Usefulness of Body Composition CT Analysis in Patients with Idiopathic Pulmonary Fibrosis: A Pilot Study.

PURPOSE: To evaluate the feasibility of a chest CT-based body composition analysis in idiopathic pulmonary fibrosis (IPF), and to investigate the respective contribution of lung and muscle CT quantitative analyses to the prognosis of IPF. METHOD: A total of 71 IPF patients were recruited at diagnosis. All patients underwent a standard chest CT-scan and a bioelectrical impedance analysis considered as reference standard for estimating malnutrition through the use of the fat-free mass index (FFMI). The skeletal muscle index (SMI) was measured on chest-CT at the level of the first lumbar vertebra by two radiologists. Lung fibrosis extent was quantified by three radiologists in consensus. The extent of emphysema, the pulmonary artery to aorta (PA/AO) diameter ratio and lymph node enlargement were also reported. Mortality and hospitalization over a 14-month follow-up were recorded. RESULTS: A low FFMI defining malnutrition was identified in 26.8% of patients. SMI was significantly lower in these patients (p<0.001) and was correlated with FFMI (r=0.637, p<0.001). Interobserver agreement of SMI measurement was very good (ICC=0.91). For diagnosing malnutrition, SMI showed a 0.79 sensitivity, a 0.69 specificity, a 0.48 PPV and a 0.90 NPV. In univariate analysis, fibrosis extent was significantly associated with death, while SMI did not reach significance. In multivariate analysis, fibrosis extent and PA/AO ratio were independently associated with hospitalization. CONCLUSIONS: SMI measured on chest CT could be a reliable tool to exclude malnutrition in IPF. A quantitative analysis of both fibrosis and skeletal muscle may allow holistic management of IPF patients.

Efficacy of TCM therapy of tonifying lung-kidney's Qi-deficiency in a case of idiopathic pulmonary fibrosis: A case report.

RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a special form of spontaneous, chronic, progressive interstitial fibrotic pneumonia of unknown cause, and treatments for IPF have shown a poor prognosis. This study reports a new treatment, traditional Chinese medicine (TCM) therapy, for tonifying Qi-deficiency of lung-kidney in a 64-year-old patient with IPF. PATIENT CONCERNS: A 64-year-old man, who complained of cough and asthma, was diagnosed as IPF with mild impairment in lung function by thoracic high-resolution computed tomography and pulmonary function test. He received an 18-month N-acetylcysteine monotherapy but had no improvement in lung function. DIAGNOSES: IPF with mild impairment in lung function was diagnosed. INTERVENTIONS: The Chinese herbal medicine composition was decocted in 300 ml water for oral administration with 150 ml decoction twice daily in June 2017. OUTCOMES: The pulmonary function test showed that diffusing capacity for carbon monoxide had increased to 81% of predicted back to normal after 2-month TCM monotherapy. And diffusing capacity for carbon monoxide had increased to 89% of predicted, and forced expiratory volume in 1 s/forced vital capacity ratio increased to 92% at 14-month follow-up. No adverse events occurred during the 14 months of therapy and observation. LESSONS: The treatment by TCM therapy of tonifying lung-kidney's Qi-deficiency for IPF can improve the pulmonary function and reverse disease progression; it may be considered as a complementary treatment for IPF with mild-to-moderate impairment. However, the insights provided in this case report require further exploration and verification.

The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies.

RATIONALE: Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS. OBJECTIVES: To elucidate the long-term outcome of ARS-ILD with and without PM/DM and compare it to that of IPF. METHODS: A two-center retrospective study was conducted. The study population comprised 36 patients with ARS-ILD (8 with PM, 12 with DM, and 16 without myositis throughout the course), 100 patients with IPF without anti-ARS, and 7 patients with NSIP without anti-ARS. The presence of anti-ARS was determined by RNA immunoprecipitation using the sera obtained at the time of diagnosis before specific treatment. MEASUREMENTS AND MAIN RESULTS: During the observational period (median 49 months; range, 1-114 months), 7 patients with ARS-ILD (19%; 3 with PM, 1 with DM, and 3 without PM/DM) and 51 patients with IPF (51%) died. Patients with ARS-ILD had better overall survival than those with IPF (log-rank test, P < 0.001) and similar survival compared to those with NSIP (log-rank test, P = 0.59). The prognosis for patients with ARS-ILD was similar between those with and without myositis (log-rank test, P = 0.91). At the median follow-up time of 76.5 months, 14 of the 36 patients with ARS-ILD had deteriorated. Both a decline in forced vital capacity or an initiation of long-term oxygen therapy during the course (odds ratio [OR], 5.34) and acute exacerbation (OR, 28.4) significantly increased the mortality risk. CONCLUSIONS: The long-term outcome of ARS-ILD was significantly better than that of IPF regardless of the presence or absence of myositis.