Is it necessary to treat all patients with idiopathic pulmonary fibrosis?
OBJECTIVE
To investigate the clinical course of untreated patients with idiopathic pulmonary fibrosis (usual interstitial pneumonia) (IPF/UIP).


METHODS
Forty-three patients with IPF/UIP, divided into two groups. Group I consisted of 29 patients treated at diagnosis, while Group II comprised 14 patients who did not receive treatment. The indication of treatment was established whenever patients referred to a significant progression of the degree of dyspnea during the year prior to diagnosis.


RESULTS
At diagnosis, patients from Group I had lower FVC (mean +/- SEM, 56+/-3% vs 73+/-3%) (p = 0.0004) and a greater extent of ground glass pattern in high resolution CT scan (18+/-4% vs 4+/-1%) (p = 0.004) than those from Group II. In group I, a follow-up study was carried out on 26 patients for 24+/-4 months. Thirteen of these 26 patients (50%) died 11+/-4 months after the initial assessment. Serial pulmonary functional tests were performed on 19 patients. Thirteen patients from Group II were followed up for 23+/-3 months. Seven of these 13 patients were treated 12+/-3 months after the diagnosis because of progression of the disease. The remaining 6 patients remained untreated and with the disease stable at the end of the follow-up, representing 15% (6 out of 39) of the whole study group. No patients from this group died during the follow-up. At the end of the follow-up, there were no differences in lung function changes between treated patients (19 from Group I and 7 from Group II), and the 6 untreated patients.


CONCLUSIONS
Some patients with IPF/UIP remain stable for extended periods of time without treatment.
Xaubet
A
A
Servei de Pneumologia i Al.lèrgia Respiratória, Institut Clinic de Pneumologia i Cirurgia Torácica, Hospital Clinic, Universitat de Barcelona, Spain. axaubet@clinic.ub.es
Agustí
C
C
Luburich
P
P
Roca
J
J
Ayuso
M C
MC
Marrades
R M
RM
Rodriguez-Roisin
R
R
D000368:Aged; D000369:Aged, 80 and over; D018893:Bronchoalveolar Lavage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy*; D012129:Respiratory Function Tests