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

Abstract

To assess the prognostic value of capillaroscopy findings for the development of connective tissue disease in children and adolescents with Raynaud phenomenon, we followed up a group of 250 (mean age 15 years) for 1 to 6 years after the first capillaroscopy was performed. Every 6 months they were screened for signs and symptoms of connective tissue disease. Analysis was performed on capillary changes registered 6 months before the development of connective tissue disease. Capillary changes were classified into three types: normal, nonspecific, and sclerodermatous. At the end of the follow-up period, 191 (76%) subjects had primary Raynaud phenomenon, 27 (10.8%) were diagnosed as having undifferentiated connective tissue disease, and 32 (12.8%) fulfilled the criteria for a diagnosis of a specific connective tissue disease. Systemic lupus erythematosus was found in nine (3.6%) patients, rheumatoid arthritis in 10 (4%) patients (six of them with juvenile onset rheumatoid arthritis), and scleroderma spectrum disorders in 13 (5.2%). The mean time for the evolution of Raynaud phenomenon into undifferentiated connective tissue disease or a form of the disease was 2 years. Most of the subjects with primary Raynaud phenomenon (173/191, 91%), undifferentiated connective tissue disease (22/27, 81%), juvenile onset rheumatoid arthritis/rheumatoid arthritis (7/10, 70%), and systemic lupus erythematosus (6/9, 67%) had normal capillary findings. Nonspecific capillary changes occurred in 3 of 10 (30%) patients with rheumatoid arthritis, 2 of 9 (22%) with systemic lupus erythematosus, 4 of 27 (15%) with undifferentiated connective tissue disease, and 18 of 191 (9%) with primary Raynaud phenomenon. Of all the subjects, only 10 (4%) showed sclerodermatous disease type capillary changes 6 months before the expression of a particular disease: eight (62%) of these developed scleroderma spectrum disorders, one expressed systemic lupus erythematosus, and one had undifferentiated connective tissue disease. We concluded that there were no specific capillary changes predictive for future development of systemic lupus erythematosus, juvenile onset rheumatoid arthritis/rheumatoid arthritis, and undifferentiated connective tissue disease in children and adolescents with Raynaud phenomenon. Most of our study subjects with Raynaud phenomenon who developed these diseases had normal capillary findings or nonspecific changes. Children and adolescents who developed scleroderma spectrum disorders showed a sclerodermatous type of capillary changes 6 months before the expression of the disease, indicating that this type of capillary changes in children and adolescents with Raynaud phenomenon highly correlated with further development of scleroderma spectrum disorders.

Capilaroscopia periungueal em crianças e adolescentes com doenças reumáticas

“…Those changes are more often found in patients with polyarticular JIA and positive for RF and ANA. 19,20 In our study, those changes were not found, maybe because of the small amount of patients with those antibodies. However, patients with RP show more changes on NFC, indicating that they probably have vasculopathy.…”

Nailfold capillaroscopy microscopy - an interdisciplinary appraisal

“…The very low risk of developing SSc and other CTD in people presenting with normal capillary pattern has been confi rmed in a recent larger trial [36]. While up to 60 % of healthy persons might exhibit single or combined abnormalities of a scleroderma pattern, megacapillaries and avascular fi elds are not expected in the 'normal population', nor is it expected in primary RP [18,19].…”

“…The prediction of a typical scleroderma pattern for the identifi cation of actual connective tissue disease or its subsequent development is high, whereas the negative prediction for a normal microscopic pattern is excellent [36,42,49,64].…”

“…At the end of the follow-up period, 23.6 % had progressed to CTD, mainly undiff erentiated CTD. The mean time in this study for developing disease was 2.4 years [36]. In the other cohort of 123 children < 19 years with RP, the transition rate to CTD was 8 %, retrospectively [37].…”

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

“…Kız/erkek oranı 1.7'di. Hastaların medyan (minimum-maksimum) semptom başlangıç yaşı 12 (5-16); tanı yaşı 13 (5.5-16); çalışmaya dahil edildikleri andaki yaşları 15 (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) Raynaud fenomeninin tedavisi için yapılmış klinik çalışma ve kılavuzlar halen yetersiz olsa da genel görüş soğuk, stres gibi tetikleyici faktörlerden kaçınmanın faydalı olduğu yönündedir (15). Genel önlemlerin başarısız olduğu olgularda ilk basamak tedavi kalsiyum kanal blokörleridir.…”