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Presentation Number: 1881
Purpose: Raynaud’s Phenomenon (RP) may affect 5-10% of the healthy population; however, it may also be the first symptom of a connective tissue disease (CTD). The presence of RP associated with positive antinuclear antibody (ANA) without other signs of a CTD has generally been accepted to represent an “incomplete CTD”, “early-stage scleroderma spectrum disorder”, or “a syndrome variant of systemic sclerosis”. Previous studies report that these patients evolve into a defined CTD within 2 to 7 years. Here, we reviewed the long term clinical course of 20 patients with RP and positive ANA who did not have a CTD at initial evaluation to determine their progression into a CTD or systemic sclerosis (SSc).
Methods: Twenty patients with positive ANA and RP without definite diagnosis of CTD at initial evaluation were identified. Demographic data, length of follow up, onset and duration of RP, current complaints, serological studies, and physical findings were recorded. In addition, results of pulmonary function tests, high resolution chest computed tomography and transthoracic echocardiograms were analyzed for findings of CTD or SSc.
Results: There were 17 female and 3 male patients (range 18-57 yrs). Patients were followed a mean of 7.7 (range 2-18) years from time of initial evaluation (15 patients ≥ 4 years and 5 patients <4 years). The median titer ANA was 1:320. Patterns included nucleolar (4), speckled (3), multiple patterns (3), and centromere (2). Scl-70 antibody was positive in 4 patients. At the end of the study, among the 15 patients followed for ≥ 4 years, 3 patients met criteria for classification of SSc based on the presence of puffy hands, sclerodactly, telangiectasias, reflux, and abnormal capillary microscopy, and one of them had interstitial lung disease. One additional patient with positive SSA, SSB and RNP antibodies had evidence of interstitial lung disease in addition to capillary microscopy findings at 16 year follow up and was classified as a CTD. The remaining 11 patients were followed for at least 4 years and did not develop any evidence of progression to SSc or to a defined CTD at the end of the long term follow up.
Conclusions: Limited studies exist on the evolution of patients with positive ANA and RP to CTD. In our study from a cohort of 15 patients with RP and positive ANA in the absence of CTD at initial evaluation, 4 patients followed ≥4 years progressed to a CTD, whereas 11 patients remain without evidence of a defined CTD. The results with a long term follow up of one of the largest populations with RP and positive ANA in the absence of CTD at initial evaluation indicate that there is a subset of patients who remain with only positive ANA and RP and do not develop a defined CTD.
Methods: Twenty patients with positive ANA and RP without definite diagnosis of CTD at initial evaluation were identified. Demographic data, length of follow up, onset and duration of RP, current complaints, serological studies, and physical findings were recorded. In addition, results of pulmonary function tests, high resolution chest computed tomography and transthoracic echocardiograms were analyzed for findings of CTD or SSc.
Results: There were 17 female and 3 male patients (range 18-57 yrs). Patients were followed a mean of 7.7 (range 2-18) years from time of initial evaluation (15 patients ≥ 4 years and 5 patients <4 years). The median titer ANA was 1:320. Patterns included nucleolar (4), speckled (3), multiple patterns (3), and centromere (2). Scl-70 antibody was positive in 4 patients. At the end of the study, among the 15 patients followed for ≥ 4 years, 3 patients met criteria for classification of SSc based on the presence of puffy hands, sclerodactly, telangiectasias, reflux, and abnormal capillary microscopy, and one of them had interstitial lung disease. One additional patient with positive SSA, SSB and RNP antibodies had evidence of interstitial lung disease in addition to capillary microscopy findings at 16 year follow up and was classified as a CTD. The remaining 11 patients were followed for at least 4 years and did not develop any evidence of progression to SSc or to a defined CTD at the end of the long term follow up.
Conclusions: Limited studies exist on the evolution of patients with positive ANA and RP to CTD. In our study from a cohort of 15 patients with RP and positive ANA in the absence of CTD at initial evaluation, 4 patients followed ≥4 years progressed to a CTD, whereas 11 patients remain without evidence of a defined CTD. The results with a long term follow up of one of the largest populations with RP and positive ANA in the absence of CTD at initial evaluation indicate that there is a subset of patients who remain with only positive ANA and RP and do not develop a defined CTD.
M.S. O'Brien, None.