Highlights
- •Interstitial pneumonia with autoimmune features (IPAF) patients had more frequently a definite connective tissue disease (CTD) diagnosis during the disease course, especially antisynthetase syndrome and systemic sclerosis.
- •Identifying specific clinical and serological factors associated with CTD occurrence is essential at interstitial lung disease (ILD) diagnosis.
Abstract
Objectives
Among interstitial pneumonia with autoimmune features (IPAF) patients, identifying
those at risk to develop a connective tissue disease (CTD) during the disease course
is a key issue. The aim of this study was to evaluate the incidence of definite CTD
diagnosis in IPAF patients during follow-up.
Methods
We performed a multicentric cohort study of interstitial lung disease (ILD) from 2010
to 2017 in pneumology and immunology departments of tertiary care centers. Patients
with a known cause of ILD (including established CTD) at diagnosis were excluded.
Among patients with idiopathic ILD and at least three years of follow-up, two groups
(IPAF and non-IPAF) were retrospectively analyzed at time of diagnosis.
Results
A total of 249 patients with ILD were enrolled, including 70 IPAF and 179 non-IPAF
patients. After a mean follow-up time of 77 ± 44 months, 18/70 IPAF patients (26%)
had a CTD diagnosis – 9 antisynthetase syndrome, 8 systemic sclerosis and 1 overlap
myositis – compared with 4/179 non-IPAF patients (2%). IPAF patients were at higher
risk of CTD occurrence at 3 years of follow-up compared to non-IPAF patients (HR 10.1,
95% CI 3.1–33.1, p < 0. 01). IPAF patients progressing to CTD tended to be younger, more often female
and have more frequently puffy fingers, capillaroscopy abnormalities and antisynthetase
antibodies at diagnosis.
Conclusions
We found that a significant proportion of IPAF patients had associated CTD diagnosis
during follow-up. Prospective studies are needed to confirm baseline predictive factors
of CTD occurrence in IPAF patients.
Keywords
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Article info
Publication history
Published online: December 22, 2021
Accepted:
December 17,
2021
Received in revised form:
November 5,
2021
Received:
September 10,
2021
Identification
Copyright
© 2021 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.