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ABSTRACT: The macrophage activation syndrome (MAS) is a rare and potentially fatal disease. This syndrome is founded on congenital or acquired dysfunction of NK cells resulting in secondary activation and proliferation of macrophages with excessive cytokine production and organ infiltration. Causes of acquired MAS include viral infections (chiefly EBV and CMV), malignancies, and autoimmune diseases. The macrophage activation syndrome is usually associated with juvenile idiopathic arthritis and adult-onset Still's disease and rarely with rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, and systemic sclerosis. Fever, hepatosplenomegaly, lymphadenopathy, and bi- or pancytopenia in peripheral blood represent typical symptoms of MAS. Hyperferritinemia, hypertriglyceridemia, hypertransaminasemia, and hypofibrinogenemia are among the common laboratory findings. The macrophage activation syndrome is a life-threatening condition requiring aggressive therapy due to multiple organ dysfunction. Treatment also includes elimination of the triggering infection and high-dose glucocorticosteroids. Second-line therapy is based on cyclosporin, intravenous immunoglobulins, and etoposide. The present work focuses on diagnostic and therapeutic difficulties in three patients with the macrophage activation syndrome.
Annales Academiae Medicae Stetinensis 01/2012; 58(1):55-61.
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ABSTRACT: Global disease activity measurement in systemic lupus erythematosus (SLE) patients is important for the clinical estimation and adjustment of therapy. By contrast, immune system activation plays a significant role in disease pathogenesis, with CD4+ lymphocytes acting as central cells in the immune response. We investigated which scale better correlates with immunologic changes in the blood of SLE patients, the SLE Disease Activity Index (SLEDAI) or the Systemic Lupus Activity Measure (SLAM) scale. Samples of peripheral blood were obtained from 45 SLE patients with different disease activity as assessed by the SLEDAI and the SLAM scales on the same day. We assessed the percentage of CD4+ T cells with activation-associated receptors: CD69, CD25int, CD95, HLA-DR, and CD4+ T cells with killing properties containing perforin and granzyme B. Our results indicated that the percentage of CD4+CD69+ and CD4+CD25(int) cells did not correlate with either the SLEDAI or the SLAM scale. Significant and positive correlations were observed between percentages of CD4+CD95+ and CD4+HLA-DR+ lymphocytes and SLE activity, but only when activity was measured using the SLAM scale, not with the SLEDAI scale. The percentage of CD4+perforin+ and CD4+granzyme B+ cells also strongly correlated with disease activity measured only with the SLAM scale. We conclude that the SLAM scale better reflects changes of immune system activity in SLE patients compared with the SLEDAI scale.
Human immunology 08/2011; 72(12):1160-7. · 2.55 Impact Factor
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ABSTRACT: Systemic lupus erythematosus and Still's disease are chronic autoimmune disorders of unknown etiology. Symptomatology of these diseases may be similar causing diagnostic difficulties. Long-term observation and immunological studies are essential to identify the definite disorder. We present a case of a 24-year-old patient with high fever, sore throat and arthritis. During hospitalization rash accompanying fever, nodular erythema, pulmonary changes, liver damage and splenomegaly were observed. Although initially adult-onset Still's disease was diagnosed according to the Yamaguchi criteria, the diagnosis of systemic lupus erythematosus was made after re-analysis of the clinical course and immunological tests.
Przegla̧d lekarski 01/2011; 68(4):239-41.
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ABSTRACT: Takayasu's arteritis (TA) is a granulomatous inflammation of the aorta and its major branches, usually occurring in young women. The disease is rare in Europe. The aim of this study was to describe clinical manifestations of TA among patients at the Department of Connective Tissue Diseases, University Medical Center in Gdańsk.
We conducted a retrospective data analysis and performed a clinical examination of the patients. All patients met ACR criteria for TA. The mean age at disease onset was 26 years and the mean diagnostic delay was 19 months. Four out of 12 patients had a history of extensive stroke. Bypass surgery or balloon angioplasty was done in four patients. One patient underwent aortic valve replacement.
Early diagnosis and pharmacologic treatment may significantly improve the prognosis in TA.
Annales Academiae Medicae Stetinensis 01/2010; 56 Suppl 1:95-8.
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ABSTRACT: The aim of the study was to assess myocardial perfusion in women with systemic lupus erythomatosus (SLE) and no symptoms of coronary artery disease (CAD).
Twenty two women with SLE of mean age 40.5 +/- 7.2 were enrolled in the study. The average duration time of the disease was from 2 to 19 years, mean 8 +/- 4.6 years. The inclusion criterion was the absence of stenocardial symptoms. The myocardial perfusion was studied by using Single Photon Emission Computerized Tomography (SPECT) utilising (99m)Tc-MIBI and a triple-head gamma-camera. We also analyzed risk factors of heart ischemic disease in our group.
Myocardial perfusion stress scanning showed abnormal perfusion in 12 patients, 54.5% of the whole group, mostly in the anterior wall. At rest hypoperfusion abnormalities were found in 7 individuals. In patients with positive myocardial perfusion, out scanning, risk factors of CAD were more pronounced than in a sub-group with a negative result of myocardial perfusion scanning.
In young women with SLE and no symptoms of coronary artery disease, myocardial perfusion defects may be detected by means of myocardial perfusion scintigraphy. Exercise and resting electrocardiography tests could be not sufficient for CAD diagnosis in women with SLE. The presence of coronary artery disease risk factors in women with SLE could be an indication to perform myocardial perfusion SPECT scanning.
Nuclear medicine review. Central & Eastern Europe: journal of Bulgarian, Czech, Macedonian, Polish, Romanian, Russian, Slovak, Yugoslav societies of nuclear medicine and Ukrainian Society of Radiology 02/2004; 7(2):171-4.
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ABSTRACT: As Sjögren syndrome is regarded by ophthalmologists as a synonim of dry eye syndrome, we decided to evaluate how often these patients demonstrate other than ocular symptoms, especially systemic complications.
We examined 30 patients with primary (PZS) and 45 patients with secondary Sjögren syndrome (WZS).
Pathological findings in course of Sjogren syndrome concerned predominantly eyes (94%) and mucous membrane of the mouth (91%), salivary glands (65%) and skin. The most frequent complaint was fatigue (91%).
Sjogren syndrome is a chronic systemic disease, in which predominant symptoms are: dryness of mucous membranes, chronic fatigue, painful joints and stomach disturbances.
Klinika oczna 02/2004; 106(3 Suppl):466-8.
