Dataset Information

The Risk Factors of Exacerbation in Interstitial Pneumonia With Autoimmune Features: A Single-Center Observational Cohort Study.

ABSTRACT:

Objectives

To investigate the long-term outcomes, including risk factors, for exacerbation between monotherapy and combination therapy in patients with interstitial pneumonia with autoimmune features (IPAF).

Methods

We assessed 672 patients between April 2009 and March 2019 who were evaluated using high-resolution computed tomography (HRCT) of the chest. We applied the IPAF criteria. Fifty-two patients who visited our department for at least 6 months were diagnosed with IPAF. Clinical, laboratory, and imaging data were collected from medical records and statistically analyzed.

Results

Among the 52 cases of IPAF, we compared the characteristics at diagnosis between treated (n = 28) and untreated patients (n = 24). The exacerbation rates were 42.9% (n = 12) and 8.3% (n = 2) (P = 0.0051), respectively. Among the treated patients, smoking history, high titer of KL-6, and the duration from diagnosis to the start of treatment were significant risk factors for exacerbation (P = 0.0062, 0.011, and 0.019, respectively). The number of risk factors was significantly and positively associated with exacerbation rate (P = 0.0053). Among the treated patients, glucocorticoid (GC) monotherapy was used in 13 cases, and GC and immunosuppressant (IS) combination therapy was used in 14 patients. There was no significant difference in the treatment methods between patients with and without risk factors (P = 0.47). When comparing the long-term outcomes between the monotherapy and combination therapy groups, the 3-year non-exacerbation rates were 72.9 and 45.9% (P = 0.020), respectively.

Conclusions

IPAF patients with risk factors had a high exacerbation rate, regardless of the type of treatment. New interventions aimed at preventing exacerbations in these patients are required.

SUBMITTER: Murata O

PROVIDER: S-EPMC8572251 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:To identify the prognostic factors for survival in patients with interstitial pneumonia with autoimmune features (IPAF) who meet the serological domain of the IPAF criteria.We retrospectively analysed 99 IPAF patients who met the serological domain and were hospitalised at the Respiratory Medicine Unit of Kurashiki Central Hospital from 1999 to 2015. The high-resolution computed tomography findings were usual interstitial pneumonia (UIP; n = 1), non-specific interstitial pneumonia (NSIP; n = 63), NSIP with organizing pneumonia (OP) overlap (n = 15), and OP (n = 20). One patient who had radiological UIP pattern, and met the serological and clinical domains was excluded. The clinical characteristics, radiological findings, administered therapy, and prognosis of the remaining 98 IPAF patients who met the serological and morphological domains were analysed.The median age of the 98 IPAF patients was 68 years, and 41 (41.8%) of them were men. Twelve (12.2%) of the 98 IPAF patients developed other characteristics and were diagnosed with connective tissue disease (CTD) later during the median follow-up of 4.5 years. Univariate Cox analysis revealed systemic sclerosis (SSc)-specific and SSc-associated antibodies (ANA nucleolar pattern, ANA centromere pattern, anti-ribonucleoprotein and anti-Scl-70) positive IPAF, radiological NSIP pattern, bronchoalveolar lavage fluid lymphocytes >15%, and age as significant prognostic factors for survival. Multivariate Cox analysis revealed radiological NSIP pattern (hazard ratio [HR], 4.48; 95% confidence interval [CI], 1.28-15.77, p = 0.02) and age (HR, 1.07; 95% CI, 1.02-1.11, p = 0.01) were significantly associated with worse survival.We confirmed that radiological NSIP pattern and age are poor prognostic factors for the survival of IPAF patients. This study suggested that the autoantibodies that are highly specific for certain connective tissue diseases might be less important for the prognosis of IPAF compared with the radiological-pathological patterns. The relatively high proportion of IPAF patients who developed CTD later suggests the importance of careful observation for evolution to CTD in IPAF.

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