Takako Hirota

Rapid decrease in forced vital capacity in patients with idiopathic pulmonary upper lobe fibrosis

BACKGROUND We are occasionally presented with patients with unclassifiable interstitial pneumonia of unknown etiology. Idiopathic pulmonary upper lobe fibrosis (IPUF) does not fit any of the currently defined subsets of idiopathic interstitial pneumonias (IIPs). This study was performed to examine clinical, functional, and pathological characteristics of IPUF. METHODS We present 9 cases of histologically confirmed IPUF. The clinical and histological characteristics of the 9 patients were evaluated. The baseline respiratory function of all patients was measured. There were 7 patients whose forced vital capacity (FVC) had been monitored for at least a year who were selected to quantify the yearly decline in FVC. RESULTS All patients were slender, with a body mass index of 16.0-19.8 kg/m(2). Seven patients had a history of pneumothorax. Six patients died 1.8 to 5.7 years after the onset of the first symptoms. Fundamental histological features were intraalveolar collagen deposition and densely packed elastic fibers in the subpleural areas. These findings are the same as those seen in pleuroparenchymal fibroelastosis. However, the visceral pleura was thickened with dense collagen in only 2 patients, and pleural thickening was localized, if present, in the remaining 7 patients. Ventilatory impairment was also a characteristic. The time course decline of FVC was rapid and almost linear. The median yearly decline in FVC was -20.3% (range, -7.7% to -26.5%), which was more rapid than that reported for chronic fibrosing interstitial pneumonias such as idiopathic pulmonary fibrosis. CONCLUSIONS IPUF is a unique pulmonary fibrosis that results in rapid deterioration of ventilatory function and poor prognosis.

Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection

To the Editor: Idiopathic pleuroparenchymal fibroelastosis is a peculiar pulmonary fibrosis proposed by Frankel et al . [1] in 2003 and is almost the same concept as idiopathic pulmonary upper lobe fibrosis proposed by Amitani et al. [2]. There are no known causes for fibrosis in idiopathic pleuroparenchymal fibroelastosis. Sometimes, pleuroparenchymal fibroelastosis (PPFE) has underlying diseases or conditions, such as collagen vascular diseases, anti-cancer chemotherapy, irradiation, asbestos exposure and bone-marrow transplantation [3]. Herein, we report the case of a female who received living-donor lung transplantation and died of pulmonary fibrosis, which was pathologically compatible with PPFE in addition to constrictive bronchiolitis, which is a manifestation of chronic lung allograft dysfunction (CLAD) [4]. A 30-yr-old female suffering from idiopathic pulmonary arterial hypertension underwent living-donor lung transplantation surgery and received a right lower lobe from her younger sister and a left lower lobe from her mother in December 2003. 20 months after the lung transplantation she had dyspnoea and a chest radiograph disclosed bilateral ground-glass shadows. 1 month later, right open lung biopsy was performed and a diagnosis of interstitial pneumonia was obtained. Pulse therapy with methylprednisolone slightly improved her condition and prednisolone was administered after the pulse therapy. However, bilateral interstitial opacities gradually deteriorated (fig. 1) with increased dyspnoea. 49 months after the lung transplantation, her daily life had worsened to almost whole-day bed rest. 18 days prior to her death she …

The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis

To the Editor: Pleuroparenchymal fibroelastosis (PPFE) was first reported by Frankel et al. [1]. PPFE can occur without any aetiology or underlying diseases (idiopathic PPFE), or with underlying diseases or conditions. Idiopathic PPFE has been listed as one of the rare idiopathic interstitial pneumonias (IIPs) in the revised international multidisciplinary consensus classification of IIPs [2]. The natural history of PPFE is variable, some are slowly progressive and others sometimes show rapid deterioration resulting in poor prognosis, like idiopathic pulmonary fibrosis (IPF). Idiopathic pulmonary upper lobe fibrosis (PULF), first proposed by Amitani et al. [3], is currently considered to be almost identical to idiopathic PPFE [1, 4, 5], which is now globally accepted as a representative nomenclature for this disorder. Therefore, we use the term PPFE to describe the same disease as PULF. Amitani et al. [3] recognised a characteristic constitution in patients with PPFE: they are slender and their thoracic cage is flattened, i.e. the ratio of the anteroposterior diameter of the thoracic cage (APDT) to the transverse diameter of the thoracic cage (TDT) is abnormally lower than in normal populations. Herein, we have provisionally named this deformity of the thoracic cage as “flat chest”. Other investigators have also noticed this deformity in idiopathic PPFE [6–8]. Flat chest may result from a congenital disposition or …

Histological evolution of pleuroparenchymal fibroelastosis

To investigate the histological evolution in the development of pleuroparenchymal fibroelastosis (PPFE).

Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis

BACKGROUND The histological pattern of pleuroparenchymal fibroelastosis (PPFE) is well defined, but its clinical features remain unclear. METHODS We retrospectively examined the predominantly involved lung-fields (based on abnormal opacities on computed tomography [CT] images), and the initial value and annual decline of respiratory function in patients with pulmonary fibrosis presenting with histologically confirmed PPFE. RESULTS Thirteen female and nine male subjects were included. Eleven interpreters independently analyzed 231 CT image series. One-third of the CT series (78/231) was interpreted as demonstrating equal involvement of the upper and lower lung fields, i.e., six out of 21 patients had equal involvement of the upper and lower lung fields, based on a majority decision of the interpreters. The residual volume/total lung capacity (RV/TLC) was increased and correlated inversely with forced vital capacity (FVC) at the initial measurement. FVC followed two patterns of decline over time: a gradual decline over a follow-up period of more than 6 years (-55mL/year, R(2)=0.799), and a relatively rapid decline over a shorter period (-364mL/year, R(2)=0.855) as determined by mixed-effect linear regression. CONCLUSIONS The predominantly involved sites seen on CT images of PPFE were not limited to the upper lobes. In some cases, upper lung fields were predominantly involved, but in other cases, both upper and lower lung fields were equally involved. Two patterns of FVC decline exists: a rapid decline over a short period and a slow decline over a longer period, suggesting that the disease follows a heterogeneous clinical course.

Adenoid Cystic Carcinoma of the Lung with an EGFR Mutation.

An 80-year-old woman was referred to our hospital due to the presence of a mass that was identified on a chest X-ray. A further investigation demonstrated advanced adenoid cystic carcinoma of the lungs. Anti-cancer chemotherapy with docetaxel was carried out and the lesion remained as stable disease. Subsequently, pleural effusion was detected, and an investigation of the pleural effusion revealed the existence of malignant cells with an epidermal growth factor (EGFR) mutation. Gefitinib was administered and the pleural effusion resolved. This is the first case of a positive EGFR mutation of adenoid cystic carcinoma of the lung with a favorable response to an EGFR-tyrosine kinase inhibitor.

Obstructive Ventilatory Impairment in Sarcoidosis

Ryosuke Hirano, Yuji Yoshida, Takemasa Matsumoto, Naoki Tashiro, Taishi Harada, Takako Hirota, Motokimi Shiraishi, Masaki Fujita, Kentaro Watanabe 【要旨】 サルコイドーシスの閉塞性換気障害について検討した. 2000年1月から2012年8月の間に当科で診断したサルコイドーシス症例でFEV1/FVCが70%未満の症例を後ろ向きに検 討した.スパイロメトリーが施行された75例のうち,初診時あるいは経過中にFEV1/FVCが70%未満の症例が20例(27%) あった.気管支拡張薬の吸入によって有意な可逆性を示した症例はなかったが,FEV1/FVCが70%未満の症例に小児喘息 の既往のある症例が有意に多かった.ACEを含めて各種臨床パラメーターや気管支鏡の肉眼所見と閉塞性換気障害との間 に有意な関連はなかった.一方FVC が予測値の80%未満の拘束性換気障害を呈する症例は6例のみであり,閉塞性換気障 害を示す症例が多かった. 閉塞性換気障害はサルコイドーシスにおいて稀ならず遭遇する.気流閉塞は予後との関連が報告されており,見逃さな いように留意すべき病態である. [日サ会誌 2013; 33: 79-82] キーワード:呼吸機能検査,肺サルコイドーシス,閉塞性換気障害,ネットワーク形成