Motokimi Shiraishi

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.

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 …

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