodies and anti-infectious agents antibodies among healthy adults. Clin Rev Allergy 2012;42:154-63.
• Falcini F, Rigante D, Candelli M, Martini G, Corona F, Petaccia A, et al. Anti-nuclear antibodies as predictor of outcome in a multi-center cohort of Italian children and adolescents with Raynaud’s phenomenon. Clin Rheumatol 2015;34:167-9.