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FRI0598 Characterization of patients with an initial diagnosis of undifferentiated connective tissue disease. observations from a longstanding monocentric cohort
  1. C Sepúlveda1 2 3,
  2. AV Taulaigo2 3,
  3. F Carreiro2 3 4,
  4. MF Moraes-Fontes2 3
  1. 1Serviço de Medicina, Centro hospitalar Médio Tejo, Abrantes
  2. 2Unidade de Doenças Auto-imunes/Medicina 7.2, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central
  3. 3NEDAI - Núcleo de Estudos de Doenças Autoimunes da Sociedade Portuguesa de Medicina Interna, Lisbon
  4. 4Serviço de Medicina, Hospital do Divino Espírito Santo, Ponta Delgada, Azores, Portugal

Abstract

Background Evolution from Undifferentiated Connective Tissue Disease (UCTD) to a defined Autoimmune Disease (AID) remains unexplained and usually occurs in the first 3 to 5 years.

Objectives To determine the frequency and predictors of differentiation from UCTD to AID in a selected cohort of patients originally labelled as UCTD within the first 3 years of follow-up.

Methods Demographic and clinical features were retrieved: (i) retrospectively, from the AID's Unit current database in December 2016 (n=195), including Systemic Lupus Erythematosus (SLE), incomplete SLE, Sjögren's Syndrome (SS), Systemic Sclerosis (SSc), VEDOSS and Mixed Connective Tissue Disease (MCTD); and (ii) from a prospectively UCTD database created in January 2012 (n=48). Inclusion criteria for the latter pertains to ANA positive patients, not fulfilling any of the existing classification criteria for AID and without severe organ involvement [1]. Patients with cutaneous lesions suggestive of lupus, inflammatory myopathies, erosive arthritis, systemic sclerosis and certain auto-antibody profiles were excluded a priori from the definition of UCTD as these are thought to herald defined conditions, as previously defined [2]. Definition of stable UCTD includes patients with at least one clinical manifestation of AID, positive ANA result and disease duration of at least 3 years [3]. Comparisons between stable UCTD and progressing patients were made using Wilcoxon Rank Sum and Chi square tests, p values of <0.05 were considered statistically significant.

Results Each individual patient was analysed for differentiation into AID at yearly intervals. Overall, the main features of the prospective UCTD cohort were arthralgia (79%), rash (31%), arthritis (19%), sicca symptoms (19%), photosensitivity (17%) and Raynaud (13%). Prospective analysis in the UCTD cohort revealed differentiation in 4/48 patients (8.3%): into rheumatoid arthritis (n=2), psoriatic arthritis (n=1) and SLE (n=1). The main difference between stable UCTD and those that progressed to AID was the presence of arthritis (p=0.003). Median age of onset and symptom duration was similar between both groups. Retrospective analysis yielded very few patients presenting as UCTD (n=5/195): 2/106 SLE; 1/13 incomplete SLE; 1/43 SS; 1/19 SSc; 0/7 VEDOSS and 0/7 MCTD with no distinguishing features.

Conclusions Very few patients differentiated in the UCTD cohort after 3 years of follow-up and in our retrospectively studied cohort, in accordance to a previous study [4]. Apart from arthritis, there were no other predicting factors for differentiation to AID. UCTD at disease onset seems to be a rare event.

References

  1. Mosca M, et al. Clin Exp Rheumatol. 1999;17(5):615–20.

  2. Mosca M, et al. Lupus. 2008;17(4):278–80.

  3. Mosca M, at al. J Autoimmun. 2014;48–49:50–2.

  4. Danieli MG, et al. Clin Exp Rheumatol. 1999;17(5):585–91.

References

Disclosure of Interest None declared

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