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1.
Although sarcoidosis is generally considered a disease of young and middle aged adults, there have been a certain number of cases among elderly. However it is unknown whether sarcoidosis in the elderly is recurrence of prior disease or initial onset at old ages. We present a 77-year-old woman with sarcoidosis the onset of which was considered to be in the last 6 months prior to the initial diagnosis. The patient was admitted to our hospital for further evaluation of bilateral hilar and mediastinal lymphadenopathy (BHL) and uveitis. BHL was not present in a chest radiograph taken 6 months prior to the admission. A clinical diagnosis of sarcoidosis was made by elevated serum angiotensin converting enzyme (ACE) and lysozyme (24.9 IU/L and 18.2 micrograms/ml, respectively), negative tuberculin skin test, concomitant presence of uveitis, and a high proportion of lymphocytes (33.2%) in bronchoalveolar lavage fluid with an elevated CD4/CD8 ratio (24.5). This is a noteworthy case of sarcoidosis in which we could confirm elderly onset of the disease.  相似文献   

2.
Ocular sarcoidosis is frequent in Japan, but in many cases the condition remains undiagnosed in patients with suspected ocular sarcoidosis. Bronchoalveolar lavage (BAL) was performed in order to study the clinical implications of lymphocytosis of BAL fluid in such patients with characteristic ocular manifestations. The subjects included in this study were 39 patients with suspected ocular sarcoidosis. The patients were divided into four types based on high-resolution computed tomography (HRCT) findings; no lung involvement (HRCT-0), bilateral hilar lymphadenopathy (BHL) without lung involvement (HRCT-I), lung involvement and BHL (HRCT-II), and lung involvement and no BHL (HRCT-III). Transbronchial lung biopsy (TBLB) and BAL were conducted after examining serum angiotensin-converting enzyme and serum lysozyme values, skin test for purified protein derivative chest radiograph, HRCT, and gallium scintigram. Twenty patients were histologically diagnosed as having sarcoidosis, and 19 patients remained undiagnosed. Granuloma was identified by TBLB in 19 of 20 patients in type HRCT-II but in only one of 19 patients in types HRCT-0 and HRCT-I (p<0.0001). Lymphocytosis in BAL (>15%) was identified in all patients who showed lung field involvement (type HRCT-II) and in 16 of 19 patients without lung field involvement (types HRCT-0 and HRCT-I). There were 10 patients whose only relevant findings were lymphocytosis in BAL. Among these 10 patients, an increased CD4+/CD8+ ratio (>3.5) in BAL was seen in 60%. The authors conclude that high-resolution computed tomography results yield the same degree of diagnostic accuracy as transbronchial lung biopsy in ocular sarcoidosis suspects. However, bronchoalveolar lavage revealed significant lymphocytosis in patients with negative high-resolution computed tomography results. It should be kept in mind that a diagnostic group of patients with sarcoidosis who manifest ocular involvement and lymphocytosis in bronchoalveolar lavage exists.  相似文献   

3.
Subcutaneous sarcoidosis is the specific subset of cutaneous sarcoidosis frequently associated with systemic disease. However, the disease activity, severity, and prognosis have not yet been elucidated due to the limited number of reported cases. The purpose of this study was to identify the clinical, laboratory, and prognostic differences between subcutaneous sarcoidosis and other type of cutaneous sarcoidosis. All patients with sarcoidosis diagnosed histopathologically from 2000 to 2012 at our institution were enrolled. The clinical, laboratory, chest X-ray, and pulmonary function test results were retrospectively evaluated in the patients with cutaneous sarcoidosis. In the 130 patients with sarcoidosis, cutaneous sarcoidosis was diagnosed in 37 patients (28.4 %), and 9 (6.9 %) of these patients had subcutaneous sarcoidosis. The serum levels of soluble interleukin-2 receptor (sIL-2R) were significantly elevated in the group of patients with subcutaneous sarcoidosis in comparison to the patients with other types of cutaneous sarcoidosis, whereas there was no significant difference in the severity score between the two groups. Following a 2-year observation period, three patients were in remission, five patients demonstrated stable disease, and one patient had progressive disease. Subcutaneous sarcoidosis may be associated with the disease activity, although it was not found to be associated with the disease severity and it was not a predictive factor for the prognosis. Furthermore, the prevalence of subcutaneous sarcoidosis may be higher than that in previously reported series.  相似文献   

4.
A 27-year-old female was admitted to our hospital after a regular medical check revealed BHL and cavitation in the right upper lung field on a chest radiograph. Ga scintigraphy showed abnormal uptake bilaterally in the mediastinal and hilar lymph nodes. We strongly suspected lung sarcoidosis, then performed TBLB and BAL. BAL fluid disclosed a high proportion of lymphocytes with a marked elevation of the CD4/CD8 ratio, compatible with sarcoidosis. A TBLB specimen revealed non-caseating epithelioid cell granuloma compatible with a diagnosis of lung sarcoidosis. From the clinical and radiological observations, it was concluded that the cavitation in the present case was primary pulmonary cavitation in sarcoidosis, as distinct from infection, malignancy, bulla or cystic bronchiectasis. Chest radiographs taken a half year after diagnosis showed reduction of the cavitary lesion and disappearance of BHL.  相似文献   

5.
A 45-year-old woman had bleary eyes and recurrent episodes of fever and arthritis in the knees and ankles. The patient had anterior uveitis, negative findings of the tuberculin test, and an increased serum lysozyme level, but bilateral hilar lymphadenopathy (BHL) was absent. During the course of her disease, the serum calcium and angiotensin-converting enzyme levels gradually increased to above the normal level, and the patient was clinically diagnosed as having sarcoidosis. The clinical features of arthritis were typical of those of L?fgrens syndrome although BHL and erythema nodosum were absent. The patient was successfully treated with 15 mg/day of prednisolone.  相似文献   

6.
目的探讨结节病的治疗、影响预后的因素及判断病情和预后的指标。方法回顾性分析1984年1月-2003年1月北京协和医院住院治疗且随访1〉2年的59例结节病临床资料。结果随访时间(61.9±49.4)个月。(1)0期患者7例,均在确诊后即口服糖皮质激素(以下简称激素)治疗,其中4例完全缓解。Ⅰ期患者22例,确诊后未予治疗的5例患者中1例进展,3例自愈,1例部分缓解;确诊后即予激素治疗的17例患者中,9例完全缓解,1例进展至Ⅱ期。Ⅱ期患者23例,1例在未予治疗3个月加重后及22例在确诊后开始激素治疗,13例完全缓解,2例进展至Ⅲ期。Ⅲ期5例患者均在确诊后即予激素治疗,1例治愈,2例进展至Ⅳ期。Ⅳ期患者2例,尽管长期激素治疗,病情仍在缓慢进展。(2)Ⅰ期、Ⅱ期患者发病时的支气管肺泡灌洗液(BALF)中细胞总数和分类、CD4/CD8比值及血清血管紧张素转换酶(sACE)水平差异无统计学意义。(3)共有24例患者复发,其中胸内结节病20例,14例有肺外受累;未复发者中10例有肺外受累;有肺外受累与无肺外受累比较,P=0.006。(4)完全缓解者发病时BALF中中性粒细胞数低于尚未缓解者(P〈0.001)。结论结节病病程有一定的自愈性。对Ⅰ期患者可先观察。有肺外病变的胸内结节病患者更易复发。发病时BALF中中性粒细胞数可能作为一个预测预后的指标。  相似文献   

7.
Sixty-three patients with clinically definite sarcoidosis confirmed histologically and/or by a positive Kveim test were studied according to the clinical and radiographic extent and known duration of their disease and symptoms. Immunoglobulin levels were not raised among patients with bilateral hilar lymphadenopathy (BHL) alone, but IgG and IgA were raised among patients with pulmonary sarcoidosis, most markedly among patients with long-standing active disease. Serum IgM was found elevated in extrathoracic sarcoidosis. West Indian males had the highest levels of IgG. The presence of circulating immune complexes reacting with conglutinin was strongly associated with a recent symptomatic onset of sarcoidosis; they were more prevalent in patients with BHL and erythema nodosum. In contrast, polyethylene glycol precipitable immunoglobulins were associated with long-standing active disease and with neurologic involvement.  相似文献   

8.
Mast cell tryptase plays an important role in fibrosis. Tryptase levels in bronchial alveolar lavage fluid (BALF) from patients with interstitial lung diseases are frequently increased, but little is known of the clinical significance. The study population consisted of 93 patients [38 with sarcoidosis, 23 with collagen vascular disease (CVD), and 32 with idiopathic pulmonary fibrosis (IPF)]. BALF tryptase levels were measured with a newly developed B12 antibody-fluoroimmunocap method (UniCAP method), which can detect an activated form of tryptase. We examined the relationship between BALF tryptase levels and clinical parameters of the diseases. BALF tryptase was detected in 7 (18.4%) patients with sarcoidosis, 7 (30.4%) with CVD, and 14 (45.8%) with IPF. In tryptase-positive group, serum ACE levels and the numbers of BALF-mast cells and lymphocytes were higher than the tryptase-negative group in sarcoidosis, serum LDH levels were higher in CVD, and the number of BALF-lymphocyte and Hugh-Jones grade were higher in IPF. Furthermore, tryptase-positive IPF cases had a poorer outcome than the tryptase-negative group by Kaplan-Meier analysis. Tryptase in BALF detected with the UniCAP method may be associated with disease activity in sarcoidosis and CVD, and with severity and poor prognosis in IPF. BALF tryptase measurement may be useful in the assessment of disease activity and severity in various interstitial lung diseases.  相似文献   

9.
K Kutty  B Varkey 《Chest》1984,85(4):533-536
Case summaries of four patients with bilateral hilar lymphadenopathy (BHL) caused by metastatic renal cell carcinoma are presented, and these and eight similar cases from the literature are analyzed. In nine patients, sarcoidosis was the provisional clinical diagnosis, but four of these patients had a past history of renal cell carcinoma. In the remaining five patients, a distinction from sarcoidosis could not be made by history, physical examination, and chest roentgenogram. This underscores the need for tissue confirmation in the diagnosis of sarcoidosis and alerts the physician to consider metastatic renal cell carcinoma in the differential diagnosis of BHL.  相似文献   

10.
The mean values of serum angiotensin-converting enzyme (ACE) activities and lysozyme (LZM) concentrations measured during different phases of sarcoidosis coincided well with the clinical evaluation of the state of the disease. However, both enzymes, especially LZM, decreased before improvement was detected. Changes in these enzymes were in accord with the simultaneous clinical development in three fourths of cases. Incompatibility between clinical observations apnd LZM fluctuations was most frequently seen during active stable or inactive disease. LZM often decreased during the active stable phase and fluctuated irregularly during inactive disease. During the former phase LZM decrements possibly reflect decreasing activity of granulomatous macrophages and, in fact, precede detectable improvement. During inactive disease, on the other hand, cells were not connected with the disease process dominate LZM production. ACE changes paralleled the clinical development more often than corresponding LZM changes during stable sarcoidosis. This may have been misleading and due to a delayed reaction of serum ACE, compared with LZM, inreflecting the activity of granylomatous cells. This delayed reaction was also observed in connection with erythema nodosum. Stable ACE activity during inactive sarcoidosis indicated the usefulness of measurements when trying to predict a relapse. We conclude that ACE may be a secondary feature of sarcoidosis rather than a primary funtion of macrophage activity.  相似文献   

11.
Sarcoidosis is a granulomatous disease of unknown etiology that involves the lungs or intrathoracic lymph nodes in more than 90% of patients. The clinical spectrum of sarcoidosis is protean, but pulmonary manifestations often dominate. Chest radiographs are abnormal in 90 to 95% of patients with sarcoidosis; the most characteristic feature is bilateral hilar lymphadenopathy (BHL), present in 50 to 80% of patients. Pulmonary parenchymal infiltrates are present in 25 to 50% of patients. In this article, we review the radiographic features of sarcoidosis (both typical and atypical), and the impact of chest radiographic stage on long-term prognosis. Computed tomographic (CT) scans are more sensitive than chest radiographs in delineating parenchymal, mediastinal, and hilar structures, and distinctive CT patterns may be virtually pathognomonic for sarcoidosis in some patients. Routine CT scan is not appropriate to diagnose or manage sarcoidosis, but CT may be invaluable in patients with atypical clinical or chest radiographic findings or specific complications of sarcoidosis (pulmonary or extrapulmonary), or to assess prognosis. High-resolution thin-section CT scans (HRCT) may be helpful in selected patients with stage II or III sarcoidosis to discriminate active inflammation from irreversible fibrosis. This article discusses the salient HRCT features of sarcoidosis, accuracy of CT in the differential diagnosis, and correlations of HRCT with disease extent and activity, pulmonary function, and lesion reversibility.  相似文献   

12.
Levels of soluble IL-2 receptor in sera of 18 patients with sarcoidosis were measured by a sandwich ELISA method established by the authors and were found to be significantly higher than those in sera of normal subjects. Levels of soluble IL-2 receptor in sera of sarcoidosis cases of bilateral hilar lymphadenopathy (BHL) were significantly higher than those in sera of sarcoidosis cases without BHL. In patients with sarcoidosis, levels of soluble IL-2 receptor did not differ in relation to the presence or absence of ocular lesions, or in relation to positive or negative response to protein purified derivative of tuberculosis (PPD).  相似文献   

13.
MOTIVATION: It has been debated whether patients need to have erythema nodosum to be classified as having L?fgren's syndrome. In this study, we have therefore in detail evaluated and compared a large number of patients with an acute onset of sarcoidosis and bilateral hilar lymphadenopathy (BHL), with or without erythema nodosum (EN). This study is important because it may lead to a more accurate definition of L?fgren's syndrome, and an exact phenotype of patients is crucial in modern medical research. BACKGROUND: L?fgren's syndrome is commonly regarded as a distinct clinical entity. METHODS: We have in detail evaluated a large group of patients (n = 150) with an acute onset of sarcoidosis with BHL, in most cases with fever, EN, and/or bilateral ankle arthritis or periarticular inflammation. Within this group, 87 patients had EN (EN positive), whereas 63 were without EN (EN negative), though with distinct symmetric ankle inflammation. RESULTS: EN-positive and EN-negative patients were identical in every aspect except that there were significantly more women in the EN-positive group: 58 women (67%) in the EN-positive group compared with only 17 (27%) women in the EN-negative group (p < 0.0001). In all other aspects, such as age, smoking habits, seasonal clustering of disease onset, rate of positive biopsies, chest radiography, pulmonary function, bronchoalveolar lavage cell distributions including the typically increased CD4/CD8 ratio, and clinical development of the disease, the EN-positive and EN-negative groups were close to identical. The two groups were also identically strongly associated with HLA-DRB1*0301/DQB1*0201, with 60 (69.0%) and 44 (69.8%) patients having this particular HLA type in the EN-positive and EN-negative groups, respectively. Such patients recovered to the same degree-that is, at almost 100%. CONCLUSIONS: We conclude that manifestations of L?fgren's syndrome differ between men and women, with EN found predominantly in women, whereas a marked periarticular inflammation of the ankles or ankle arthritis without EN is seen preferentially in men.  相似文献   

14.
Scandinavian human leukocyte antigen-DR17-positive (DR17+) sarcoidosis patients are characterised by a good prognosis. They also reveal an accumulation in bronchoalveolar lavage fluid of T-lymphocytes expressing the T-cell receptor V gene segment AV2S3 at disease onset. The authors of this study wished to establish whether AV2S3 T-lymphocyte accumulation changes from disease onset to clinically resolved disease and how this relates to other activity parameters. Bronchoalveolar lavage fluid and serum from nine DR17+ sarcoidosis patients were examined at disease onset and after spontaneous resolution of clinical and radiographical signs of disease. Nine DR17+ patients with lung accumulated CD4+ AV2S3+ T-cells were investigated after clinically recovery. At re-examination the percentage of CD4+ AV2S3+ lymphocytes in bronchoalveolar lavage fluid was normalised (29 versus 5.4%). A significant reduction in lymphocyte percentage (14 versus 4.4%) and a decrease in cellular concentration (179x10(6) x L(-1) versus 111x10(6) x L(-1)) and CD4/CD8 ratio (5.2 versus 2.4) were also seen. In serum, the activity of angiotensin-converting enzyme (24.9 versus 14.0 U x mL(-1)) as well as the levels of neopterin (7.8 versus 5.3 nmol x L(-1)) decreased significantly after recovery. These results indicate that the locally accumulated AV2S3 positive T-lymphocytes in bronchoalveolar lavage are involved in the pathogenic process of sarcoidosis in this patient group.  相似文献   

15.
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.  相似文献   

16.
OBJECTIVES: To date, insufficient evidence is available to recommend serum soluble interleukin-2 receptor (sIL-2R) measurement as a routine test in the assessment of sarcoidosis. Therefore, we evaluated the clinical value of this test. DESIGN: Forty-seven patients with sarcoidosis, all presenting with active disease, were included in the study. Initial serum sIL-2R levels were determined by enzyme-linked immunosorbent assay, and clinical data at presentation and follow-up were collected retrospectively. RESULTS: The median follow-up period of all patients was 44 months (range, 6 to 100 months), and 38 patients had follow-up data present over at least 24 months. The median sIL-2R level was 1,068 U/mL (range, 248 to 4,410 U/mL; upper limit of normal, 710 U/mL). A positive correlation was found between serum sIL-2R levels and the number of CD4+ T lymphocytes in BAL (rs = 0.53, p < 0.001). In accordance with this result, both sIL-2R level and the number of CD4+ T lymphocytes were elevated in stage I compared to stage III disease (p < 0.05). Patients with extrapulmonary disease (ED) [excluding L?fgren's syndrome] showed higher sIL-2R levels than those presenting with only pulmonary sarcoidosis (p = 0.001). No relation was found between sIL-2R level and response to treatment, and there was no association between sIL-2R levels and radiographic evolution and lung function outcome. CONCLUSIONS: Our data suggest a role for serum sIL-2R as marker of pulmonary disease activity and ED in patients with sarcoidosis.  相似文献   

17.
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.  相似文献   

18.
We investigated whether the level of serum KL-6 could be an activity marker for pulmonary sarcoidosis. In 33 patients with pulmonary sarcoidosis, the relationships between serum KL-6 levels and diagnostic imaging, serum angiotensin-converting enzyme (ACE) levels, serum lysozyme levels, steroid therapy, and prognosis were evaluated. There were no significant differences in the level of serum KL-6 when the patients were divided on the basis of radiographic findings, but the level of serum KL-6 was markedly elevated in some patients with stage-II pulmonary sarcoidosis. There was a significant correlation between serum KL-6 levels and the following two parameters: serum ACE and lysozyme levels. Among patients with a high initial level of serum KL-6, pulmonary sarcoidosis tended to become exacerbated within one year. Steroid therapy significantly decreased the level of serum KL-6, suggesting that the level of serum KL-6 could be an activity indicator for pulmonary sarcoidosis. Immunohistochemical staining by anti-KL-6 antibody revealed that KL-6 was localized in proliferating type-II alveolar epithelial cells.  相似文献   

19.
H Yotsumoto 《Chest》1982,82(5):556-559
Serial measurements of serum angiotensin-converting enzyme activity were made to estimate its usefulness in following the course of 17 patients with sarcoidosis. In nine cases with spontaneous remissions, the enzyme levels decreased gradually, accompanied by improvements in chest radiologic findings. In eight patients given corticosteroids, the enzyme levels decreased rapidly preceding improvements in the chest roentgenograms. The levels returned to pretreatment values when there was radiologic relapse of disease. Reelevation of the enzyme level was also observed without determination of the chest radiologic findings in four of five patients who responded to therapy, but the elevated enzyme level remained lower than the pretreatment level. These observations suggest that the serum angiotensin-converting enzyme level reflects the activity of disease in untreated and corticosteroid-treated patients with sarcoidosis. However, a partial reelevation of the decreased enzyme activity in corticosteroid-treated patients does not necessarily indicate a relapse of the disease.  相似文献   

20.
INTRODUCTION: The release of tumor necrosis factor (TNF-alpha) is increased in sarcoidosis patients. TNF-alpha exerts its effect by binding to specific cell surface receptors. There are only fragmentary data concerning the expression of tumor necrosis factor receptors (TNFRs) on bronchoalveolar lavage fluid (BALF) and peripheral blood (PB) lymphocytes. The aim of the study was to evaluate TNFRI (CD120a) and TNFRII (CD120b) expression on T cells and the level of soluble TNFRs in specimens of patients with different clinical manifestation and clinical outcome of sarcoidosis. MATERIAL AND METHODS: We examined 49 patients with newly diagnosed pulmonary sarcoidosis. TNFRI and TNFRII density on CD4+ and CD8+ BALF and PB cells surface was estimated using monoclonal antibodies and a flow cytometry technique. The level of TNFRs in PB serum and BALF cell culture supernatant (CCS) was measured using ELISA. Immunological analyses were also performed on PB samples collected from 10 healthy volunteers. RESULTS: The level of soluble TNFRI (sTNFRI) in PB serum was similar in sarcoidosis patients and healthy subjects, whereas the concentration of sTNFRII in serum was significantly higher in the sarcoidosis group (P<0.001). Patients without acute symptoms of sarcoidosis, patients with radiological stage II/III as well as patients with further disease progression showed a tendency to higher levels of sTNFRs in PB serum and lower levels of sTNFRs in BALF CCS compared to L?fgren syndrome and radiological stage I subjects, and patients with spontaneous resolution of sarcoidosis. More than 80% of BALF and PB lymphocytes of sarcoidosis patients expressed both CD120a and CD120b antigens. The percentage of double-positive CD4+CD120a+ and CD4+CD120b+ cells in PB was significantly higher (P<0.005) in sarcoidosis patients than in healthy subjects. The highest percentage of CD4+CD120a+ and CD4+CD120b+ lymphocytes in BALF was determined in patients with acute disease, and in PB of patients with further spontaneous improvement. CONCLUSION: The evaluation of sTNFRs and TNFRs expression on T-helper cells may be useful in the estimation of sarcoidosis activity.  相似文献   

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