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

Abstract

Objective. To identify in patients with Raynaud's phenomenon (RP) independent markers that predict progression to definite systemic sclerosis (SSc) and to determine in patients with progression to SSc the type and sequence of microvascular damage and its relationship to SSc-specific autoantibodies.Methods. Consecutive patients referred for evaluation of RP who had no definite connective tissue disease were evaluated for microvascular damage by nailfold capillary microscopy (NCM) and for anticentromere (anti-CENP-B), anti-Th/To, anti-topoisomerase I, and anti-RNA polymerase III (anti-RNAP III) autoantibodies by specific assays. Patients were studied prospectively.Results. Of the 586 patients who were followed up for 3,197 person-years, 74 (12.6%) developed definite SSc. A characteristic sequence of microvascular damage was identified, starting with enlarged capillaries, followed by capillary loss, and then by capillary telangiectases. Definite SSc was diagnosed in close temporal relationship to capillary loss. Enlarged capillaries, capillary loss, and SSc-specific autoantibodies independently predicted definite SSc. Anti-CENP-B and antiTh/To antibodies predicted enlarged capillaries; these autoantibodies and anti-RNAP III predicted capillary loss. Each autoantibody was associated with a distinct time course of microvascular damage. At followup, 79.5% of patients with 1 of these autoantibodies and abnormal findings on NCM at baseline had developed definite SSc. Patients with both baseline predictors were 60 times more likely to develop definite SSc. The data validated the proposed criteria for early SSc.Conclusion. In RP evolving to definite SSc, microvascular damage is dynamic and sequential, while SScspecific autoantibodies are associated with the course and type of capillary abnormalities. Abnormal findings on NCM at baseline together with an SSc-specific autoantibody indicate a very high probability of devel-

Early diagnosis and management of systemic sclerosis

“…25,26 In a study that included 586 patients who were followed up for 3197 person-years, the presence of SSc-specific autoantibodies and SSc-type capillaroscopic abnormalities were independent predictors of the development of SSc (hazard ratios of 4.7 and 4.5 respectively). 27 Patients with both abnormalities were 60 times more likely to develop SSc. Figure 3 presents an overview of the British Society of Rheumatology/British Health Professionals in Rheumatology (BSR/BHPR) guideline for the treatment of SSc, 9 which is a useful reference tool for clinicians in both primary and secondary care alike.…”

Seroconversion from Anti-Th/To to Anticentromere Antibodies in a Patient with Systemic Sclerosis

“…Antinuclear antibodies (ANA) are involved in the diagnosis of systemic sclerosis (SSc), being present in 90% of the patients 1,2,3 . In the majority of ANA-positive patients with SSc, a single SSc-specific autoantibody can be detected, i.e.…”

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.…”