Nobuhiko Nagata

Sclerosing hemangioma of the lung. Immunohistochemical characterization of its origin as related to surfactant apoprotein.

Lung tissues from 13 patients with pulmonary sclerosing hemangioma were studied with antibody against surfactant apoprotein, Factor VIII‐related antigen, or lysozyme. Surfactant apoprotein was detected in the cytoplasm of the cells lining cystic spaces and papillary projections. Surfactant apoprotein was found in a small number of stromal cells with abundant eosinophilic or clear cytoplasm and round to oval nuclei, which were characteristic in pulmonary sclerosing hemangioma as the main component. Surfactant apoprotein was also found in the stromal cells with small, dark nuclei similar to the lining cells. The lining and stromal cells contained neither Factor VIII‐related antigen nor lysozyme. Our demonstration of surfactant apoprotein in these cells provides further support for the idea that pulmonary sclerosing hemangioma primarily consists of epithelial cells with differentiation to type II pneumocytes, as was deduced from ultrastructural investigations.

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 …

Histological evolution of pleuroparenchymal fibroelastosis

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

A case of allergic bronchopulmonary aspergillosis successfully treated with itraconazole

Oral corticosteroid treatment of allergic bronchopulmonary aspergillosis (ABPA) prevents the bronchial environment from being hospitable to Aspergillus by inhibiting harmful immunologic reactions. Despite these benefits of corticosteroids, it is widely known that they have long-term side-effects. Itraconazole is a new oral triazole antifungal agent with low toxicity that is active against Aspevgillus spp. in vitro as well as in vivo (1). The present authors administered itraconazole monotherapy in a young woman with ABPA. The symptoms, signs, partial atelectasis and laboratory findings improved after 6 weeks. Although she experienced two mild relapses, the symptoms subsided without corticosteroid therapy. She has been taking itraconazole for 20 months, and no adverse effects have been observed.

Paradoxical Pleural Response to Antituberculous Chemotherapy and Isoniazid-Induced Lupus

Among 28 inpatients receiving antituberculous chemotherapies including isoniazid (INH) and rifampicin, we found 2 patients with newly developing pleural effusion in the hemithorax during the initial phase of successful chemotherapy. The analysis of the effusion fluids revealed an elevated level of antinuclear antibody and a decreased level of total component, which are findings compatible with lupus pleuritis. The peripheral lymphocyte stimulation test using INH was positive in 1 patient. To the best of our knowledge, there has been no published disussion of the relationship between a paradoxical response to antituberculous treatment and INH-induced lupus.

Pulmonary Alveolar Proteinosis and Disseminated Atypical Mycobacteriosis in a Patient with Busulfan Lung

A 32‐year‐old Japanese man with chronic granulocytic leukemia died of respiratory failure. Autopsy revealed alveolar proteinosis and pulmonary fibrosis, complicated by disseminated atypical mycobacteriosis. Epithelial hyper‐plasia caused by busulfan therapy was probably responsible for the induction of excessive surfactant production, resulting in alveolar proteinosis, and the immunosuppres‐sive state due to chronic granulocytic leukemia was probably related to the induction of disseminated atypical mycobacteriosis. Acta Pathol Jpn 40: 63–66, 1990.

Enhanced leukotriene C4 synthase activity in thioglycollate-elicited peritoneal macrophages.

The utilization of LTA4 by peritoneal macrophages (MO) obtained from untreated rats (control) as well as by those elicited from rats was investigated at designated intervals (on days 3, 7, and 14) following the intraperitoneal injection of thioglycollate (TG). On day 7 following the injection the elicited MO converted LTA4 to LTC4 at the highest rate while the resident MO showed the lowest rate. The conversion of LTA4 to LTC4 and LTB4 was next examined by using each MO lysate. The apparent LTC4 synthase activity was significantly higher in the MO lysate both on day 3 and day 7, with the latter being the highest value obtained. The GSH S-transferase activity in each lysate using as the substrate, DNCB was significantly lower on day 3 but significantly higher on day 7 as compared to control values. However, this elevated activity was less variable than that observed with LTC4 synthase. The possible implication for these observations is discussed.

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.

Metastatic Pulmonary Epithelioid Hemangioendothelioma with Peculiar Radiographic Features

We report a case of a pulmonary epithelioid hemangioendothelioma presenting as a solitary pulmonary nodule with subsequent systemic spread, including histologically documented gingival metastasis. Chest radiography and computed tomography at initial evaluation showed a solitary 2 cm pulmonary nodule, associated with pleural indentation and vascular convergence. The tumor later spread systemically, including multiple pulmonary nodules, bilateral pleural effusions, and gingival swelling and erosion. A biopsy of the gingival lesion showed histologic features identical to that of the lung biopsy.