A case of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment]

A 23-year-old man was admitted to our hospital for a complete medical evaluation of abnormal pulmonary shadows found on a chest radiograph during his annual check-up. Chest radiography and chest CT showed a diffuse spread of micronodules in both lung fields and mediastinal lymphadenopathy. A transbronchial lung biopsy demonstrated evidence of noncaseating epithelioid cell granuloma with multinucleated giant cells, and a diagnosis of sarcoidosis was made. The pulmonary shadows improved without therapy. Twenty months later, the patient was readmitted to the hospital because of chest pain and dyspnea. Pneumothorax appeared on the right in a chest radiograph, but subsided after drainage therapy, and two weeks later, a right side pleural effusion was seen. We determined that the pleura was involved in the sarcoidosis, and the patient was treated with oral prednisolone 20 mg daily. The pleural effusion gradually subsided. This is the first reported case in Japan of pulmonary sarcoidosis with pneumothorax and pleural effusion after improvement of pulmonary impairment.     

A case of pulmonary sarcoidosis with usual interstitial pneumonia-like lesions distributed predominantly in the lower lung fields]

In 2000, in a 75-year-old man, nodular and reticular opacities were detected in both lower lung fields. He was admitted to our hospital for further examination of these abnormal shadows. Bronchoscopic examination revealed pulmonary sarcoidosis. Prednisolone was prescribed because cardiac sarcoidosis was diagnosed as a clinical complication. In April 2002, the patient visited our hospital for dyspnea on effort. Chest radiography and computed tomography showed nodular and reticular opacities in the right upper lobe, and video-assisted thoracoscopic surgery was performed on the basis of a histological diagnosis. The histological findings of the biopsied specimens revealed a lesion of the type seen in usual interstitial pneumonia, whereas non-caseous granulomas were not detected. His symptoms and chest radiographic findings improved and stabilized with prednisolone and azathioprine. In the present case of pulmonary sarcoidosis, the reticular and nodular opacities predominantly distributed in both lower lung fields, and the histological findings obtained by video-assisted thoracoscopic surgery showed a usual interstitial pneumonia-like lesion. These findings may assist in the understanding of the process of development of pulmonary sarcoidosis.     

A case of small cell lung cancer associated with pulmonary sarcoidosis]

A 63-year-old man with pulmonary sarcoidosis, diagnosed by mediastinal lymph node biopsy in 1977, was admitted in Feb. 1987 because of shortness of breath and cough. Chest X-ray showed bilateral hilar lymphadenopathy and a tumor shadow in the right lung field. Histological examination of specimens biopsied from the right lung revealed small cell carcinoma (S.C.C.). Bronchoalveolar lavage was performed to evaluate the disease activity of sarcoidosis, and the total number of cells and T-lymphocytes; the ratio of CD4+ cells to CD8+ cells was not increased. He was treated with combination chemotherapy, however, he died of respiratory failure after 7 months. An autopsy was performed, and the lesions were examined histologically. The sarcoid lesion in a lymph node obtained at autopsy was not active, in contrast to that obtained by mediastinal lymph node biopsy. Lung cancer and sarcoidosis are both common diseases, but their coexistence in the same patient is not common, and autopsied cases are rare. In this case, an autopsy was performed, and BAL had been performed prior to his death. The relationship between the BAL findings and the histology of sarcoidosis was examined. Based on the results of autopsy and BAL, the sarcoidosis was inactive prior to death, but had been histologically active 10 years previously. Therefore, this is a very interesting case, since we can examine the relationship between the two diseases, and the progression of each disease. This case also provides an interesting example of differentiation of sarcoidosis from S.C.C. Metastatic invasion of the hilar lymph nodes without bronchial stenosis and changes secondary to stenosis may often occur in patients with small cell lung cancer. Such metastatic invasion closely resembles the bilateral hilar lymphadenopathy of sarcoidosis; therefore, in some cases, it may be extremely difficult to differentiate the two diseases.     

A case of pulmonary sarcoidosis with cavitation]

A 37-year-old woman, who had first been given a diagnosis of sarcoidosis at 23 years of age, was admitted to our hospital after her condition deteriorated. Chest radiographs and CT scan films showed multiple cavitary lesions with thick walls, diffuse granular shadows and bilateral hilar and mediastinal lymphadenopathy. Skin lesions were also observed. In the bronchoalveolar lavage fluid, no pyogenic bacteria, fungi, acid-fast bacilli or malignant cells were detected. A CT-guided transcutaneus lung biopsy specimen revealed a sarcoid granuloma, and we concluded that the cavitary lesions were due to sarcoidosis. After treatment with prednisolone (30 mg/day), the multiple cavitary lesions, diffuse granular shadows and skin lesions showed obvious improvement.     

CT引导下经皮肺穿刺在肺部病变中的应用

目的探讨CT引导下经皮肺穿刺活检在肺部病变中的应用。方法对98例肺部病变患者行CT引导下经皮肺穿刺活检,回顾性分析结果和并发症。结果恶性肿瘤确诊率为91．7％,假阴性率为8．3％,无假阳性率;良性病变确诊率为84．6％,假阴性率为15．4％;总确诊率为89．8％。并发症主要是气胸和出血,气胸的发生率为11．2％;出血的发生率为8．2％;同时有气胸和出血的为3．1％。结论CT引导下经皮肺穿刺活检术对肺部病变的诊断准确性高,并发症低。     

A case of pulmonary sarcoidosis with calcification in the lung

A 35 year-old male farmer presented with complaints of productive cough and sputum. The chest X-ray films showed reticulonodular shadows bilaterally in the upper and middle lung fields, segmental infiltration in the right lung, and no BHL. Tuberculin reaction was negative. Serum angiotensin converting enzyme level was 26.5 IU/ml. Precipitating antibodies for Thermophilic actinomycetes and M.f. were negative. BAL showed moderate lymphocytosis (24.3%), and CD 4/8 was 1.67. A biopsy specimen of right scalene lymph node showed epithelioid cell granulomas and TBLB epithelioid cell granulomas and spot-like calcification. Pulmonary sarcoidosis with calcification in the lung is very rare.     

A case of small cell lung cancer occurring in a patient with pulmonary sarcoidosis]

A 62-year-old man was diagnosed as having sarcoidosis on the basis of uveitis, and the findings of bronchial alveolar lavage and transbronchial lung biopsy, in April 1988. He was admitted to hospital in September 1990, because of left hemiplegia. The chest X-ray film on admission revealed a new mass shadow in the left S6 and some increase of nodular shadows in both lung fields. A bronchial biopsy from the left B6 bronchus revealed small cell lung cancer. Although he was treated with whole brain irradiation and combination chemotherapy, he died of respiratory failure after three months. Speculations about the association between sarcoidosis and lung cancer have been made, but the mechanism is not understood, and their co-existence in the same patient is rare.     

A case of chronic bird fancier's lung in which VATS biopsy was diagnostically useful]

A 72-year-old woman was admitted to our hospital complaining of dry cough and dyspnea on effort for 9 months. Her chest HRCT findings showed diffuse reticular opacities, ground-glass opacities, and interlobular septal thickening with subpleural and peribronchovascular distribution, not typical of IPF/UIP. BAL revealed mild lymphocytosis and elevated CD4/CD8 ratio. Since TBLB was not diagnostic, we performed VATS biopsy. The pathological finding by VATS biopsy revealed collapse, fibrosis, and scar formation in both subpleural and intralobular regions with small granuloma, consistent with chronic hypersensitivity pneumonitis. Both antibodies in serum to pigeon dropping extract and inhalation provocation test using avian dropping extracts were positive. and therefore we diagnosed chronic bird fancier's lung. We should suspect chronic hypersensitivity pneumonitis in cases with HRCT findings atypical of IPF/UIP. and examine antibodies against specific antigens and perform VATS biopsy.     

Clinico-pathological study of collagen-related pulmonary lesions in cases of open lung biopsy]

We studied the clinico-pathological correlation of collagen disease-related pulmonary lesions to examine the pathological and radiological features of collagen lung, and the effect of steroid therapy. Ten open lung biopsy cases were examined; 4 male, and 6 female. The mean age was 55 years old. Seven cases developed pulmonary shadows after the diagnosis of collagen disease, and 3 cases showed pulmonary shadow prior to diagnosis. Pathologically, 6 cases proved to be bronchiolitis obliterans organizing pneumonia (BOOP), 3 cases were chronic interstitial pneumonia (UIP), and 1 case was acute interstitial pneumonia. All cases had inflammatory thickening of the interstitium involving the pleura, bronchial wall, and perivascular connective tissue. Half of the cases had bronchiolar inflammatory lesions. Radiologically BOOP cases showed either localized ground glass shadows, or diffuse reticulonodular shadows predominantly in the lower lung fields with shrinkage of affected areas. UIP cases showed reticulonodular shadows, and active UIP cases showed overlapping ground glass shadows. Steroids were administered in cases of BOOP and active UIP, and all cases showed improvement. We consider that open lung biopsy is of use in the diagnosis of some cases and in assessing whether steroid therapy is indicated.     

A case of sub-acute onset pulmonary sarcoidosis with pulmonary dysfunction]

A 60-year-old man was admitted to our hospital because of shortness of breath and dry cough. Slight pleural thickening was observed on the chest X-ray film and chest CT on presenting. Three months later, bilateral hilar lymphadenopathy and multiple small nodular shadows were shown in both lung fields by chest CT. Serum ACE level and lysozyme levels were higher than normal upper limit. Elevation of CRP and left shift of leukocytes were not detected. Restrictive ventilatory impairment was shown in the respiratory function test. This case was diagnosed as sarcoidosis most probably with histological evidence of epithelioid cell granulomas in the lung tissue obtained by transbronchial lung biopsy. After prednisolone medication (40 mg per day) was started, symptoms, pulmonary function and chest CT findings improved. Our case was considered to be a rare case of pulmonary sarcoidosis with subacute onset of symptoms and restrictive pulmonary function abnormality which improved smoothly as a result of steroid therapy.     

A case of sarcoidosis with primary pulmonary cavitation]

A 30-year-old woman presented with multiple nodular shadows which enclosed a cavity on a chest radiograph. Chest computed tomographic (CT) images showed mediastinal lymphadenopathy, and multiple nodular opacities enclosing a cavity. Histopathological findings of biopsy specimens from the lung and mediastinal lymph nodes revealed noncaseating epithelioid cell granulomas without any evidence of Mycobacterium or fungal growth. The lesion in the lung included granulomatous vasculitis. Even without corticosteroid or any other therapy, the lung lesions resolved and the cavity disappeared. We report a case of sarcoidosis with primary acute cavitation.     

Diagnosis and treatment of pleural lesions in sarcoidosis of the respiratory organs]

Among the 2775 respiratory sarcoidosis patients who were examined over the last ten years 278 (10%) had pleural affections: thickening of interlobular pleura and pleural deposits (98.2%), exudative pleurisy (1.1%) and spontaneous pneumothorax (0.7%). The most common pleural affection was found in sarcoidosis of stages II and III (16.8 and 70.8%, respectively). The clinical manifestations in all forms of this pathology had poor symptoms. Complex therapy proved to be highly effective. It included use of corticosteroids, antioxidants and immunomodulators; massive exudative pleurisy was managed by pleural puncture and removal of exudate.     

A case of sarcoidosis with pleural involvement

Sarcoidosis is a chronic, multisystem inflammatory disorder of unknown etiology that is characterized by noncaseating granulomas. Although lung involvement is common in sarcoidosis, pleural involvement is rare. Pleural involvement may manifest as a pleural effusion, pneumothorax, pleural thickening and nodules, hydropneumothorax, hemothorax, or chylothorax. Here, we describe a case of sarcoidosis with pleural nodular thickening.     

A case of sarcoidosis with diffuse pulmonary arteriovenous malformations]

A 27-year-old woman was admitted because of right dry eye and blurred vision. She was given a diagnosis of uveitis due to sarcoidosis. Chest X-ray film showed bilateral hilar lymphadenopathy and multiple nodules in both lungs. The nodules were considered to be associated with sarcoidosis. However chest CT demonstrated that the nodules were well-defined and connected with two vessels. We diagnosed diffuse pulmonary arteriovenous malformations (AVMs) by 3D-CT. In three of sixteen AVMs, the feeding vessels were more than 3 mm wide in diameter on pulmonary arteriography, and those were embolized by platinum coils. The embolotherapy of diffuse AVMs could not ameliorate the shunt fraction sufficiently, but should reduce the possibility of the catastrophic complication of brain abscess.     

A case of sarcoidosis with primary acute pulmonary cavitation]

A 23-year-old man was admitted to our hospital on June 24, 1991, because of worsening chest X-ray findings of sarcoidosis. In August 1990, he was referred to our outpatient office, because of BHL and nodular lesions on chest X-ray film performed at his company 4 months earlier. At that time, serum ACE was elevated to 34.0 IU/l, and Ga scintigraphy showed abnormal uptake in bilateral lacrimal and salivary glands, mediastinal and hilar lymph nodes, and in the lung fields. TBLB specimen showed noncaseating epithelioid granuloma with giant cells and negative stains for acid-fast bacilli. Although it was planned to follow this patient without medication, he did not return to our outpatient department. In June 1991, because of worsening of lesions in the lung at annual checkup at his company, he was referred and admitted for steroid therapy. Chest X-ray film on admission showed BHL, multiple nodular lesions in both lung fields, and bullous change in the left upper lobe. Chest CT on admission showed three cavitating lesions within preexisting nodules. PPD skin test was negative, and sputum smears and cultures were repeatedly negative for pyogenic bacteria and acid-fast bacilli. Therapy was initiated with prednisolone 30 mg daily. Four months later, there was marked resolution of BHL and nodular lesions, and the cavitating lesions were no longer visible on chest X-ray film. From the clinical and radiological observations, it is concluded that the cavitating lesions in the present case were primary acute pulmonary cavitation in sarcoidosis, distinct from infection, bullae, or cystic bronchiectasis which are seen in the chronic and fibrotic stages of sarcoidosis.     

A patient with pleural thickening of sarcoidosis]

We report a 51-year-old woman with characteristic pleural involvement of sarcoidosis. Video-assisted thoracoscopic examination identified diffuse pleural thickening in the right lung, which coincided in distribution with parenchymal reticular shadows demonstrated with high-resolution thoracic CT scan. Biopsied specimens revealed epithelioid cell granuloma with noncaseating necrosis and multi-nucleated giant cells in the parenchymal lung tissue. Infiltration of inflammatory cells was demonstrated in the thickened pleural tissue, but no typical sarcoid lesion. This pleural lesion was considered as pleural involvement of sarcoidosis, since deterioration of the pleural thickening was accompanied with progression of a parenchymal sarcoid lesion during the period of two months after biopsy. Video-assisted thoracoscopy and high-resolution CT scan both supported the diagnosis of sarcoidosis with pleural involvement.     

A case of sarcoidosis presenting with Heerfordt's syndrome, associated with hepatosplenomegaly, pleural effusion, and ascites]

A 30-year-old female developed symptoms consistent with Heerfordt's syndrome (complete type) and was effectively treated with steroid. However, she developed hepatosplenomegaly, bloody pleural effusion, and ascites one year after discontinuation of steroid therapy. The symptoms were considered to be due to sarcoidosis since serum ACE level, and ACE level and OKT4/8 ratio in pleural and peritoneal effusions were significantly elevated, and readministration of steroid normalized these changes. Bloody pleural and peritoneal effusions are very rare complications of sarcoidosis.     

A case of pulmonary sarcoidosis with B lymphocytosis in peripheral blood]

A 24-year-old man was admitted on October 14, 1988, with bilateral cotton-like shadows on chest X-ray film. Chest CT demonstrated multiple nodular shadows in bilateral lung fields. Laboratory data on admission showed peripheral blood lymphocytosis and slight decrease of serum IgG and IgA. ACE was within normal limits, and BALF findings showed no increase of lymphocytes. Surface marker analysis of peripheral blood lymphocytes showed an increase of B lymphocytes of antibody independent stage (B1, B4, J5, OKB7, sIgM, and sIgD positive cells were increased). Southern blot analysis of peripheral blood showed no rearrangement of immunoglobulin chains (H chain and L chain). In order to differentiate sarcoidosis from lymphoproliferative disorder, open lung biopsy was performed. Pathologic study revealed multiple epithelial granulomas without caseation. Immunohistochemical study demonstrated no accumulation of B cells of the lung. This patient was pathologically diagnosed as having pulmonary sarcoidosis. The abnormal chest shadows gradually improved without therapy; however, B cell lymphocytosis and slight decrease of serum IgG and IgA persisted. In general, lymphocytopenia of peripheral blood is usual in sarcoidosis. We report a rare case of pulmonary sarcoidosis with polyclonal B lymphocytosis of antibody independent stage in peripheral blood.