SCLERODERMA SYSTEMATICA WITH INTERSTITIAL LUNG LESION: COMPARATIVE CLINICAL CHARACTERISTICSWITH PATIENTS WITHOUT LUNG LESION

Objective. To compare disease history data and clinical and laboratory parameters in patients with scleroderma systematica (SDS) with high-resolution
computed tomography (HRCT)-verified interstitial lung lesion (ILL) versus those without lung involvement.
Subjects and methods. An examination was made in 138 patients with SDS who had been consecutively admitted in 2006-2008, female/male ratio, 124 :
14; limited : diffuse : mixed forms, 78 : 40 : 20; mean age, 47±13 years; median disease duration, 6 (2.5 11) years. The history data (occupational hazards,
smoking, respiratory diseases) and clinical manifestations of SDS and laboratory data were studied. The diagnosis of ILL was established on the basis of
chest HRCT.
Results. According to HRCT data, the signs of varying ILL were found in 82% of the patients with SDS. The duration of SDS was similar in the patients with
and without lung involvement; but the latter were younger at the time of disease onset. There were no significant differences between the groups compared in
history data, clinical forms of SDS, the frequency of involvement of visceral organs and systems. Crepitation was heard only in the patients with ILL. The
frequency of respiratory manifestations increased with a larger number of the involved lung segments. The prevalence of ILL was found to be positively correlated
with age at the onset of SDS (r=0.29; p<0.0005), SDS activity index (r=0.24; p<0.005), lactate dehydrogenase (r=0.39; p<0.0004), erythrocyte sedimentation
rate (r = 0.2; p<0.02) and C-reactive protein (r=0.23; p<0.01). Anticentromere antibodies (ACAb) were significantly more frequently detectable
in the patients without lung involvement.
Conclusion. The clinical and X-ray pattern of ILL does not depend on history data, gender, and the subtype of SDS. The frequency of respiratory manifestations
increases with a larger number of the involved lung segments. Crepitation is an important physical sign of ILL, which is indicative of the extensive
nature of changes. The large extent of lung parenchymal involvement was associated with higher inflammatory activity. In patients without lung involvement,
SDS occurred at a younger age and was more frequently associated with ACAb.

scleroderma systematica, interstitial lung lesion, high-resolution computed tomography

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