Περίληψη:
Idiopathic pulmonary fibrosis and diffuse cutaneous systemic sclerosis
(dSSc) involve the lung by a fibrotic process. In recent years, there
has been increasing awareness that the natural history of these two
types of pulmonary fibrosis might be different. The purpose of this
study was to compare lung involvement in these two diseases in a
prospective fashion in order to address differences in their clinical
course. Forty-three consecutive patients, 18 with lone interstitial
pulmonary fibrosis (lone IPF) and 25 with dSSc-IPF were evaluated
clinically, radiologically and physiologically at the entry into the
study and the evolution of their disease was contrasted by survival
analysis. Patients with lone IPF compared with dSSc-IPF were
characterized by male predominance (p < 0.001), older age at disease
onset (p < 0.001), shorter disease duration (p < 0.001), more frequent
crackles on auscultation and clubbing (p < 0.001 and p < 0.0001,
respectively), more severe dyspnea (p < 0.0001) and more advanced
radiological involvement (p < 0.0001). Functional indices presented
comparable values and did not reach statistically significant
differences except for the values of single breath CO diffusing capacity
(p < 0.0001) and the PaO2 (p < 0.01) which was worse in patients with
lone IPF. Finally 12 of the 18 patients with lone IPF died in 2.66 +/-
1.18 years from the onset of respiratory symptoms, while none of the
dSSc-IPF patients had died 5.6 +/- 4.25 years from the first ever
appearance of respiratory involvement (p < 0.001). In conclusion,
although the two groups of patients were not at an absolutely comparable
stage of their disease, a worse prognosis for patients with lone IPF
seems to emerge from this study.
Συγγραφείς:
Papiris, SA
Vlachoyiannopoulos, PG
Maniati, MA
Karakostas,
KX
Constantopoulos, SH
Moutsopoulos, HH
