Background/Purpose:

In recent years there has been a growing interest in establishing the concept of Undifferentiated CTD (UCTD) and defining its clinical evolution. Remarkable are the Marta Mosca et al studies, who have proposed preliminary definition criteria and reported progression to a Defined CTD (DCTD) in 30% of patients after 5-year follow-up. Some studies have pointed out certain clinical, serological and capillaroscopic features as potential predictive factors, with contrasting results. In our study we aimed to describe the evolution of a cohort of UCTD patients with at least 5-year follow-up and to establish clinical, serological and capillaroscopic features associated with an increased risk of progression to a DCTD.

Methods:

Patients with first nailfold capillaroscopy (NFC) performed between 1999-2008 who met at least 2 of the following baseline criteria: 1) Raynaud’s phenomenon (RP), 2) ANA ≥1/80 and/or positive ENA, 3) pathological NFC pattern, were included. Nailfold capillaroscopies were performed by the same observer with a Leica 10×23 10447123 handheld lighted microscope. The association between outcome and the presence of several baseline features (RP, arthralgias, sicca syndrome, skin and esophageal abnormalities, low complement, Ig and hematologic disorders, ANA titre and pattern, ENA, anti-dsDNA and aPL antibodies, RF, and NFC pattern) was analyzed.

Results:

Among 758 patients with first NFC performed in the cited period, 223 fulfilled inclusion criteria. Ninety seven patients who met classification criteria for a DCTD at disease onset (including Prescleroderma (PSSc)) and 28 with less than 5-year follow-up were excluded. Among 98 analyzed, 97 (99%) were women with a baseline mean age of 42 years (s: ± 15.8). After a mean of 10.6 years follow-up (s: ± 3.1), 61 (62%) remained as UCTD, 23 (24%) evolved into remission and 14 (14%) into DCTD: 8 SLE, 4 PSSc, 1 MCTD, and 1 overlap syndrome. NFC pattern progression was observed in 31% of the patients with a second NFC performed 5 years after disease onset (78/98). Significant differences between clinical outcome and presence of cytopenias (p:0.030), ENA (p:0.008) and aPL antibodies (p:0.032), and a pathological NFC pattern (p:0.026) were observed. Homogeneous (29%) and centromere (36%) patterns were more frequent in DCTD group, while in the remission group was the coarse speckled one (41%) (p:0.117). Positive correlation between ANA titre and outcome was demonstrated (rho:0.21, p:0.025). ANA titre ≥1/640 (OR:17 [1.75-165], p:0.015), a pathological NFC pattern (OR:6.48 [1.48-28.3], p:0.013) and NFC pattern progression after 5-year follow-up (OR:7 [1.35-36.3], p:0.021) were associated with an increased risk of evolving into DCTD.

Conclusion:

1. The rate of progression to DCTD in our cohort was similar to that reported in previous studies.

2. Careful monitoring should be recommended in patients with baseline ANA titre ≥ 1/640 and/or pathological NFC pattern, especially those who present capillaroscopic progression during the follow up.

3. Results concerning potential predictive role of baseline IIF pattern, cytopenias, ENA and aPL antibodies, are coherent with previous studies.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/progression-predictive-factors-in-patients-with-undifferentiated-connective-tissue-disease-a-cohort-study/