Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features.
OBJECTIVE
To assess internal organ involvement in early SSc at presentation.


METHODS
One hundred and fifteen patients admitted to a tertiary centre because of RP, who did not present any routinely detectable scleroderma-related internal organ involvement, were investigated for ANA and videocapillaroscopy, and underwent history and physical examination to detect symptoms/signs suggestive of SSc. Patients were then subdivided into three groups: (i) early SSc, constituted by patients without clinical manifestations other than RP, but with scleroderma marker autoantibodies and/or typical capillaroscopic abnormalities; (ii) probable SSc, constituted by patients with the same autoantibody and/or capillaroscopic status as early SSc patients, but with any of the following manifestations: digital ulcers/scars, puffy fingers, arthritis, telangiectasia, dysphagia/heartburn, shortness of breath; (iii) UCTD, constituted by patients with a specific (i.e. disease antibody marker) ANA and capillaroscopic findings plus any disease manifestation. All patients were investigated by lung functional study and B-mode echo-Doppler-cardiography. Patients who consented underwent oesophageal manometry.


RESULTS
An inverted mitral E : A ratio (i.e. early scleroderma cardiac involvement) and/or a diffusing lung capacity for CO <80% of the predictive value (i.e. early lung involvement) and/or basal low oesophageal sphincter pressure <15 mmHg (i.e. early oesophageal involvement) were detected in 37/51 probable SSc patients (72%), 8/19 early SSc patients (42%) and 12/45 UCTD patients (27%).


CONCLUSIONS
A scleroderma-related internal organ involvement was detected in patients from each group and, more importantly, was pre-clinical in a number of cases.
Valentini
Gabriele
G
Unità di Reumatologia della Seconda Università di Napoli, Policlinico, Via Pansini, 5-80131 Napoli, Italy. gabriele.valentini@unina2.it
Cuomo
Giovanna
G
Abignano
Giuseppina
G
Petrillo
Ambrogio
A
Vettori
Serena
S
Capasso
Alessia
A
Cozzolino
Domenico
D
Del Genio
Gianmattia
G
Santoriello
Carlo
C
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D005767:Gastrointestinal Diseases / Q000503:physiopathology; D006331:Heart Diseases / Q000503:physiopathology; D006801:Humans; D007674:Kidney Diseases / Q000503:physiopathology; D008171:Lung Diseases / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011928:Raynaud Disease / Q000150:complications* / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000503:physiopathology*; D013997:Time Factors; D055815:Young Adult