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SAT0242 Lung Involvement in Undifferentiated Connective Tissue Diseases (UCTD): Relationship with Clinical and Capillaroscopic Features
  1. R. Irace,
  2. A. Riccardi,
  3. S. Vettori,
  4. M. Iudici,
  5. G. Valentini
  1. Department of Internal and Experimental Medicine, rheumatology unit, naples, Italy

Abstract

Background Patients with UCTD (1) have been reported to present lung involvement most frequently characterized by nonspecific interstitial pneumonia (NSIP) at High Resolution Computerized Assial Tomography (HR-CAT), FVC and DLCO values lower than predicted (2).

Objectives To investigate UCTD patients for the presence of lung involvement and search for demographic, serological, capillaroscopic and organ involvement features associated with it.

Methods Eighty-six patients consecutively admitted to the Rheumatology Unit of the Second University of Naples and satisfying criteria for the classification of UCTD (1) were enrolled into the study. Patients presenting with any major CTD marker autoantibody other than anti-Ro (SSA) and/or scleroderma capillaroscopic specific pattern were excluded. Each patient underwent a careful history, a complete physical examination, evaluation of serum Antinuclear (ANA) and anti Extraible Nuclear Antigens antibody (anti-ENA), videocapillaroscopy, echo-Doppler-cardiography with evaluation of Left ventricular E/A ratio. Those who consented also underwent HR-CAT and esophagotonometry. Interstitial lung disease (ILD) was defined by HR-CAT or, if unavailable, by FVC<80% plus DLCO<80%.

Results The table lists the features detected in the patients subdivided according to the presence of lung involvement. DLCO <80% was found in 30/86 (34.8%) patients; FVC <80% in 3/86 (1.2%); HR-CAT was carried in 36/86 (41.8%) patients. It pointed a lung involvement NSIP pattern in 5/36 patients (13%).

An isolated reduction of DLCO resulted the be the most frequent finding and might depend on pulmonary vascular disease. Anti-SSA positivity and Capillaroscopic alterations were detected more frequently in patients with lung involvement (either isolated reduction of DLCO or ILD).

Conclusions We detected a prevalence of ILD in UCTD lower than previously reported (2) (80%). Nevertheless, we found an isolated reduction of DLCO in about 35% of the UCTD patients. Lung involvement resulted to be significantly associated with capillaroscopic alterations and anti-SSA positivity.

  1. Mosca M, Tani C, Vagnani S, Carli L, Bombardieri S.The diagnosis and classification of undifferentiated connective tissue diseases. J Autoimmun. 2014;48–49:50–2

  2. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, Jones KD, King TE Jr. Am J Respir Crit Care Med. Oct 1;176(7):691–7

Disclosure of Interest None declared

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