Two cases of idiopathic pulmonary hemosiderosis: analysis of chest CT findings]

Chest CT findings are reported in two cases of idiopathic pulmonary hemosiderosis. In both cases, CT was performed after remission of an acute exacerbation following corticosteroid therapy. Case 1 was a 17-year-old woman with Down's syndrome. Chest radiograph showed diffuse ground-glass like and reticulonodular shadows, which were predominant in the bilateral lower lung fields. Chest CT showed a diffuse increase of lung filed density, especially in the dorsal zone of both lower lobes. Open lung biopsy revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli, and in addition, marked fibrous thickening of the alveolar septa. Case 2 was a 7-year-old girl. Chest radiograph showed diffuse micronodular shadows in both lungs. Chest CT showed diffuse poorly-circumscribed micronodular lesions with uniform distribution, and lung field density was normal except for the right upper lobe with patchy infiltrates. Although lung biopsy was not performed, fibrous thickening of the alveolar septa was presumed to be mild even if present, since pulmonary function and blood gas analysis were within normal limits. In these two cases, lung field density of CT seemed to reflect the degree of diffuse fibrous thickening of the alveolar septa, and it is suggested that CT is valuable in the evaluation of fibrous thickening of the alveolar septa secondary to recurrent pulmonary hemorrhages. Comprehensive review of CT findings of idiopathic pulmonary hemosiderosis was also performed.     

A case of tuberous sclerosis with pulmonary lymphangiomyomatosis]

A 52-year-old woman complained of exertional dyspnea. She had two sons with tuberous sclerosis and presented with facial angiofibroma and bilateral renal angiomyolipomas. Her chest radiograph demonstrated diffuse reticular shadows and chest CT revealed numerous well-defined cysts outlined by thin walls. Pulmonary lymphangiomyomatosis was diagnosed by transbronchial biopsy that showed multifocal proliferation of immature smooth muscle cells located in the walls of some bronchi, and in alveolar walls and pulmonary vessels. Immunoreactivity for SMA and HMB 45 was present in the same cells, but immunostaining for estrogen and progesterone receptors was negative. Much is still unknown about pulmonary lymphangiomyomatosis and pulmonary involvement in tuberous sclerosis. Further studies, such as gene analysis, will clarify the relationship between these diseases.     

Pulmonary lymphangiomyomatosis

Pulmonary lymphangiomyomatosis is a disease of young females, who typically present with progressive dyspnea, hemoptysis, cough, repeated spontaneous pneumothoraces, and chylous effusions. The disease should be suspected when these findings are associated with an obstructive ventilatory impairment and disproportionately abnormal gas exchange. Symptoms may occur in the presence of radiographically normal-appearing pulmonary parenchyma. As the disease progresses, diffuse, prominent interstitial markings, suggestive of pulmonary fibrosis, are seen radiographically. The diagnosis is made by open lung biopsy.     

不同肺活检方法诊断肺癌的价值比较

目的对比分析3种肺活检不同方法在疑似肺癌中诊断中的价值和利弊。方法门诊及住院患者经肺部X线照片和CT检查结果诊断为肺部块影而疑似肺癌的患者78例,分别应用经支气管镜肺活检(TBLB)、CT下经皮肺活检、外科肺活检进行检查,对病理的及临床资料进行对比分析。结果报道78例疑似肺癌患者,诊断率为46.15%。TBLB术确诊率为39.5%,外科肺活检术确诊率为91.7%。CT下经皮肺活检术确诊率为35.7%。而Ⅰ～ⅢA检出率为34.6%。并发症发生率依次为外科肺活检、CT引导下肺活检、TBLB。结论 TBLB是诊断肺癌的首选确诊手段,其优点为阳性率高、费用低、并发症小,有利于肺癌的鉴别诊断和分期,而位于周边型肺癌应首选CT下经皮肺活检,对于其他未能确诊且疑难肺部肿块者宜选用外科肺活检。     

Pulmonary lymphangiomyomatosis (LAM) developing chylothorax

We describe a case of pulmonary lymphangiomyomatosis (LAM) with chylothorax that developed in a 46-year-old Japanese woman. This patient exhibited clinical symptoms of dyspnea and chest X-ray showed right pleural effusion. Thoracocentesis demonstrated chylous effusion. Chest computed tomography (CT) scan revealed multiple cystic lesions. Subsequent thoracoscopy revealed the chylorrhea from swelled vessels on the diaphragm. The clinical diagnosis, based on histological examinations with biopsy specimens obtained by thoracoscopy, was pulmonary LAM. Although the hormone therapy was not effective, chylous effusion was improved by the pleurodesis. Pulmonary LAM developing chylothorax is rare in Japan.     

A case of multicentric Castleman's disease with pulmonary involvement]

In a man aged 34 who had been experiencing frequent coughing since November 2001, a chest radiograph showed infiltration shadows in both lung fields. Chest CT showed diffuse centrilobular nodules and multiple mediastinal lymphadenopathy. Laboratory examination revealed high values for C-reactive protein and the erythrocyte sedimentation rate, together with polyclonal hyperimmunoglobulinemia and an elevated interleukin-6 level. We suspected multicentric Castleman's disease, and so performed thoracoscopic mediastinal lymph node biopsy and lung biopsy. The former disclosed follicular hyperplasia and plasma cell infiltration in the interfollicular area, suggesting a diagnosis of Castleman's disease, plasma cell type. The lung biopsy showed heavy infiltration of plasma cells. The diagnosis was therefore multicentric Castleman's disease (MCD) with pulmonary involvement. The chest CT findings were tpical characteristics of pulmonary involvement in patients with MCD.     

隐源性机化性肺炎的临床病理特征和影像学表现

目的探讨隐源性机化性肺炎(COP)的临床病理特征和影像学表现。方法分析5例隐源性机化性肺炎病例的临床特点、影像学表现、肺活检的病理特征,并复习相关文献。结果 COP常见的临床表现为咳嗽、进行性呼吸困难和吸气末肺部爆裂音。肺活检病理检查显示肺泡管、肺泡腔内见肉芽组织栓。胸部CT表现为含支气管充气征的实变阴影,伴或不伴磨玻璃影。患者对糖皮质激素治疗有显著疗效。结论临床表现结合影像学特点可提示COP临床诊断,肺活检是诊断COP有效的检查方法。     

Identification of propionibacterium acnes in a case with stage III pulmonary sarcoidosis involving hepatosplenic disease]

A 35-year-old woman was admitted to our hospital because of an abnormal chest radiograph. Chest X-ray on admission showed multiple small nodular shadows in both lung fields but no bilateral hilar lymphadenopathy (BHL). Moreover, abdominal CT showed some nodules in the liver and spleen, and serum ACE was slightly increased to 23.3U/L (normal range: 8.3-21.4U/L). Transbronchial lung biopsy and liver biopsy resulted in a diagnosis of stage III pulmonary sarcoidosis with hepatosplenic disease. Histopathological findings demonstrated non-caseating epithelioid cell granulomas with giant cells in both specimens. Interestingly, propionibacterium acnes (P. acnes), the possible pathogen of sarcoidosis, was detected in giant cells in the lung and epitheliod cell granuloma of liver tissue. This case was of interest considering P. acnes might have been the causative pathogen.     

肺淋巴管肌瘤病并肺母细胞瘤1例临床分析并文献复习

目的 加强肺淋巴管肌瘤病（LAM）合并恶性肿瘤的临床、影像、病理特点的认识,提高对该病的早期认识和治疗水平.方法 分析2013年3月收治的1例肺LAM并肺母细胞瘤患者临床资料及诊治经过,并结合相关文献进行复习.截止至2014年2月,以“Lymphangiomyomatosis、cancer”为检索词,在PubMed检索系统进行检索,论著3篇,综述1篇;在万方数据库中以“淋巴管肌瘤病、恶性肿瘤”为检索词进行检索,病例报告1篇.结果 患者,女,37岁,慢性病程,主要症状为咳嗽、咯血、胸痛3个月,既往有“双肾错构瘤”史,曾行右肾错构瘤切除术,术后病理示血管平滑肌脂肪瘤,左肾未予处理.曾有“自发性气胸”病史.胸部高分辨率CT示右中下肺巨大肿块并纵隔淋巴结肿大,考虑恶性病变;肺LAM.血清血管内皮生长因子D为1 092.61 ng/L.免疫组化示CD56灶（＋）,Vim小细胞（＋）,Syn（＋）,CD99（＋/-）,组织改变考虑为肺母细胞瘤.复习国内外文献,尚无LAM合并肺母细胞瘤的病例报道.结论 近十年来,随着LAM分子发病机制的深入研究,LAM目前可定义为低度恶性转移性肿瘤.本例患者为LAM合并高度恶性肿瘤肺母细胞瘤,在国内外尚属首例报道.但究其机制,是LAM细胞癌变为肺母细胞瘤,或是二者同时存在,目前尚无定论.     

A case of pulmonary lymphangiomyomatosis induced by pregnancy

A 35-year-old primigravida was admitted to the Department of obstetrics complaining of dyspnea and left back pain at 21 weeks' gestation. Chest roentgenogram revealed diffuse reticulonodular shadows predominantly in both lower lung fields and arterial hypoxemia was present. Pulmonary function tests showed restrictive impairment and decreased carbon monoxide diffuse capacity. From these results, interstitial pneumonia was suspected and she was first treated with prednisolone. However during her pregnancy, spontaneous pneumothorax occurred. Following spontaneous delivery of healthy infant at 37 weeks, left chylothorax occurred, and pleurodesis was performed with OK432. Thereafter the histological diagnosis of pulmonary lymphangiomyomatosis was made by transbronchial lung biopsy and treatment of prednisolone was stopped. She was treated with tamoxifen. In addition, progesterone-receptor was detected in the pulmonary tissue obtained at open lung biopsy. She was treated with cyclophosphamide in addition to tamoxifen. At present, shortness of breath has decreased slightly in comparison with one year previously, but no improvement has been seen in lung function tests or chest roentgenogram.     

Three cases of the nodular pulmonary amyloidosis with a longterm observation

Longterm observation with chest radiograph and computed tomography (CT) scan was performed for pulmonary amyloidosis. There are few reports of primary pulmonary amyloidosis with a longterm observation. We encountered three cases of nodular pulmonary amyloidosis observed by intermittent chest radiograph or CT for 5 years or more. The patients were a 54-year-old man, and 67- and 68-year old women. For diagnosis, transbronchial biopsy and percutaneous lung biopsy were performed. Amyloid nodules grew slowly and two cases showed findings of cavity and calcification.     

A case of sarcoidosis with atypical high resolution CT findings]

A 36-year-old woman was referred to our hospital for investigation of erythema on the upper arms and thighs and abnormal radiological findings in the chest. A chest radiograph showed poorly defined fine nodules in both lung fields and high-resolution CT revealed fine nodular opacities with a random distribution throughout both lungs. No lymph node enlargement or peribronchovascular interstitial thickening were seen. Bronchoscopic evaluation revealed bronchial mucosal hypervascularity. Transbronchial lung biopsy and skin biopsy specimens showed noncaseating epithelioid cell granulomas. These findings confirmed the diagnosis of sarcoidosis. This is a rare case of sarcoidosis presenting with atypical high-resolution CT findings, which are difficult to differentiate from those of miliary tuberculosis or metastatic carcinoma.     

Pulmonary lymphangiomyomatosis. Demonstration of smooth muscle antigens by immunofluorescence technique.

A case is reported of pulmonary lymphangiomyomatosis in 44-year-old woman presenting with progressive exertional dyspnoea, unproductive cough and haemoptysis. The patient showed no symptoms or signs of tuberous sclerosis, a condition often found in association with pulmonary lymphangiomyomatosis. A pulmonary biopsy specimen showed disruption of alveolar septa and fibrosis. Subpleural lymph vessels and pulmonary venules showed cuffs of spindle-shaped cells. The leiomyomatous nature of these cells was evident from their appearance and staining reactions with light microscopy, and the presence of smooth muscle antigens as demonstrated by immunofluorescence technique. Since the lesion is not restricted to lymphatics, but can also be present in blood vessels, the condition may more appropriately be designated as pulmonary angiomyomatosis.     

A case of sarcoidosis and idiopathic thrombocytopenic purpura accompanied with invasive pulmonary aspergillosis]

A 54-year-old woman with a 21-year history of sarcoidosis was admitted to our hospital with dyspnea on exertion, weight loss, and the appearance of consolidation in chest radiographs. The serum level of soluble IL-2 receptor was high, and CT findings demonstrated mediastinal, hilar and abdominal lymphadenopathy. The histological findings of subpleural consolidation in a transbronchial lung biopsy of the left lung showed giant cells; and those of a CT-assisted biopsy of a retroperitoneal lymph node revealed non-caseous epithelioid cell granulomas. After the biopsy, severe thrombocytopenia (6,000/microliter) developed. With prednisolone treatment, the platelet count rose to normal and the subpleural consolidation on chest radiography was improved. Five weeks later, the had a productive cough with fever, rapidly progressive cavitary lesions and consolidation on chest radiography. Aspergillus fumigatus was detected in the sputum by PCR, and Aspergillus antigen was detected in the serum. She died of progressive respiratory failure, in spite of therapy with amphotericin B and itraconazole. We report a rare case of sarcoidosis and idiopathic thrombocytopenic purpura accompanied with invasive pulmonary aspergillosis.     

Sj?gren's syndrome with multiple bullae and pulmonary nodular amyloidosis]

We report a case of Sj?gren's syndrome with pulmonary involvement diagnosed by open lung biopsy. The patient was a 62-year-old woman with antiphospholipid antibody syndrome. Her chest radiograph and CT scan showed multiple bullae diffusely scattered throughout the lung. The open lung biopsy specimens revealed marked inflammatory mononuclear cell infiltration and nodular amyloid deposits in the bronchiolar walls. The mechanism of bulla formation appeared to be the check valve mechanism caused by the narrowing of the airway by the bronchiolitis. The patient was treated with oral corticosteroids, and her symptoms and laboratory findings became stable.     

Erdheim-Chester disease presenting with pulmonary lesion]

A 49-year-old man first visited our hospital in 1991 for further examination of abnormal pulmonary shadows. A chest radiograph and computed tomographic (CT) scan showed diffuse reticular shadows in both lung fields. The findings from a transbronchial lung biopsy specimen were not conclusive. Although there was little change in the abnormal pulmonary shadows, the patient's lung functions gradually deteriorated, indicating an obstructive defect. The patient was admitted in 1998 with the chief complaint of increasing dyspnea on exertion. A thoracoscopic lung biopsy specimen revealed proliferation of histiocytes with fibrosis in the pleura and perivascular interstitium. Immunohistochemically, the histiocytic cells were CD68-positive, alpha 1-antichymotripsin-positive, S100 protein-negative, and CD1a-negative. A bone scintigram and magnetic resonance images showed symmetrical diametaphyseal bone lesions in the distal femurs and the proximal tibiae; however, the epiphyses were spared. These findings were consistent with Erdheim-Chester disease. This is the first reported case of Erdheim-Chester disease with pulmonary involvement in Japan.     

A 14-year-old with pulmonary hamartomatous lymphangiomyomatosis associated with bilateral pneumothoraces]

A 14-year-old girl was admitted because of cough, chest pain and hemosputum. Chest roentgenogram on admission showed a pneumothorax and a cavitary lesion with niveau formation in the right lung and cystic lesions in the bilateral lung fields. After bed rest and intravenous administration of antibiotics for two weeks, the right lung inflated well and the niveau formation disappeared, and the patient was discharged. One week later, she was readmitted with sudden-onset severe dyspnea, caused by bilateral pneumothoraces. Emergency tube thoracostomy and wedge resection of the bullous lesion was performed. Macroscopically, multiple small cystic changes were seen on the surface of the right lung. Histological examination revealed nodular proliferations of smooth muscle cells in the interstitium and vessel walls in the lung, which contained slit-like lymphatic channels. The diagnosis of pulmonary lymphangiomyomatosis was made. In this case, we could not measure receptors for estrogen and progesterone. Recently, hormonal therapy and oophorectomy have been reported as being useful. Tamoxifen (Norvadex) was therefore initiated, and the patient has remained well with slight dyspnea on exertion. There has been no recurrence of pneumothorax. Lymphangiomyomatosis is a rare disease of unknown etiology which occurs exclusively in women, mostly in those of reproductive age. We report a 14-year-old female patient with lymphangiomyomatosis associated with repeated pneumothorax, who had been under treatment for epilepsy. We believe this case to be of importance because of the long discussed relation between pulmonary lymphangiomyomatosis and tuberous sclerosis.     

Three cases of pulmonary eosinophilic granuloma]

CASE 1. A 55-year-old man was admitted because of an abnormality in chest radiographs. Chest HRCT showed multiple cystic lesions with thick and thin walls, and nodules; and strongly suggested pulmonary eosinophilic granuloma (EG). Open lung biopsy revealed granuloma formation and a fibrotic area consistent with EG. CASE 2. A 28-year-old woman was admitted because of chest pain and dyspnea. Chest radiography revealed bilateral pneumothorax. Chest HRCT showed multiple cystic lesions with thick walls involving the entire lung. As lung biopsy revealed, proliferative lesions and cavitation containing S-100 protein-positive histiocytes, we diagnosed this case as pulmonary EG in the active stage. CASE 3. A 32-year-old woman was admitted because of dyspnea. Chest CT showed bullous changes in the lung. As an open lung biopsy revealed, honeycomb changes with S-100 protein-positive histiocytes, this case was diagnosed as the regressive phase of EG. It is known that the chest radiography and HRCT findings of EG are characteristic and vary with the stage, so a surgical lung biopsy is necessary for diagnosis of EG. It is considered that the prognosis of the active phase of EG involving the entire lung is poor.     

A case of arc welder's lung with ground-glass opacities and progressive massive fibrosis]

A 62-year-old man who had worked as a welder for 35 years was admitted with abnormal chest radiograph shadows. Chest CT scan showed ground-glass opacities (GGO) and nodular shadows (progressive massive fibrosis: PMF) with spiculation in both lung fields. Transbronchial lung biopsy (TBLB) findings of a nodule (left segment 8) revealed many iron particles in the alveoli and positive staining for Fe (Berlin blue stain). Moreover, bronchoalveolar lavage (BAL) fluid of a GGO (left segment 4) revealed many iron particles and positive staining for Fe (Berlin blue stain) in macrophages. Serum ferritin was extremely high (6.352 ng/ml) and ferritin in the BAL fluid was 210 ng/ml. Taking the clinical course and pathological findings together, pneumoconiosis (arc welder's lung) was diagnosed. The most common chest CT pattern in arc welder's lung is ill-defined micronodules diffusely distributed in the lung like hypersensitivity pneumonitis. Arc welder's lung rarely presents as PMF. We report a case of arc welder's lung accompanied with PMF.