The Study Group of Pneumothorax and Cystic Lung Diseases

Setagaya Ku, Japan

The Study Group of Pneumothorax and Cystic Lung Diseases

Setagaya Ku, Japan
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Nishino K.,Juntendo University | Nishino K.,The Study Group of Pneumothorax and Cystic Lung Diseases | Yoshimi K.,Juntendo University | Yoshimi K.,The Study Group of Pneumothorax and Cystic Lung Diseases | And 16 more authors.
Internal Medicine | Year: 2017

This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract. Endoscopic mucosal biopsy revealed colonic lymphangiectasia in one patient; whereas the site in the other patient was intestinal. Treatment with sirolimus led to the complete resolution of PLE within several months; additionally, marked shrinkage was observed in the lymphangioleiomyomas of both cases. These findings suggest that colonic or intestinal lymphatic congestion due to neighboring lymphangioleiomyomas was the mechanism for the development of PLE. At the time of writing this report, the beneficial effect of sirolimus has lasted for more than 3 years. © 2017 The Japanese Society of Internal Medicine.


Ando K.,Juntendo University | Ando K.,The Study Group of Pneumothorax and Cystic Lung Diseases | Kurihara M.,Nissan Tamagawa Hospital | Kurihara M.,The Study Group of Pneumothorax and Cystic Lung Diseases | And 12 more authors.
Respiratory Investigation | Year: 2013

Background: Lymphangioleiomyomatosis (LAM) is a rare disease caused by dysregulated activation of the mammalian target of rapamycin (mTOR). Sirolimus, an inhibitor of mTOR, has been reported to decrease the size of angiomyolipomas and stabilize pulmonary function in patients with LAM. However, the optimal dose for the treatment of LAM remains unclear. Methods: We conducted a retrospective, observational study of 15 patients with LAM who underwent sirolimus therapy for more than 6 months. The efficacy was evaluated by reviewing the patients' clinical courses, pulmonary function and chest radiologic findings before and after the initiation of sirolimus treatment. Results: All patients had blood trough levels of sirolimus lower than 5ng/mL. Sirolimus treatment improved the annual rates of change in FVC and FEV1 in the 9 patients who were free from chylous effusion (FVC, -101.0 vs. +190.0mL/y, p=0.046 and FEV1, -115.4 vs. +127.8mL/y, p=0.015). The remaining 7 patients had chylous effusion at the start of sirolimus treatment; the chylothorax resolved completely within 1-5 months of treatment in 6 of these cases. These results resembled those of previous studies in which blood trough levels of sirolimus ranged from 5 to 15ng/mL. Conclusions: Low-dose sirolimus (trough level, 5. ng/mL or less) performed as well as the higher doses used previously for improving pulmonary function and decreasing chylous effusion in patients with LAM. © 2013 The Japanese Respiratory Society.


Kunogi Okura M.,Juntendo Tokyo Koto Geriatric Medical Center | Kunogi Okura M.,The Study Group of Pneumothorax and Cystic Lung Diseases | Yae T.,Juntendo Tokyo Koto Geriatric Medical Center | Nagashima O.,Juntendo Tokyo Koto Geriatric Medical Center | And 4 more authors.
Internal Medicine | Year: 2013

Spontaneous pneumothorax in the elderly commonly occurs due to underlying pulmonary diseases, such as chronic obstructive pulmonary disease, interstitial lung disease, lung cancer, etc. A 73-year-old woman developed pneumothorax for the first time that was a clinical clue to a diagnosis of Birt-Hogg-Dubé syndrome (BHDS), an autosomal dominant condition characterized by fibrofolliculomas of the skin, renal tumors and multiple lung cysts predisposing to pneumothorax. Although BHDS patients frequently develop pneumothorax during their twenties to forties, the present case indicates that BHDS should be considered as an underlying cause of pneumothorax in the elderly with undisclosed BHDS. © 2013 by The Japanese Society of Internal Medicine.


Murakami A.,Juntendo University | Hayashi T.,Juntendo University | Hayashi T.,The Study Group of Pneumothorax and Cystic Lung Diseases | Terao Y.,Juntendo University | And 6 more authors.
Internal Medicine | Year: 2014

A 57-year-old woman, who had undergone hysterectomy for uterine myoma 11 years earlier presented with cystic, nodular and cavitary lesions simultaneously visible on computed tomography images of the chest. Histological examinations of both the resected lung and past "myoma" specimens demonstrated that the original uterine tumor was a low-grade endometrial stromal sarcoma (ESS) that had metastasized to the lungs. No previous reports have described the coexistence of cystic, nodular and cavitary lesions with pulmonary metastasis of ESS; however, we successfully correlated the radiologic appearance with the corresponding pathologic findings. Medroxyprogesterone acetate therapy has effectively kept the patient asymptomatic for approximately five years. © 2014 The Japanese Society of Internal Medicine.


Kumasaka T.,Red Cross | Kumasaka T.,The Study Group of Pneumothorax and Cystic Lung Diseases | Hayashi T.,The Study Group of Pneumothorax and Cystic Lung Diseases | Hayashi T.,Juntendo University | And 18 more authors.
Histopathology | Year: 2014

Aims: To characterize the pathological features of pulmonary cysts, and to elucidate the possible mechanism of cyst formation in the lungs of patients with Birt-Hogg-Dubé syndrome (BHDS), a tumour suppressor gene syndrome, using histological and morphometric analyses. Methods and results: We evaluated 229 lung cysts from 50 patients with BHDS and 117 from 34 patients with primary spontaneous pneumothorax (PSP) for their number, size, location and absence or presence of inflammation. The BHDS cysts abutted on interlobular septa (88.2%) and had intracystic septa (13.6%) or protruding venules (39.5%) without cell proliferation or inflammation. The frequencies of these histological characteristics differed significantly from those seen in the lungs of patients with PSP (P < 0.05). Although the intrapulmonary BHDS cysts were smaller than the subpleural BHDS cysts (P < 0.001), there was no difference in size between them when there was no inflammation. The number of cysts diminished logarithmically and the proportion of cysts with inflammation increased as their individual sizes became greater (P < 0.05). Conclusions: These results imply that the BHDS cysts are likely to develop in the periacinar region, an anatomically weak site in a primary lobule, where alveoli attach to connective tissue septa. We hypothesize that the BHDS cysts possibly expand in size as the alveolar walls disappear at the alveolar-septal junction, and grow even larger when several cysts fuse. © 2014 The Authors. Histopathology Published by John Wiley & Sons Ltd.


PubMed | Juntendo University and The Study Group of Pneumothorax and Cystic Lung Diseases
Type: Journal Article | Journal: Physiological reports | Year: 2016

Birt-Hogg-Dub syndrome (BHDS) is an autosomal dominant inherited disorder caused by germline mutations in the FLCN gene, and characterized by skin fibrofolliculomas, multiple lung cysts, spontaneous pneumothorax, and renal neoplasms. Pulmonary manifestations frequently develop earlier than other organ involvements, prompting a diagnosis of BHDS However, the mechanism of lung cyst formation and pathogenesis of pneumothorax have not yet been clarified. Fibroblasts were isolated from lung tissues obtained from patients with BHDS (n=12) and lung cancer (n=10) as controls. The functional abilities of these lung fibroblasts were evaluated by the tests for chemotaxis to fibronectin and three-dimensional (3-D) gel contraction. Fibroblasts from BHDS patients showed diminished chemotaxis as compared with fibroblasts from controls. Expression of fibronectin and TGF-1 was significantly reduced in BHDS fibroblasts when assessed by qPCR Addition of TGF-1 in culture medium of BHDS lung fibroblasts significantly restored these cells abilities of chemotaxis and gel contraction. Human fetal lung fibroblasts (HFL-1) exhibited reduced chemotaxis and 3-D gel contraction when FLCN expression was knocked down. To the contrary, a significant increase in chemotactic activity toward to fibronectin was demonstrated when wild-type FLCN was overexpressed, whereas transduction of mutant FLCN showed no effect on chemotaxis. Our results suggest that FLCN is associated with chemotaxis in lung fibroblasts. Together with reduced TGF-1 expression by BHDS lung fibroblasts, a state of FLCN haploinsufficiency may cause lung fibroblast dysfunction, thereby impairing tissue repair. These may reveal one mechanism of lung cyst formation and pneumothorax in BHDS patients.

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