Session Information
Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II
Session Type: Abstract Submissions (ACR)
Background/Purpose: Raynaud’s phenomenon (RP) is classified as primary (PRP) or secondary (SRP) depending on its association with an underlying disease (1,2). PRP can evolve to SRP during followup (1,3). This is a prospective study to investigate timing and outcome of transition from PRP to SRP in a large cohort of patients, as well as to assess the progression of the nailfold scleroderma-patterns of microangiopathy in patients with isolated RP.
Methods: 2,853 patients with RP were investigated. Patients achieving a diagnosis of PRP at first visit (normal nailfold videocapillaroscopy (NVC), normal or negative laboratory findings including autoantibodies, absence of other clinical findings and exclusion of an underlying disease) were followed to monitor the appearance of specific morphological alterations at NVC, autoantibodies positivity, or clinical symptoms for a mean period of 52±40SD months (1,3). Nailfold microangiopathy was detected by NVC, and capillary abnormalities were classified according to different patterns of microangiopathy (4-5).
Results: At first visit, 797 (28%) patients out of 2,853 showed a NVC scleroderma-pattern, and got the diagnosis of SRP. Among the remaining 2,056 patients showing a normal capillaroscopy at baseline, 1,677 patients were lost during follow-up or not prospectively evaluable, whereas 379 patients were followed for a mean time of 52±40SD months. Among these last, 180 patients (47%) were positive for ANA, and 199 (53%) were ANA negative (PRP patients). During the follow-up, the NVC scleroderma-pattern was diagnosed in 62 patients out of 379 (16%). In particular, it was found in 48 (27%) of the ANA-positive patients (27 “early”, 4 “active”, 9 “late”, 8 “like” NVC scleroderma-pattern at the end of the follow-up): these patients achieved the diagnosis of systemic sclerosis (46 patients), UCTD (1 patient) and Sjogren syndrome (1 patient). NVC was found positive also in 14 (7%) of the ANA-negative patients (transition to SRP) (13 “early”, and 1 “like” NVC scleroderma-pattern at the end of the follow-up): these patients developed systemic sclerosis (9 patients) and UCTD (5 patients). The time of transition from normal/not specific capillary alterations to “early” scleroderma-pattern was 37±29 months. In particular, in ANA-positive patients NVC became positive within 43±36SD months, while in ANA-negative patients in 18±16 months. The 132 ANA-positive patients with a not defined NVC pattern developed respectively systemic sclerosis (7 patients), UCTD (104 patients), MCTD (8 patients), autoimmune thyroiditis (6 patients), Sjogren syndrome (4 patients), antiphospholipid syndrome (1 patients), LES (1 patients).
Conclusion: This large cohort study demonstrates that 7% of patients initially diagnosed as affected by PRP may transit to SRP in a mean time of 37±29 months. The transition from normal NVC pattern to scleroderma-pattern was more frequent in ANA-positive patients (27%).
References. 1. Cutolo M, et al. Arthritis Rheum 2007;56:2102-3. 2. LeRoy EC, et al. Clin Exp Rheumatol 1992;10:485–8. 3. Hirschl M, et al. Arthritis Rheum. 2006;54:1974-81. 4. Cutolo M, et al. Rheumatology 2004; 43:719-26.4. 5. Smith V, et al. Ann Rheum Dis 2010; 69:1092-6.
Disclosure:
A. Sulli,
None;
G. Ferrari,
None;
E. Bernero,
None;
C. Pizzorni,
None;
V. Smith,
Actelion Pharmaceuticals US,
5;
B. Ruaro,
None;
M. Cutolo,
Actelion Pharmaceuticals US,
2,
BMS,
2,
Mundipharm,
2,
Sanofi-Aventis Pharmaceutical,
2.
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