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SAT0280 Raynaud's Phenomenon as An Early Symptom of Rheumatic Disease in Childhood
  1. M. Chęć,
  2. A. Szabat,
  3. V. Opoka-Winiarska
  1. Department of Pediatric Pulmonology and Rheumatology, Medical University of Lublin, Lublin, Poland

Abstract

Background Raynaud's phenomenon (RP) can be an initial manifestation of rheumatic disease [1]. In 2013 PReS Congressnewe consensus standards for clinical practice for children with RP was presented [2]. In 2014 a panel of experts established new diagnostic criteria for RP [3]. Nailfold capillaroscopy is an easy test with a well defined diagnostic role in the management of rheumatic diseases [4].

Objectives The aim of the study was the assessment of RP in children and adolescents with the evaluation of nailfold capillaroscopy and the relation of the clinical features to the capillaroscopic pattern.

Methods 77 children aged 7 to 18 years with symptoms of RP diagnosed according to the new diagnostic criteria [3] in Department of Pediatric Pulmonology and Rheumatology Medical University of Lublin were included in the study. RP was differentiated into primary (PRP) and secondary (SRP) RP in accordance with the new criteria [3]. Patients were reviewed for Raynaud's and other clinical symptoms, antinuclear antibodies (ANA) titer and other features. Capillaroscopic patterns were assessed by qualitative and semiquantitative methods and defined as normal, nonspecific changes or scleroderma pattern. Images of the study were archived.

Results 77 patients were included in the study (66 female and 11 male), average age of RP onset was 14,2 years. ANA and capillaroscopy were examined in all patients. Positive ANA (titer≥1/160) were found in 36 (46,75%) patients. Capillaroscopy results were normal in 33 (42,85%) cases, nonspecific changes in 25 (32,46%). The scleroderma patterns were recognized in 19 (24,7%) patients. According to diagnostic criteria for RP, 49 (63,6%) patients had secondary RP, among them systemic sclerosis was diagnosed in 1, juvenile dermatomyositis - 1, juvenile lupus erythematosus - 1, mixed connective tissue disease - 1, juvenile idiopathic arthritis in 3 patients and undifferentiated connective tissue disease in 16. Moreover, we recognized autoimmune thyroiditis in 7 patients, who had SRP. In patients with SRP: 19 (38,8%) had scleroderma pattern in capillaroscopy, 15 (30,6%) - nonspecific changes, 15 - 30,6% results were normal. In children with PRP: the results of capillaroscopy were normal in 18 cases (64,3%), nonspecific changes were observed in 10 (35,7%).

Conclusions In the present group of patients, more than half had secondary RP recognized.

Typically, RP had a beginning in teenage years with marked female predominance. Almost half of the patients with SRP was diagnosed with a rheumatic disease.

The new criteria and consensus standards for clinical practice in children with RP defined and the objective criteria for the assessment of the capillaroscopic exam significantly clarify the procedure of management of children or adolescents with RP.

A high percentage of secondary RP in our experience confirms the need for further follow up, even after the transition to adult age.

  1. Goundry B et. al. Diagnosis and management of Raynaud's phenomenon. BMJ 2012;344:e289.

  2. Constantin T et al. PReS-FINAL-2126: How to follow up children with Raynaud syndrome - recommendations based on the Hamburg consensus meeting 2012. Pediatric Rheumatology 2013, 11(Suppl 2):P138

  3. Maverakis E et al. International consensus criteria for the diagnosis of Raynaud's phenomenon. J Autoimmun. 2014;48–49:60–5.

  4. Sulli A et al. Scoring the nailfold microvascular changes during capillaroscopic analysis in systemic sclerosis patients. Ann Rheum Dis 2008;67:885–7.

Disclosure of Interest None declared

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