Magdalena Szmyrka-Kaczmarek

Peripheral blood Th17/Treg imbalance in patients with low-active systemic lupus erythematosus.

INTRODUCTION The balance between proinflammatory Th17 cells and regulatory T cells plays an important role in the pathogenesis of autoimmune diseases, including systemic lupus erythematosus (SLE). In particular, an increased ratio of Th17/Treg cells has been shown to correlate with active SLE and specific organ involvement. The aim of our study was to assess Th17 and Treg cell populations in peripheral blood (PB) of patients with clinically quiescent SLE, and to evaluate their correlation with organ involvement. MATERIAL/METHODS We performed flow cytometric analysis of studied T CD4+ cell subpopulations in PB from 21 patients with SLE and 13 healthy controls. Disease activity was measured with the SELENA-SLEDAI index; organ involvement was divided into renal, neurological and hematological. RESULTS A statistically significant difference (p<0.01) between the mean percentages of CD4+CD25highFoxP3+ Treg cells in SLE patients (18.57%) and healthy controls (32.08%) was observed. Similarly, proportions of functional CTLA-4+ Treg cells were markedly lower in SLE patients than in healthy controls--19.3% vs. 23.82% (p=0.03). In contrast, SLE patients exhibited a significantly increased frequency of circulating Th17 cells with the phenotype CD4+IL-17+ compared to controls--1.36 % vs 0.19% (p<0.01). Also the ratio of Th17 cells to Th1 cells was markedly higher in SLE patients than in the control group (p<0.01). We did not find any correlation of PB Th cell distribution with organ involvement in SLE patients examined. CONCLUSIONS Our report showed for the first time that systemic Th17/Treg imbalance occurred also in patients with low disease activity and in remission. We suggest that immunological alterations may precede clinical and laboratory symptoms of the disease activity.

Clinical and immunological characteristics of Polish patients with systemic lupus erythematosus

BACKGROUND Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with varied clinical manifestations, which creates difficulties and delays in establishing a diagnosis. OBJECTIVES The aim of this study was to evaluate the prevalence and nature of the clinical symptoms of SLE, both at the onset of the disease and in its further course. An attempt to assess the immunological characteristics of the patients and to analyze autoantibodies variability over time was also made. MATERIAL AND METHODS This retrospective study included 71 Caucasian patients, 63 women and 8 men, meeting the criteria for diagnosis of SLE according to ACR. RESULTS The ratio of women to men was approximately 7.9:1. The average age of the onset of SLE was 31.5 years. The average time from the onset of symptoms to diagnosis was 5 years. The most common first manifestation of SLE were joint and muscles symptoms - 71.8%, skin lesions - 69.0%, fever - 57.7%. The main symptoms in the further course of the disease were neurological disorders - 69.0%, joint and muscle changes - 67.7%, and general symptoms - 59.2%. There was an increase in the incidence of renal involvement and neurological symptoms throughout the disease course. The most commonly detected antibodies were anti-dsDNA - 47.9%, anti-Ro/SSA - 40.8%, anti-nucleosomal antibodies - 29.6%, and lupus anticoagulant - 22.5%. A panel of antibodies typically did not change. CONCLUSIONS There is no typical clinical picture of SLE, the population suffering from this disease is very various. Therefore, early and accurate diagnosis can be a big challenge for any clinician, which justifies the need for this type of study to better characterize the disease.

Pregnancy exacerbates complications of acquired hemophilia in a patient with systemic lupus erythematosus

Acquired hemophilia (AH) is a rare disease characterized by the production of antibodies directed against blood coagulation fVIII leading to coagulation disorders due to lowering of its activity. These are mostly the IgG class of antibodies which does not fixate the complement (IgG1 and IgG4 class) against A2 or C2 domains of fVIII which are responsible for its activity. The incidence of AH is 1.3–1.5 cases per 1 000 000 population per year and may affect both women and men [1]. Acquired hemophilia may be a primary disease or occur in the course of malignant diseases (e.g. prostate and lung cancer, lymphoproliferative and myeloproliferative syndromes), autoimmune diseases, during drug therapy (e.g. penicillin, sulfonamides, phenytoin, chloramphenicol, methyldopa, fludarabine and interferon) and pregnancy and puerperium. There were reported cases of AH in the course of systemic lupus erythematosus (SLE), primary Sjogrens syndrome, rheumatoid arthritis, giant cell arteritis and polymyositis. In around 50% of cases the exact cause is unknown [2, 3]. Acquired hemophilia can occur at any age and is characterized by hemorrhagic diathesis caused by bleeding into soft tissues, skin and mucosa. Other symptoms include large areas of ecchymoses, hematomas under the skin, nosebleed, hemoptysis, vaginal, urinal, gastrointestinal and retroperitoneal bleeding. In contrast to congenital hemophilia, hemarthroses are rare [2]. Laboratory tests reveal prolonged activated partial thromboplastin time (APTT), which is not corrected by infusion of fresh frozen plasma (FFP) and prothrombin time (PT) within normal range. Acquired hemophilia is usually characterized by a severe course of the disease and a high mortality rate [4, 5].