Sarcoidosis最新文献

Chest X ray showing bilateral hilar adenopathies of the mediastinum associated with erythema nodosum suggests the diagnosis of Löfgren syndrome rather than the presence of a lymphoproliferative disorder. However, the occasional finding of high serum levels of copper can induce diagnostic doubts since serum copper increase is an index of lymphoproliferative disorders, particularly of Hodgkin's disease. We observed four patients with Löfgren syndrome presenting with fever, arthralgies, bilateral hilar adenopathies of the mediastinum and erythema nodosum. All patients underwent whole staging for sarcoidosis and serum copper levels were measured. In all cases clinical and instrumental data allowed the diagnosis of sarcoidosis and in all the patients we found high levels of serum copper with an average of 34.8 mumol/L (30.7-39.4) at the onset of the disease. Three months later, the sarcoid process spontaneously remitted and the serum copper levels returned to normal range. Although the finding of an increase of serum copper in patients with mediastinal adenopathies is usually indicative of a lymphoproliferative disorder (Hodgkin's disease), our data suggest that its increase can be related also to non neoplastic adenopathies of the mediastinum, including sarcoidosis.

Serum concentrations of IgG, IgA, and IgM antibodies to three heat shock proteins (HSPs)--ubiquitin, HSP70, and HSP90--were measured using ELISA in 37 patients with pulmonary sarcoidosis. When compared to healthy controls (n = 44), increased concentrations of IgA and IgG antibodies to ubiquitin were found in 13 (35.1%, p < 0.002) and 7 (18.9%, p < 0.05) patients, respectively. In 10 patients (27.0%) elevated concentrations of IgG antibodies to HSP70 were detected (p < 0.02), whereas IgA antibodies to this protein were found in 7 cases (18.9%, p < 0.05). IgM antibodies to ubiquitin and HSP70, and antibodies to HSP90 were not detected in patients' sera. The levels of antibodies to ubiquitin and HSP70 correlated well with each other within the given immunoglobulin class (r = 0.7391, p < 1E-5 and r = 0.9854, p < 1E-5 for IgG and IgA class, respectively). There was also a weak correlation between the level of IgG antibodies to HSP70 and both serum activity of angiotensin converting enzyme (SACE; r = 0.4668, p < 0.005) and serum level of soluble receptor for interleukin 2 (sIL-2-R; r = 0.4142), p < 0.02). A similar tendency was seen with IgG antibodies to ubiquitin. Furthermore, there was an association between the increased concentration of C-reactive protein (CRP) and increased levels of IgG antibodies to ubiquitin and HSP70 (p < 0.005 and p < 0.05, respectively). Our results suggest that antibodies to various HSPs are present in a subset of patients with sarcoidosis. The humoral immune response to HSPs relates probably to the immune activation and/or infection.

Previous studies on the relationship between activated BAL T lymphocytes and clinical features in sarcoidosis revealed controversial results. We determined lavage lymphocytes, T lymphocytes, activated T lymphocytes, helper, suppressor and natural killer cells in 50 patients with pulmonary sarcoidosis and compared the results with clinical findings, serum angiotensin converting enzyme (ACE), the radiological pattern and lung function data (vital capacity [VC], total lung capacity [TLC], FEV1, transfer coefficient KCO and arterial-alveolar oxygen difference [AaDO2] during exercise). Patients with erythema nodosum (n = 7) showed a lower proportion of activated T lymphocytes (p < 0.05) and lymphocytes (p < 0.01) than the other patients. There was a significant correlation between activated T lymphocytes and AaDO2 during exercise (r = 0.49, p < 0.001), and an inverse correlation was seen between activated T lymphocytes and VC or TLC (r = -0.35 and r = -0.36, p < 0.01). We conclude that the percentage of activated BAL T cells may be related to the degree of parenchymal involvement as expressed by functional disturbance.

Unlabelled: Sarcoidosis can be presented to the physician as an active or silent disorder, which may resolve or lead to pulmonary fibrosis. Various serum or bronchoalveolar lavage fluid (BAL) markers have been suggested when trying to find one marker or a combination of markers which could be representative of the disease. The aim of the study was to evaluate a number of biochemical markers in comparison to cellular parameters in BAL of 45 patients with active or inactive sarcoidosis, as well as in 44 patients with other disorders and of 10 healthy volunteers. Moreover, in order to get insight into ACE activity in different compartments, ACE values in BAL and serum were compared. In active sarcoidosis BAL-procollagen-III-peptide was significantly increased in contrast to the very low values in inactive disease. In healthy volunteers procollagen-III-peptide levels were below the detection limit. In BAL no significant differences were found for beta-2-microglobulin, hyaluronan or ACE. Laminin in BAL was not detectable. Serum ACE, significantly enhanced in sarcoidosis, was not discriminative between active and inactive disease.

In conclusion: only procollagen-III-peptide in BAL was found to discriminate between active and inactive disease. Besides cell differentiation and T-helper/suppressor cell (CD4/CD8) ratio in BAL, no further biochemical parameters pointing to disease or activity of disease were found.

A 36-year-old housewife was referred to our hospital in March, 1993. Her chest X ray films showed mediastinal and bilateral hilar lymphadenopathy. She was confirmed to have pulmonary sarcoidosis. We did not administer any medications for her. Four months after the diagnosis of sarcoidosis, opacities with cavitation appeared on the chest X ray. Histologic findings of lung biopsy specimens revealed granulomas with the existence of encapsulated Cryptococci. We administered an antifungal drug, itraconazole. The opacities were greatly improved by the medication. We report focal pulmonary cryptococcal infection in an untreated patient with sarcoidosis.

In disorders affecting the alveoli and lung interstitium and altered composition of the epithelial lining fluid, i.e. the surfactant, may affect the outcome of the disease. The phospholipid composition in bronchoalveolar lavage (BAL) fluid was determined in healthy non-smoking (n = 8) and smoking (n = 12) volunteers, and in non-smoking patients with clinically active sarcoidosis (n = 7). The total amount of phosphatidylcholines (median +/- SD) were in the non-smoking control group (21.8 +/- 5.7 mumol/L) and in the non-smoking sarcoidosis group (26.1 +/- 9.1 mumol/L), while healthy smokers had significantly (p < 0.05 for both) lower amounts (14.6 +/- 5.6 mumol/L). The composition of phosphatidylcholines was similar in all three groups with one exception. Palmitoylmyristoyl-phosphatidylcholine constituted a significantly higher fraction among the smokers (12.7 +/- 2.1 mol%) compared to the non-smoking control group (10.6 +/- 1.4 mol%; p < 0.05) and the sarcoidosis group (10.6 +/- 0.6 mol%; p < 0.01). In conclusion, no quantitative or qualitative differences in phosphatidylcholines were observed between non-smoking healthy volunteers and non-smoking patients with clinically active sarcoidosis. However, in smoking healthy volunteers the total amount of phosphatidylcholines was reduced and their composition altered. Earlier reported conflicting results may be due to the fact that the smoking habits have not been considered.

A previously healthy man presented with acute abdominal pain that extended from the left lumbar area to the left iliac fossa and to the omolateral testicular region. Abdominal ultrasonography and CT scan showed a bulky mesenteric mass and mesenteric, paraaortic, and paracaval lymph nodes. Biopsy specimens of the mass revealed non caseating granulomas. Chest CT scan and 67Gallium thoracic scan demonstrated lymph node and parenchymal pulmonary involvement. Bronchoalveolar lavage (BAL) confirmed the presence of a low intensity alveolitis. Serum angiotensin converting enzyme (SACE) level was elevated. Two years after steroid therapy, markers of disease activity and abdominal ultrasonography are in the normal range.

A retrospective evaluation of 244 electrocardiograms obtained at rest at time of diagnosis from a population of 254 patients with intrathoracic sarcoidosis was carried out. Thirty-five (14%) were abnormal. Sinus tachycardia was present in 17 (7%), the rest encompassing various degrees of right-sided bundle branch, atrioventricular first degree block, ventricular extrasystolia and ST-depression. At follow-up after a median time of 27 years we found a significantly higher mortality risk in the group with ECG-changes compared to those without. Excess mortality was increased when comparing patients with tachycardia to those without. However, when adjusted for FEV1, the risk of death was not significantly different between the two groups.