Abstract
BackgroundThe effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia (iNSIP), and factors affecting treatment outcome, are not fully understood. We aimed to investigate the long-term treatment response and factors affecting the treatment outcome in iNSIP patients from a multi-center study in Korea.MethodsThe Korean interstitial lung disease (ILD) Study Group surveyed ILD patients from 2003 to 2007. Patients were divided into two groups to compare the treatment response: response group (forced vital capacity (FVC) improves ≥10% after 1 year) and non-response group (FVC <10%). Factors affecting treatment response were evaluated by multivariate logistic regression analysis.ResultsA total of 261 patients with iNSIP were enrolled, and 95 patients were followed-up for more than 1 year. Corticosteroid treatment was performed in 86 patients. The treatment group showed a significant improvement in lung function after 1-year: FVC, 10.0%; forced expiratory volume (FEV1), 9.8%; diffusing capacity of the lung for carbon monoxide (DLco), 8.4% (p < 0.001). Sero-negative anti-nuclear antibody (ANA) was significantly related with lung function improvement. Sero-positivity ANA was significantly lower in the response group (p = 0.013), compared to that in the non-response group. A shorter duration of respiratory symptoms at diagnosis was significantly associated with a good response to treatment (p = 0.018).ConclusionTreatment with corticosteroids and/or immunosuppressants improved lung function in iNSIP patients, which was more pronounced in sero-negative ANA and shorter symptom duration patients. These findings suggest that early treatment should be considered in iNSIP patients, even in an early disease stage.
Highlights
The effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia, and factors affecting treatment outcome, are not fully understood
Overall lung function was slightly decreased compared to normal; forced vital capacity (FVC) (%) was 71.8 ± 18.4, forced expiratory volume (FEV1) (%) was 79.8 ± 20.9, and diffusing capacity of the lung for carbon monoxide (DLCO) (%) was 64.9 ± 21.3
Our study showed that corticosteroid and/or immunosuppressant therapy was effective in interstitial pneumonia (iNSIP), resulting in an improvement in lung function after 1 year
Summary
The effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia (iNSIP), and factors affecting treatment outcome, are not fully understood. We aimed to investigate the long-term treatment response and factors affecting the treatment outcome in iNSIP patients from a multi-center study in Korea. NSIP can present as idiopathic or is associated with secondary conditions, such as connective tissue disease (CTD), human immunodeficiency virus infection, IgG4-related disease, bone marrow transplant, or toxin/drug-related conditions [2,3,4]. NSIP with connective tissue disease has recently been reclassified as interstitial pneumonia with autoimmune disease [5]. The natural course of iNSIP is not yet known, previous studies showed that the prognosis of NSIP is favorable when compared with idiopathic pulmonary fibrosis (IPF) [6,7,8]. Previous studies have addressed the risk factors and medical conditions associated with mortality rate, relapse, and progression of the disease, but the degree of response and factors affecting treatment have not been well studied [10, 11]
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