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.     

An elderly case of sarcoidosis with multiple pulmonary cysts]

We report an elderly case of sarcoidosis with multiple pulmonary cysts. An 80-year-old woman was admitted to our hospital for detailed examinations of multiple cysts in both lungs. Chest radiography and chest CT revealed multiple cystic lesions with thin walls which were clearly separated from normal lung. A transbronchial lung biopsy revealed epitheloid cell granuloma, indicating sarcoidosis. Although elderly cases of sarcoidosis with multiple pulmonary cysts are very rare, sarcoidosis should be considered in the differential diagnosis.     

Treatments for pulmonary sarcoidosis

Corticosteroids (oral or inhaled) are commonly used to treat pulmonary sarcoidosis; however, there is no consensus about when to start treatment, what dose of steroids to give and for how long. Immunosuppressive and cytotoxic agents (used in immunosuppressive doses) are used in addition to oral corticosteroids to treat multisystem and chronic sarcoidosis, or as steroid-sparing agents. We summarize the findings from two Cochrane systematic reviews that have examined the efficacy of corticosteroids and immunosuppressive and cytotoxic drugs in the treatment of pulmonary sarcoidosis. Studies of corticosteroids differed in outcome measures, dose of drug given and length of treatment. For many outcome measures, data could not be pooled for meta-analysis. Oral corticosteroids improved chest X-ray appearance over 3-24 months, with improvement in global score in one study. Little evidence was found of improvement in lung function or of any long-term disease-modifying effect. Follow-up data could not be analysed. Inhaled corticosteroids improved symptoms in one small study but not lung function or chest X-ray. Side-effects of steroids were not well reported. In the immunosuppressive and cytotoxics review, no data could be combined for meta-analysis. Data on lung function, chest X-ray and dyspnoea were largely inconclusive. Methotrexate had a steroid-sparing effect in one small study. Significant adverse events were associated with cyclosporine A, chloroquine and pentoxifylline. Evidence from randomized-controlled trials (RCTs) supporting the use of immunosuppressive and cytotoxic agents is limited.     

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.     

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.     

Two cases of pulmonary eosinophilic granuloma with multiple nodular shadows]

We encountered two rare cases of pulmonary eosinophilic granuloma with multiple nodular shadows in both lungs. The patient in case 1 was a 54-year-old man complaining of dry cough and chest pain. He had smoked 20 cigarettes a day for 36 years. The patient in case 2 was a 37-year-old woman complaining of dry cough. She had smoked 15 cigarettes a day for 20 years. Chest radiography and CT revealed multiple nodular shadows in both lungs. Diagnosis was made by open lung biopsy in case 1 and by percutaneous lung biopsy in case 2. After smoking cessation, symptoms improved markedly and the shadows in the chest radiographs and CT disappeared. In cases of bilateral multiple nodular shadows, other than metastatic lung tumor cases, pulmonary eosinophilic granuloma should be considered.     

Three cases of primary acute pulmonary cavitation in sarcoidosis]

Sarcoidosis has a variety of pulmonary manifestations including the nodular, or infiltrative form. However, primary cavitary formation is rare. In this report, we describe three cases of sarcoidosis with primary acute pulmonary cavitations. All three patients were young adults (2 men and 1 woman) and chest radiography and chest CT showed bilateral Hilary lymphadenopathy, multiple cavitations with thin, smooth walls, and diffuse granular shadows. Primary acute pulmonary cavitation of sarcoidosis was diagnosed, since a transbronchial biopsy specimen revealed sarcoid granuloma, and there was no evidence of infection, emphysematous change, or fibrotic or cystic bronchiectatic changes on chest radiography in any case. Corticosteroid therapy was given in all cases for severe extrathoracic disease (2 of severe uveitis and 1 of CNS involvement). After the treatment, the multiple cavities and diffuse granular shadows in the lung fields disappeared. We accordingly suggest that corticosteroid therapy is effective for primary acute cavitation in sarcoidosis.     

大容量全肺灌洗治疗重度肺泡蛋白沉积症五例分析

目的　评价大容量全肺灌洗治疗重度肺泡蛋白沉积症的临床疗效及安全性。方法采用全身麻醉下双腔气管插管 ,大容量分次肺泡灌洗 ,负压回收灌洗液并记录出入量 ,计算回收率。以患者症状的改善及胸片或胸部CT及肺功能、动脉血气的改变评价疗效。结果　 5例患者的症状均得到明显改善 ,胸片显示大部分阴影消失 ,肺功能及动脉血气有明显好转。 5例患者无一例出现严重并发症。结论　大容量全肺灌洗治疗重度肺泡蛋白沉积症疗效高、安全性好     

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.     

Inhaled corticosteroid rapidly improved pulmonary sarcoidosis

We report two cases in whom inhaled corticosteroid rapidly improved pulmonary sarcoidosis. In the first case, fluticasone at 400 microg/day was initiated, because dry cough and small nodular shadows on chest X-ray persisted for six months. But her cough and the nodular shadows were persisted, therefore the treatment was replaced with budesonide at 800 microg/day. Two months later, her dry cough subsided and pulmonary shadows improved. Serum angiotensin-converting enzyme (ACE) level was decreased and pulmonary function improved. In the second case, bumethasone was already administered at a local clinic. Budesonide at 400 microg/day was combined with oral steroid, because pulmonary shadows continued for eight years. Also two months later, the serum ACE level was decreased and the pulmonary shadows slightly improved. Inhaled corticosteroid therapy for two to three months is tolerable, and may be a useful treatment option in some patients with sarcoidosis.     

Pulmonary sarcoidosis and antiphospholipid syndrome

Various autoimmune diseases have been reported to occur in patients with sarcoidosis. However, coexistence of sarcoidosis and antiphospholipid syndrome (APS) is extremely rare. We describe a 59-year-old female patient with pulmonary sarcoidosis who had preceding APS. Her previous medical history consisted of a miscarriage and ischemic colitis. She was diagnosed as APS during the onset of a brainstem infarction with positive reaction to beta2-glycoprotein I-dependent anticardiolipin antibody. Two years later, chest CT revealed enlargement of the hilar and mediastinal lymph nodes and small nodules in the lung fields. Transbronchial lung biopsy demonstrated non-caseating epithelioid cell granuloma leading to the diagnosis of definite pulmonary sarcoidosis. This is the first APS case where pulmonary involvement with sarcoidosis has been confirmed through lung biopsy. Our case report suggests that APS should be recognized as an accompanying disorder of sarcoidosis.     

Two cases of Mycobacterium szulgai pulmonary disease in the elderly

The rate of pulmonary nontuberculous mycobacteriosis (NTM) in the total pulmonary mycobacteriosis has been continuously increasing. While M. avium complex is the most common cause of NTM, there are a few case reports of pulmonary infection due to M. szulgai. We described two cases of pulmonary NTM caused by M. szulgai. A 75-year-old male was admitted to our hospital because of dyspnea on effort, and productive cough. A chest X-ray showed an infiltrative shadow with cavity in the right upper lobe. A sputum smear for mycobacteria was positive, and a culture grew M. szulgai which was identified by DNA-DNA hybridization. He was treated with isoniazid, rifampicin, and ethambutol. His symptoms and CT and X-ray findings improved, and his sputum smear and culture converted to negative for mycobacteria. Second case was a 73-year-old male who had previously been diagnosed as MAC and pulmonary aspergillosis, and had been treated with antituberculous and antifungal drugs. He was readmitted to our hospital, because of general fatigue and hemoptysis. A chest X-ray revealed a consolidation with bronchiectasis and cavity in the both upper lung fields. A sptum smear for mycobacteria was positive, and a grown culture was identified as M. szulgai. He was treated with rifampicin, ethambutol and kanamycin based on the results of susceptibility testing. After 3 months of this treatment his sputum smear and culture converted to negative for mycobacteria, and his symptoms, and CT and X-ray findings improved.     

Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis

OBJECTIVE: To investigate the frequency of pulmonary hypertension (PH) and clinical parameters associated with PH in sarcoidosis patients. METHODS: A prospective, observational study was performed on 246 consecutive Japanese sarcoidosis patients followed up at the outpatient sarcoidosis clinic in the Central Clinic of Kyoto. The patients were evaluated for PH by Doppler echocardiography. Among these patients, 192 underwent pulmonary function tests. In addition, high-resolution CT of the lung was evaluated for the presence of lymph node enlargement, lung opacity, and thickening of bronchovascular bundles in 122 patients. PH was defined as estimated systolic pulmonary artery pressure (sPAP) > or = 40 mm Hg. The frequency of PH was evaluated, and clinical parameters were compared between patients with PH and those without PH. RESULTS: Among 212 patients who were successfully evaluated for sPAP, 12 patients (5.7%) had PH. Patients with PH had the following clinical characteristics: advanced chest radiographic stage, decreased oxygen saturation, predominantly male gender, and decreased percentage of predicted vital capacity, percentage of predicted FVC, percentage of predicted FEV1, percentage of predicted functional residual capacity, and percentage of predicted total lung capacity (%TLC). Multivariate logistic regression analysis showed that decreased %TLC was independently associated with PH. There was a weak negative correlation between sPAP and %TLC (p < 0.05). CONCLUSIONS: The frequency of PH in Japanese sarcoidosis patients was 5.7% evaluated with Doppler echocardiography. Decreased lung volume increases the risk of PH developing in patients with sarcoidosis.     

Effects of corticosteroid treatment on pulmonary haemodynamics in patients with sarcoidosis

Effects of 12 months steroid treatment on pulmonary haemodynamics in 24 patients with pulmonary sarcoidosis, stage II and III, were investigated. All patients had a chest radiograph, lung function tests, and pulmonary haemodynamics (measured at rest and during submaximal exercise) prior to the treatment. Resting pulmonary hypertension was found in 3 patients. In 18 an abnormal increase in pulmonary arterial mean pressure (PAP) on exercise was observed. All investigations were repeated after a year of treatment. In almost all patients (22) a regression of radiological changes in the lungs and improvement of pulmonary function were observed. The changes in pulmonary circulation were less uniform. In only half of the patients studied was regression of the disease seen on chest X-ray and improvement in lung function accompanied by improvement in pulmonary haemodynamics.     

Case of pulmonary Mycobacterium mageritense infection: the difficulty of differential diagnosis of granulomatous lung diseases

A 36 year-old female was pointed out of pulmonary abnormal shadows in the annual chest survey. Chest radiograph and computed tomography (CT) disclosed bilateral diffuse infiltrative shadows and tree-in-bud appearance in the right upper lung field and the left lingula. A sputum smear for acid-fast bacilli was negative. Histopathologically, the transbronchial lung biopsy specimen revealed non-caseous epithelioid granulomas with numerous giant cells. Acid-fast bacilli were cultured from her sputum, however, nontuberculous mycobacteria was not detected by DNA-DNA hybridization method. Mycobacterium mageritense was identified by 16S ribosomal RNA sequencing with 100% matching. The isolated colony of M. mageritense was resistant to nine anti-tuberculous drugs. Follow-up chest CT scan showed a gradual decrease of infiltrative shadows without therapy. To the best of our knowledge, M. mageritense infections are rare, and this is the first case report of pulmonary infection in the literature. We conclude that the pulmonary infection of M. mageritense is one of causes of granuloma formation, and in some case it is difficult to differentiate clinically from sarcoidosis.     

A case of chronic pulmonary paracoccidioidomycosis]

In a 43-year-old Japanese Brazilian who came to Japan in 2001, since subjective symptoms such as cough, sputum, and dyspnea on exertion had become severe, he was referred to our hospital because of suspicion of pulmonary tuberculosis in chest radiography and CT findings. A chest radiograph of initial examination showed interstitial shadows in both lungs with nodular, infiltrative or cavitary changes. No Mycobacterium tuberculosis was found. The mycetocyte with multipolar budding resembling the steerage of a ship, which was characteristic of Paracoccidioides was observed in sputum and transbronchial lung biopsy specimens. We cultured a fungus to show dimorphism of temperature dependency, and a diagnosis of chronic lung paracoccidioidomycosis was arrived at. By administration of ITCZ 200 mg/day, the chest radiography findings and clinical manifestations were improved. This case seems to be worthy of reporting in Japan since the affected site or organ was limited to the two lungs with multiple cavitary lesions and fibrotic changes on radiographic examination, and final diagnosis was made by cytology of sputum and pathology of TBLB specimens.     

Clinical significance of gallium-67 scintigraphy in assessing pulmonary lesions of sarcoidosis and idiopathic pulmonary fibrosis]

To evaluate whether one can predict the course and prognosis of interstitial lung diseases from lung gallium-67 (67Ga) uptake, we studied 31 subjects with sarcoidosis and 28 with idiopathic pulmonary fibrosis (IPF) retrospectively. We quantified the lung 67Ga uptake using posterior scans by Line's method, and calculated a visual index (VI). The normal range of 67Ga uptake was defined as less than 65 VI values, obtained from the 95 percent confidence interval of the control subjects. All subjects with stage I sarcoidosis, having only bilateral hilar lymphadenopathy (BHL) on chest X-ray, revealed normal lung 67Ga uptake. Fifty percent of patients with stage II sarcoidosis, with both BHL and pulmonary involvement on chest X-ray, showed increased lung 67Ga uptake. The patients with increased lung 67Ga uptake showed decreased percent vital capacity and increased alveolar-arterial oxygen tension difference, but none of them showed clinical deterioration during the follow-up period of more than 6 months. Fifty-four percent of subjects with IPF showed increased lung 67Ga uptake. But there was no difference between the subgroups with normal and increased lung 67Ga uptake with respect to the severity of dyspnea, percent vital capacity, arterial oxygen tension, or alveolar-arterial oxygen tension difference. There was also no difference between the two subgroups of IPF in the cumulative percent survival after either the onset of symptoms or the 67Ga scintigram. We conclude that lung 67Ga uptake was not able to predict the clinical course or the prognosis of patients with sarcoidosis and IPF.     

肺结节病7例临床分析

目的提高对肺结节病的认识,探讨肺结节病的临床特点及诊断和治疗方法。方法分析7例肺结节病患者的临床资料。结果 (1)40岁以下患者多见,女性多于男性;(2)症状多样且无特异性。(3)胸部X线及CT检查是早期诊断的重要手段,SACE及PPD试验对诊断有帮助;(4)确诊依赖组织病理学检查。结论肺结节病临床表现多样,糖皮质激素治疗有效。关键是提高肺结节病的诊断意识。     

结节病与肺结核的临床病理分析

目的探讨病理难以鉴别的结节病与肺结核两组患者之间的临床综合表现的差异性,以提高临床对结节病与肺结核的鉴别能力。方法回顾性分析2009~2013年广州医科大学附属第一医院病理难以鉴别的结节病与肺结核的50例患者的临床特点。归纳分析结节病和肺结核两者不同的临床表现、影像学特点及检验相关值的变化。结果结节病组22例,肺结核组28例,结节病与肺结核两组临床症状对比,咳嗽、气促症状的差异,有统计学意义(P0.05),对比胸部CT检查纵隔淋巴结肿大特别是双肺门淋巴结肿大,PPD皮试和γ-干扰素释放的T细胞斑点试验(T-SPOT.TB)阳性率的差异有统计学意义(P0.05)。结论病理难以鉴别的结节病与肺结核,通过临床症状、胸部CT影像及T-SPOT.TB检查进行分析可以提高两者的鉴别率。     

A case of spontaneous resolution of sarcoidosis with primary pulmonary cavitations]

On routine physical checkup, a 27-year-old man with productive cough was found to have multiple nodules with cavitation in the bilateral lung fields and mediastinal and hilar lymph adenopathy on chest X-ray film and CT scan. Serum levels of angiotensin converting enzyme and lysozyme were high. Tuberculin reaction was negative. Non-caseous epitheloid granulomas were confirmed in the bronchial wall specimens obtained by trans-bronchial biopsy. The number of lymphocytes and the CD4/CD8 ratio of lymphocytes in bronchoalveolar lavage fluid was increased. Therefore, pulmonary sarcoidosis was diagnosed, and the lung nodules with cavitation were considered due to sarcoidosis. The walls of the cavitations gradually thinned and had almost completely vanished after 6 months of careful observation without steroid therapy.