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Treatment response and long term follow-up results of nonspecific interstitial pneumonia.

Lee JY, Jin SM, Lee BJ, Chung DH, Jang BG, Park HS, Lee SM, Yim JJ, Yang SC, Yoo CG, Han SK, Shim YS, Kim YW - J. Korean Med. Sci. (2012)

Bottom Line: The initial dose of steroids was significantly low in the relapse group (P = 0.020).In conclusion, progression is associated with various systemic conditions in patients who show progression.A low dose of initial steroids is significantly associated with relapse.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine and Lung Institute, Seoul National University College of Medicine, Seoul, Korea.

ABSTRACT
The purpose of this study was to investigate the long-term clinical course of non-specific interstitial pneumonia (NSIP) and to determine which factors are associated with a response to steroid therapy and relapse. Thirty-five patients with pathologically proven NSIP were included. Clinical, radiological, and laboratory data were reviewed retrospectively. The male-to-female ratio was 7:28 (median age, 52 yr). Thirty (86%) patients responded to steroid therapy, and the median follow-up was 55.2 months (range, 15.9-102.0 months). Five patients (14%) showed sustained disease progression and three died despite treatment. In the five with sustained disease progression, NSIP was associated with various systemic conditions, and the seropositivity of fluorescent antinuclear antibody was significantly associated with a poor response to steroids (P = 0.028). The rate of relapse was 25%, but all relapsed patients improved after re-treatment. The initial dose of steroids was significantly low in the relapse group (P = 0.020). In conclusion, progression is associated with various systemic conditions in patients who show progression. A low dose of initial steroids is significantly associated with relapse.

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Related in: MedlinePlus

Clinical courses of the patients with nonspecific interstitial pneumonia (NSIP).
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Figure 1: Clinical courses of the patients with nonspecific interstitial pneumonia (NSIP).

Mentions: The follow-up results of the enrolled patients are summarised in Fig. 1. All 35 patients received corticosteroid therapy alone as the initial treatment, and cytotoxic agents were added in seven patients (four patients cyclophosphamide; three patients azathioprine) who showed rapid disease progression or steroid dependency. The median dose of initial prednisolone was 0.54 mg/kg/day. A higher dose of initial steroids (> 1.5 mg/kg/day) was used in only two patients, 9.5 mg/kg/day in one patient who showed rapid disease progression, and 1.8 mg/kg/day in another who showed arterial hypoxemia on room air (PaO2, 54.3 mmHg). The range of initial prednisolone doses used in patients except these two was 0.4-1.1 mg/kg/day. The prednisolone dose was slowly tapered after 4-6 weeks based on the clinical and PFT evaluation of early response. Thirty (86%) patients responded to steroid therapy, and five (14%) were considered non-responders. Three of the 5 non-responders died of disease progression with combined pneumonia, although all had received additional cyclophosphamide pulse therapy. The patients died 1.6, 1.7, and 5.2 months after beginning treatment. In contrast, steroid responders were all alive at the end of the follow-up period. Six (20%) steroid responders were stable but were considered steroid dependent because the disease worsened when steroids were reduced. Therefore, steroid treatment was maintained in these six patients for the entire follow-up period, and azathioprine was added in two patients. The maintenance dose of prednisolone was 5 mg in five patients and 15 mg in one patient. The 1- and 5-yr survival rates in all patients were 97.1% and 93.9%, respectively.


Treatment response and long term follow-up results of nonspecific interstitial pneumonia.

Lee JY, Jin SM, Lee BJ, Chung DH, Jang BG, Park HS, Lee SM, Yim JJ, Yang SC, Yoo CG, Han SK, Shim YS, Kim YW - J. Korean Med. Sci. (2012)

Clinical courses of the patients with nonspecific interstitial pneumonia (NSIP).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3369453&req=5

Figure 1: Clinical courses of the patients with nonspecific interstitial pneumonia (NSIP).
Mentions: The follow-up results of the enrolled patients are summarised in Fig. 1. All 35 patients received corticosteroid therapy alone as the initial treatment, and cytotoxic agents were added in seven patients (four patients cyclophosphamide; three patients azathioprine) who showed rapid disease progression or steroid dependency. The median dose of initial prednisolone was 0.54 mg/kg/day. A higher dose of initial steroids (> 1.5 mg/kg/day) was used in only two patients, 9.5 mg/kg/day in one patient who showed rapid disease progression, and 1.8 mg/kg/day in another who showed arterial hypoxemia on room air (PaO2, 54.3 mmHg). The range of initial prednisolone doses used in patients except these two was 0.4-1.1 mg/kg/day. The prednisolone dose was slowly tapered after 4-6 weeks based on the clinical and PFT evaluation of early response. Thirty (86%) patients responded to steroid therapy, and five (14%) were considered non-responders. Three of the 5 non-responders died of disease progression with combined pneumonia, although all had received additional cyclophosphamide pulse therapy. The patients died 1.6, 1.7, and 5.2 months after beginning treatment. In contrast, steroid responders were all alive at the end of the follow-up period. Six (20%) steroid responders were stable but were considered steroid dependent because the disease worsened when steroids were reduced. Therefore, steroid treatment was maintained in these six patients for the entire follow-up period, and azathioprine was added in two patients. The maintenance dose of prednisolone was 5 mg in five patients and 15 mg in one patient. The 1- and 5-yr survival rates in all patients were 97.1% and 93.9%, respectively.

Bottom Line: The initial dose of steroids was significantly low in the relapse group (P = 0.020).In conclusion, progression is associated with various systemic conditions in patients who show progression.A low dose of initial steroids is significantly associated with relapse.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine and Lung Institute, Seoul National University College of Medicine, Seoul, Korea.

ABSTRACT
The purpose of this study was to investigate the long-term clinical course of non-specific interstitial pneumonia (NSIP) and to determine which factors are associated with a response to steroid therapy and relapse. Thirty-five patients with pathologically proven NSIP were included. Clinical, radiological, and laboratory data were reviewed retrospectively. The male-to-female ratio was 7:28 (median age, 52 yr). Thirty (86%) patients responded to steroid therapy, and the median follow-up was 55.2 months (range, 15.9-102.0 months). Five patients (14%) showed sustained disease progression and three died despite treatment. In the five with sustained disease progression, NSIP was associated with various systemic conditions, and the seropositivity of fluorescent antinuclear antibody was significantly associated with a poor response to steroids (P = 0.028). The rate of relapse was 25%, but all relapsed patients improved after re-treatment. The initial dose of steroids was significantly low in the relapse group (P = 0.020). In conclusion, progression is associated with various systemic conditions in patients who show progression. A low dose of initial steroids is significantly associated with relapse.

Show MeSH
Related in: MedlinePlus