Limfangioleiomiomatoza (LAM) : trudności diagnostyczne

Background: LAM is a rare idiopathic disorder found almost exclusively in premenopausal women. It is characterized by a proliferation of abnormal smooth muscle cell in the pulmonary interstitium, in the lymphatic system of the thorax, retroperitoneum and by the formation of pulmonary parenchymal cysts. The most common clinical manifestations of LAM are pulmonary symptoms including progressive dyspnoea, pneumothorax and chylous effusion. Case report: The authors describe a case of a 43-year-old woman with lymphangiomyomatosis (LAM) with initial manifestation of pneumothorax and retroperitoneal cystic mass. Conclusions: HRCT is the best method for cyst detection and is essential for diagnosis. A radiologist is often the first physician to suggest the diagnosis of LAM. However, misdiagnosis is common and may result in inappropriate therapeutic procedures that can further complicate the treatment. There are characteristic abdominal findings in patients with LAM, that in conjunction with the HRCT findings of pulmonary cysts, prove to be useful in establishing this diagnosis

Enhanced expression of Fas Ligand (FasL) in the lower airways of patients with fibrotic interstitial lung diseases (ILDs)

The exact role of FasL, and particularly its soluble and membrane-bound forms, in the development of chronic ILDs and lung fibrosis has not been extensively explored. We aimed at analyzing membrane-bound FasL expression on alveolar macrophages (AM) and lymphocytes (AL) as well as soluble FasL (sFasL) levels in bronchoalveolar lavage (BAL) from ILDs patients, incl. pulmonary sarcoidosis (PS), hypersensitivity pneumonitis (HP), silicosis, asbestosis, idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), and healthy subjects (n = 89, 12, 7, 8, 23, 6, 17, respectively). In IPF, significantly increased percentage of AM FasL+ and CD8+FasL+ cells as well as sFasL levels in BAL were found. Increased sFasL levels were also observed in HP. NSIP and asbestosis were characterized by higher AM FasL+ relative number; CD8+FasL+ population was expanded in asbestosis only. There was a significant decline in AL FasL+ percentage in PS and HP. Vital capacity was negatively correlated with sFasL levels, AM FasL+ and CD8+FasL+ cell relative count. CD4+FasL+ and CD8+FasL+ percentage strongly correlated with BAL neutrophilia, an unfavorable prognostic factor in lung fibrosis. The concurrent comparative BAL analysis of FasL expression indicates that FasL+ AM and AL (mainly Tc cells) comprise an important element of the fibrotic process, mostly in IPF. FasL might play a crucial role in other fibrosis-complicated ILDs, like NSIP and asbestosis. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 4, pp. 636–645

A single photon emission computed tomography of the chest with the use of receptor radiotracer (Tc-99m-Depreotide) in sarcoidosis

Background: Sarcoidosis is a systemic and multiorgan disease with unknown etiopathogenesis. Granulomas that do not undergo necrosis and caseous degeneration are distinctive for this disease. Mostly it is connected with young adults, more frequently females than males, and changes are mainly situated in the lymph nodes of the pulmonary hilus and pulmonary parenchyma. Somatotropin release inhibiting hormone receptors could be located in epithelioid and giant cells that create sarcoidal tubercles and lymph nodes. Aim of the study was to determine the usefulness of a single photon emission tomography (SPECT) for an evaluation of the range of sarcoidal changes in the chest after using a receptor tracer. An assumption was made that scintigraphy with the use of Tc-99m-Depreotide could help with location of extrapulmonary sarcoidosis focuses. Material/Methods: Authors present five patients with clinically recognized and histopathologically confirmed sarcoidosis. Patients were given Tc-99m-Depreotide and underwent SPECT of chest. The results were compared with X-rays of these patients chests and with the accumulation of radiotracer in 2 other patients with carcinoid syndrome without visible pathological changes in examination. Patients got an intravenous injection of 500 MBq (14mCi) Tc-99m-Depreotide. SPECT of chest together with a „whole body” examination, was performed after 2 hours and 24 hours. Results: Higher radiotracer accumulation was observed in all patients in the area of some chest lymph nodes, in pulmonary tissue in 3 patients and in other groups of lymph nodes in 2 patients. Conclusions: Emission Tomography of the chest with the use of receptor radiotracer (Tc-99m-Depreotide) can be a crucial complement of sarcoidosis diagnostics in an evaluation of the extent of lung changes together with an estimation of chest lymph nodes abnormalities