Sarcoidosis最新文献

Forty-seven patients with pulmonary sarcoidosis stage II-III, fulfilling clinical indications for starting treatment with corticosteroids, received oral methylprednisolone for 8 weeks in gradually decreasing doses (starting dose 48 mg per day). From week 5 onwards, they also received inhaled budesonide, 1.6 mg daily. Treatment was continued for 18 months and all patients have been followed for at least 3 years. At 18 months treatment could be discontinued in 38 patients, who had used individually adjusted doses of budesonide depending on the clinical response (reduced doses in 14, initial dose in 16, and increased doses in 8 patients). Budesonide treatment alone was satisfactory in 31 of these 38 cases. An additional seven patients could stop treatment after receiving supplementary courses of oral steroids for 3-12 months. Treatment is ongoing in 9 patients in which 6 have extrapulmonary manifestations requiring oral steroids. The chest radiograph became normal in 22 patients and improved in 14. Significant improvements were noted in FVC and DLco in relation to predicted normal values. Serum ACE, lysozyme and beta 2-microglobulin values decreased significantly. Transient cough was seen in 5 and hoarseness in 3 patients. No systemic side-effects were noted; one patient taking 2.4 mg budesonide daily had a plasma cortisol value below the normal range. Inhaled budesonide seems to offer an effective and safe alternative to oral steroids for long-term maintenance treatment of patients with pulmonary sarcoidosis.

Soluble interleukin-2 receptor (sIL-2R) concentration is considered to reflect disease activity in patients with sarcoidosis. However, it remains to be evaluated whether or not the sIL-2R concentration reflects the total burden of granulomatous lesions, or if it can be a useful marker for other interstitial lung diseases such as IPF, the lesions of which are restricted to the lungs. In this study, we demonstrated that sIL-2R concentrations in 16 patients with active sarcoidosis increased (2031 +/- 1222 U/ml), compared to those in 29 patients with inactive disease (796 +/- 313), 24 with IPF (859 +/- 694) and 33 healthy controls (467 +/- 174). sIL-2R concentrations in patients with IPF also increased, more than those in healthy subjects. sIL-2R concentrations in 10 patients with extrathoracic lesions (ETL) were not different from those in 6 patients without ETL. Correlation between serum sIL-2R concentrations and serum ACE activity, BAL macrophage %, and BAL lymphocyte % was shown in patients with sarcoidosis. In patients with IPF, a correlation between sIL-2R concentrations and BAL macrophage % was found but there was no correlation between sIL-2R concentrations and BAL lymphocyte %. In conclusion, serum sIL-2R concentrations seem to reflect total inflammatory lesions. In addition, they reflect total inflammatory lesions of the lungs in sarcoidosis and IPF. For clinical purposes, its measurement may be more useful than that of BAL fluid concentrations in patients with sarcoidosis and IPF.

Aspecific bronchial hyperreactivity (A.B.H.) is a condition found mostly in cases of bronchial asthma, but it may also present in other pathologies affecting the respiratory tract. This study examines 30 patients presenting hilo-pulmonary sarcoidosis, 27 asthmatic patients and 25 healthy subjects as controls. The three groups were sex and age matched. No significant difference in smoking habits between controls and sarcoidosis patients was found. Patients were tested for aspecific bronchial hyperreactivity by administering increasing doses of methacholine. Methacholine inhalation was performed following a dosimetric method. Aspecific bronchial hyperreactivity was found in 6 (20.6%) of sarcoidosis patients with a significant frequency (p < 0.05). No statistically significant difference was found for mean PD20FEV1 values, but the difference between asthma and sarcoidosis patients was highly significant (p = 0.003). No statistically significant difference was found between basal FEV1 means expressed as a percentage of the former in asthmatics and sarcoidosis patients, both responsive and unresponsive. Although it is difficult to draw conclusions from these findings, aspecific bronchial hyperreactivity was seen to increase in subjects with sarcoidosis, and this may explain the not rare association between asthma and sarcoidosis.

Blau's syndrome is a familial multisystem granulomatous inflammation which may be confused with childhood sarcoidosis because it presents with iridocyclitis, posterior uveitis, granulomatous skin disease, arthritis and elevated serum angiotensin-converting enzyme. They are distinguished by the absence of pulmonary involvement and a negative Kveim-Siltzbach skin test.

Standard epidemiological and clinical features of sarcoidosis were evaluated in 24 regional centres from Moravia and Silesia (4 million inhabitants). During 1981-1990 the incidence ranged from 3.3 to 4.4/100,000 with slight decrease after 1985, when unselected chest X-ray screening was restricted. The prevalence (however disputable in sarcoidosis) increased from 41.3 to 63.1/100,000. Sex ratio male/female was 1: 2.35; only 39% of patients were younger than 40 years. Biopsy supported the diagnosis in 60% of cases. Tuberculin negativity was found in 64%. X-ray types were presented as follows: 0-2%, I-71%, II-22%, III-5%. Regional incidences varied from 0.9 to 11.7/100,000. Lower values were found in mining areas and high pneumoconiosis incidence. The course of the disease was benign with only sporadic extrapulmonary complications or pulmonary fibrosis. The results indicate that improved knowledge of sarcoidosis has brought epidemiological equilibrium to the evaluated area.

Two patients with pleural effusions have been seen among 270 patients with sarcoidosis in an eighteen-year period. In both patients pleural effusions were present at the onset of sarcoidosis and associated with extensive pulmonary or extrathoracic involvement. Pleural effusions were exudative with a high lymphocyte count. One patient was successfully treated with corticosteroids and the second died from cardiac complications of the disease.

Pleural effusion is a well-recognized clinical entity that can be associated with sarcoidosis. Nevertheless, the real prevalence of this phenomenon remains to be established. This study describes the case of a 57-year-old male sarcoid patient who presented with right exudative pleural effusion, dyspnea on exertion, and bilateral pulmonary interstitial infiltrates. Sarcoidosis was diagnosed more than 2 years prior to the onset of pleural involvement. Pleural biopsy revealed the presence of typical sarcoid non caseating granulomas. Sarcoid involvement of the pleura resolved following a 1-month course of high doses of steroids and did not recur during a 18-month follow up. When we retrospectively analyzed clinical data obtained from 624 consecutive sarcoid patients who were referred to our hospital between January 1980 and June 1993 and examined for the presence of pleural involvement, the only patient who showed pleural effusion and histologically proven sarcoidosis of the pleura was the case here described. The frequency of the phenomenon in our series is 0.16%. We conclude that pleural effusion represents a rare event in sarcoidosis.

We report an unusual case of non-Hodgkin T cell lymphoma associated with sarcoidosis. A 44-year-old woman was histologically diagnosed as having sarcoidosis in 1966. Diffuse interstitial lung disease and erythematous skin nodules disappeared following steroid therapy. Twenty years after sarcoidosis onset erythematous nodosum recurred and biopsy specimens revealed non caseating epithelioid granuloma. Thirty-seven months later a malignant T cell lymphoma developed in the skin portion where sarcoid lesions recurred. We discuss the possible relationship between the sarcoid inflammatory process and the development of a malignant T cell clone as origin of the T cell lymphoma.

When Sephadex beads (0.45mg/kg b.w) are instilled intratracheally into rats, a granulomatous alveolitis with giant cell formation and fibrosis occurs. Moreover, the events in the alveolar region are paralleled by an eosinophil-dominated peribronchitis/bronchiolitis and perivasculitis. Bronchoalveolar lavage (BAL) shows a very distinct feature with an early pronounced neutrophil increase, followed by an increase of eosinophils and lymphocytes. BAL findings returned to normal after 1-2 weeks, but tissue morphology showed persistent inflammation with large numbers of eosinophils and to a lesser degree mononuclear cells, peribronchially and perivascularly several weeks after the instillation. Fragmentation of the Sephadex beads by ultrasonication dramatically diminished the response, giving a transient neutrophil alveolitis, without eosinophils and with no granuloma formation. On the other hand, when the Sephadex dose was divided into three, given 10 days apart, a more pronounced fibrosing activity occurred, with mast cells appearing in the collagen rich granulomas. Finally, smoke exposure had a significant suppressive effect upon the response. The numbers of cells in the interstitium as well as in the peribronchial and perivascular tissue were markedly decreased in the smoke exposed group compared to the controls. This decrease was mainly due to decreased numbers of mononuclear cells, while the numbers of eosinophils remained unchanged.