Sarcoidosis最新文献

Endothelin-1 (ET-1) is a vasoactive, mitogenic peptide that is variably increased in Bronchoalveolar Lavage Fluid (BALF) and immunohistochemically found in lung tissue of patients with Interstitial Lung Disease (ILD). To assess if endogenous ET-1 production is increased in ILD we evaluated 24 hour (24h) urine excretion of ET-1 in 20 patients with ILD and 10 healthy age-matched controls (HC). Eight patients with active pulmonary sarcoidosis (S), 6 with idiopathic pulmonary fibrosis (IPF) and 6 with focal lung fibrosis due to inactive pulmonary tuberculosis (hTB) were studied. Plasma ET-1 levels (ET-1pl, pg/ml) and 24h ET-1 levels in urine (ET-1ur, ng/24h) were measured by a specific radio-immunoassay. Determinations of ET-1p1 and ET-1ur were repeated in S and IPF patients after 30 days of prednisone (0.75 mg/kg/day) treatment. ET-1p1 concentrations were not different between HC (5.34 +/- 0.48), S (5.95 +/- 0.96), IPF (4.75 +/- 1.37) and hTB (5.97 +/- 1.05) groups. ET-1ur was significantly higher in S (189.50 +/- 60.57) than in HC (69.00 +/- 10.76), IPF (62.17 +/- 19.07) and hTB (82.00 +/- 24.97). After prednisone, ET-1ur in the S group decreased significantly (189.50 +/- 60.57 to 94.00 +/- 13.60), and the decrease paralleled the improved clinical status. In S patients, ET-1ur was not significantly correlated to the degree of respiratory impairment, but it was significantly correlated to the intensity of lymphocytic alveolitis (r = 0.80).(ABSTRACT TRUNCATED AT 250 WORDS)

Pulmonary granulomatous disease can severely damage the lungs and represents a rather uniform response of the lungs to a multitude of different stimuli. Histologically, granulomatas can be broadly classified as either hypersensitivity-type or foreign-body-type. A wide variety of infectious and non-infectious etiologies discussed in this review have been identified as causes of granulomatous inflammation of the lungs. An infectious origin should always be excluded since specific etiologic therapy may be implemented. Culture techniques for microorganisms or fungi and standard or special stains of tissue samples are crucial to establish the specific etiologies. The mechanisms leading to granuloma formation are still poorly understood, but the role of parenchymal cells, fibroblasts, endothelial and epithelial cells, and cytokines, has been better appreciated; all of them are important in the initiation, maintenance, and resolution of the lesion. Whether these new insights will finally lead to new ways of treating lung granuloma remains to be determined.

Mucormycosis is a rare complication of sarcoidosis. We report only the third instance of mucormycosis occurring in a patient with sarcoidosis. Corticosteroid therapy, even short courses of less than one month duration, appears to be a major risk factor for the development of mucormycosis. Mucormycosis should be suspected upon the development of signs and symptoms of chronic sinusitis, necrotic nasal discharge, proptosis or periorbital edema. Mucormycosis is confirmed on routine hematoxylin and eosin stains by the identification of tissue invasion by the broad, aseptate mucor fungi. Prompt identification of the infection is essential to reduce morbidity and prevent mortality.

In the present study, T cell lines, designated TU/BAL and KC/LN, were established from bronchoalveolar lavage (BAL) fluid and an affected lymph node, respectively, obtained from patients with active sarcoidosis by cultivating in the presence of IL-2. These cell lines produced IL-2 and proliferated by stimulation with the patient's own peripheral blood mononuclear cells treated with mitomycin C, while three other T cell lines established from ConA-stimulated BAL cells of patients with non-sarcoid lung diseases did not show any proliferative responses. The proliferation was mediated by IL-2, because anti-IL-2R alpha-chain monoclonal antibody (mAb) inhibited this response in a dose-dependent fashion. The adherent cell was a main stimulator of the proliferation. Both CD4 and HLA-DR appeared to be involved, because mAbs against these molecules inhibited this response. These results suggest that T cells obtained from sarcoid patients respond to a certain unknown antigen associated with HLA-DR or some self antigen expressed on the monocytes. Furthermore, both TU/BAL and KN/LN represented a profile of Th1-like cells: they secreted IL-2 and IFN-gamma, but not IL-4, IL-5 and IL-6, when stimulated with PHA in the presence or absence of 12-O-tetradecanoylphorbol-13-acetate. Thus, Th1-like cells activated by some unknown antigen(s) might play roles in the pathogenesis of sarcoidosis.

Some reports correlate the administration of all forms of interferon with the development or exacerbation of autoimmune phenomena and diseases, including sarcoidosis, because of the strong and complex immune action of interferons. We report on a case of sarcoidosis following beta-interferon treatment for multiple myeloma. Differently from what had been observed in all the 8 previously reported cases of associated multiple myeloma and sarcoidosis, where the plasmacellular malignancy followed the onset of the respiratory disease, in the case of our patient, sarcoidosis arose after multiple myeloma was first diagnosed.

The reported frequency of splenomegaly in sarcoidosis has ranged from 1% to 40%. Splenomegaly has been associated with clinical evidence of more extensive extrathoracic sarcoidosis. In a previous study, we reported that splenomegaly was associated with a poor outcome of the disease. The aim of the present study was to describe the characteristics of the sarcoidosis patients with splenomegaly seen at our institution. Of 284 sarcoidosis patients, followed up at our hospital, 16 (5.6%) had splenomegaly on physical examination. These patients showed other extrathoracic manifestations as well, and all but two showed intrathoracic involvement. Eleven patients received corticosteroid therapy. Splenectomy was done in three patients, but the natural history of their sarcoidosis remained unaltered two years after diagnosis. The results of this study disclosed that patients with splenomegaly had evidence of extensive pulmonary and extrathoracic sarcoidosis with a poor prognosis in spite of steroid therapy.