Prognostic significance of body mass index and weight loss in patients with idiopathic pleuroparenchymal fibroelastosis.

BACKGROUND AND AIM: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonias; its physical characteristics include a slender build with platythorax and progressive weight loss. However, the clinical significance of body mass index (BMI) and weight loss remains unclear in patients with IPPFE. Therefore, we aimed to clarify the association between baseline BMI, weight loss after diagnosis, and the prognosis of patients with IPPFE. METHODS: This retrospective study included 71 patients diagnosed with IPPFE at our institution between 2005-2021. BMI at diagnosis was classified into three: underweight (<18.5 kg/m2), normal weight (≥18.5 to <25.0 kg/m2), or overweight (≥25.0 kg/m2). An annual rate of weight change after the diagnosis was evaluated, and ≥5% per year decrease was defined as a significant weight loss. We investigated clinical features and prognosis based on baseline BMI and weight loss. RESULTS: Of the 71 patients, 48 (67.6%) and 23 (32.4%) were classified as underweight and normal weight, respectively, and none were overweight. Significant weight loss occurred in 24 (33.8%) patients, and they tended to have more cases of dyspnea and had significantly older age, lower BMI, higher rates of co-existence of lower-lobe interstitial lung disease, lower pulmonary function test results and higher incidence of pneumothorax after the diagnosis than those without weight loss. Patients with BMI <18.5 kg/m2 and those with weight loss had a significantly worse prognosis than those with BMI ≥18.5 kg/m2 or those without weight loss, respectively (p=0.005, p<0.001). Multivariate analysis revealed that low BMI and weight loss were independent poor prognostic factors. CONCLUSIONS: Low BMI and weight loss are associated with poor prognosis in patients with IPPFE.

Clinical features of idiopathic pleuroparenchymal fibroelastosis with progressive phenotype showing a decline in forced vital capacity.

BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is heterogeneous, with some patients showing a progressive decline in forced vital capacity (FVC). However, the clinical features of these cases with progressive phenotypes remain unknown. METHODS: This retrospective study included 48 patients diagnosed with IPPFE who underwent longitudinal pulmonary function tests at our institution from 2005 to 2021. The progressive phenotype was defined as a relative decline of ≥10% in %FVC within two years from diagnosis of IPPFE, and its clinical features were evaluated. RESULTS: Of the 48 patients, 23 (47.9%) were classified as progressive IPPFE. They were significantly older with a higher rate of dyspnea, fine crackles on chest auscultation, lower-lobe usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography, and lower %FVC at diagnosis than non-progressive IPPFE. Additionally, progressive IPPFE had a significantly higher rate of long-term oxygen therapy requirement, the incidence of pneumothorax, and weight loss after diagnosis, which showed worse survival than non-progressive IPPFE. The relative decline in %FVC and weight loss showed a significant positive correlation. Multivariate analysis revealed that lower body mass index tended to predict early progression, and the coexistence of lower-lobe UIP pattern was significantly associated with early progression. A decline in %FVC was an independent poor prognostic factor in IPPFE. CONCLUSIONS: With a progressive decline in %FVC, IPPFE often has an advanced stage at diagnosis and lower-lobe UIP pattern and is associated with weight loss and worse survival.

Comparison of clinical utility between digital and analog drainage systems in patients with spontaneous pneumothorax.

BACKGROUND: Digital drainage systems can continuously and numerically monitor air leakage, which may lead to a shorter duration of drainage and hospitalization; however, the usefulness of digital drainage systems compared to that of analog drainage systems for patients with primary or secondary spontaneous pneumothorax remains unclear. METHODS: This retrospective study included 108 patients with spontaneous pneumothorax who were successfully treated with chest drainage alone at our institution. We compared the clinical efficacy of digital and analog chest drainage systems. RESULTS: From the study population, 68 patients were diagnosed with primary and the other 40 with secondary spontaneous pneumothorax. The analog drainage system was used in 44 patients, and the digital drainage system in 64 patients. Among patients with primary spontaneous pneumothorax, the digital group had a significantly shorter duration of chest drainage than the analog group (median 2 vs. 4 days; p = 0.001), but there was no significant difference in those with secondary spontaneous pneumothorax. Additionally, the length and cost of hospitalization in the digital group were significantly lower than those in the analog group for both patients with primary and secondary spontaneous pneumothorax. There was no significant difference in recurrence within 1 week after chest tube removal between the two groups, neither among patients with primary nor among those with secondary pneumothorax. CONCLUSIONS: Digital drainage system may be better than analog drainage system for patients with primary spontaneous pneumothorax who need chest drainage, but further research is needed on drainage system selection for those with secondary disease.

Sarcoid-like Granulomatous Lung Disease with Subacute Progression in Silicosis.

A 67-year-old man was admitted to our hospital with cough and fatigue. He had had long-term exposure to silica due to cement processing. Chest computed tomography showed bilateral centrilobular nodules, and hilar and mediastinal lymphadenopathy with calcification, suggesting chronic silicosis. Within a few months, these nodules enlarged, and bilateral patchy consolidations appeared. A lung biopsy revealed sarcoid-like granulomas with birefringent particles under polarized light without malignancy or infection. He was diagnosed with silicosis-associated sarcoid-like granulomatous lung disease, rather than sarcoidosis, according to the clinicopathological findings. His pulmonary manifestations improved after the discontinuation of silica exposure and combination therapy of corticosteroid and azathioprine.