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Background/Objectives: Lupus nephritis (LN) is one of the most severe organ manifestations of systemic lupus erythematosus (SLE). The aim of our retrospective cohort study was to compare the clinical characteristics, therapy, survival, causes of death, and prognostic factors of LN and non-LN lupus patients. Moreover, we compared a wide spectrum of clinical data of LN patients diagnosed before and since 2005 to determine any changes in disease course and outcomes. Methods: We assessed the clinical and laboratory data of 384 SLE patients, out of whom, 127 patients were diagnosed with LN between 1990 and 2020. Results: Based on our observations, discoid LE, subacute cutaneous LE, antiphospholipid syndrome, Sjögren's syndrome, and rheumatoid arthritis were more common in non-LN patients, while anemia and anti-RNP positivity were more frequent in LN patients. Development of LN did not affect survival rates; male sex and presence of APS were negative prognostic parameters in the non-LN group while achieving remission was a positive prognostic factor in both groups. Death caused by sepsis was more prevalent in the LN group. Serositis and neurological manifestations occurred less frequently in LN patients diagnosed after 2005. The use of mycophenolate mofetil became more common, and the cumulative corticosteroid dose decreased. The SLICC Damage Index score also decreased. Conclusions: Our study demonstrated that the disease course has changed in recent years, and the main therapeutic goal in both SLE and lupus nephritis should be to achieve remission because this significantly improves long-term prognosis and patient survival. [ABSTRACT FROM AUTHOR]

Background: Systemic lupus erythematosus is a chronic autoimmune disease with multiple organ involvements. Dup to earlier diagnosis and more effective therapeutic possibilities the survival in lupus has improved significantly in recent decades, however, critical situations may develop., Objectives: In the present work the authors aimed to summarise critical complications of lupus requiring urgent therapeutic modification, hospitalisation, sometimes at intensive care units., Results: As a systemic autoimmune disorder lupus may involve all vital organs of the human body and as such, it may result in heart failure, respiratory and renal insufficiency, and severe, life-threatening central nervous system complications. These can develop within a short period due to an acute flare, but also as the consequence of chronic progression. Infections, pregnancy, withdrawal of therapy, physical and psychological stress may provoke the worsening of the disease. However, critical situations can be the consequence not only directly of lupus, but due to side-effects of therapy, associating disorders and co-incidence with other severe diseases., Conclusions: Lupus may cause several critical situations that require urgent intervention and/or hospitalisation. The recognition and the solution of these severe lupus crises is a complex, multidisciplinary challenge that should be organised by competent, well-prepared immunologic centres.

Introduction: Chronic organ damages and complications have become one of the major problems concerning morbidity and mortality in systemic lupus erythematosus., Aims: Present study was to examine the frequencies and types of malignancies in a lupus population followed regularly in our department, and to find correlation between cancer-associated mortality and the use of immune suppressants., Methods and Results: Authors analysed data 860 lupus patients from 1965 till the end of 2004. During study period 164 patients died, 18 of whom from cancer. The frequency of cancer-associated mortality was 11% that was 2% of the total lupus population. Mortality has increased within 10-year periods reaching the maximum between 2001-2004 (8/24, 33%). Age of the 37 patients affected by malignancy was 47.7 (20-73) years. Cancers appeared 13.4 (1-45) years after the diagnosis of lupus. Breast cancer occurred most frequently (n=11) followed by gastro-intestinal malignancies (n=9), then cervix cancer and different hematologic malignancies (5-5 cases), bronchial cancer (n=4) and skin, ovarian and vesical cancers (1-1-1 cases). Relative risk of non-Hodgkin lymphoma and cervix cancer increased significantly as compared to those of the Hungarian general female population. Before the cancer developed 62.2% of patients received azathioprine and/or cyclophosphamide., Conclusion: Present results describe and analyse for the first time the association between lupus and malignant disorders in a Hungarian population calling the attention for the increased risk for the development of cancers and the importance of regular screening and extended examinations in SLE patients.

Introduction: Cyclosporin A is a peptide composed of 11 amino acids. Its immune suppressive effect is attributed to the suppression of proliferation of T lymphocytes. Recently, besides immune suppression after organ transplantation it is used in the treatment of autoimmune disorders, such as systemic lupus erythematosus., Aims: In the present work authors examined the frequency, effectiveness and safety of cyclosporin A in patients with systemic lupus erythematosus, regularly followed at the above mentioned department., Results: Of the 450 lupus patients one male and 21 females received cyclosporin A for 12.4 months (3-96). Before cyclosporin A administration 16 patients received combined immune suppressive therapy; 68.2% azathioprine, 27.3% chloroquine, 18.2% methotrexate, while 3 patients were treated with steroid alone. High autoantibody titres clinical activity and the need for reduction of steroid dose were the mean indications. Furthermore, cyclosporin A was started because of leukopenia in 45.5%, polyarthritis in 36.4% or both in 27.3% of patients. Four patients started cyclosporin A to control skin eruptions. Thrombocytopenia, autoimmune hemolysis, Evans' syndrome and pancytopenia indicated this therapy in one patient for each. Clinical effectiveness was indicated by the reduction of mean daily methylprednisolone dose from 25.1 mg to 8.36 mg and also by the reduction of the disease activity index (SLE-DAI) from 9.47 to 2.47 within the first half year of the treatment. Therapy was well-tolerated. Only 3 patients observed hypertrichosis. Cyclosporin A was stopped in 3 cases due to infection and in 1 patient due to bad compliance., Conclusion: Present results suggest that cyclosporin A is an effective and safe treatment also in a certain group of lupus patients, especially with joint, skin and hematologic complications. Further long-term follow-up is required to evaluate late side-effects.

Introduction: Immune-inflammatory processes play important role in the pathogenesis of atherosclerosis. Therefore, increasing attention is focused on rheumatic diseases with chronic inflammation, such as systemic lupus erythematosus. Besides direct influences, inflammation may modify the development of atherosclerosis by other mechanisms., Aims: To examine paraaoxonase activity and lipid profile in lupus patients., Methods: Authors entered 37 definitive lupus patients and 30 age- and sex-matched normal controls into the present study. Patients' age was 40.8 +/- 13.9 year, follow-up time 6.7 +/- 6.2 year, disease activity index 2.8 +/- 3.4. Lipid parameters (total cholesterol, LDL-C, HDL-C, Apo-AI and ApoB) were determined by an autoanalyser, paraoxonase and arylesterase activities were measured spectrophotometrically. Phenotypic distribution of the enzyme was determined by dual substrate method. Anti-oxLDL was measured by ELISA method, CRP by automatised immunoassay. Statistical analysis was performed by SPSS program., Results: Despite of long disease duration and low inflammatory activity authors found significantly (p < 0.001) decreased paraoxonase activity (121.9 +/- 65.9 U/mL) (p < 0.001) in lupus as compared to control (188.1 +/- 78.9 U/mL), which correlated with the presence of atherothrombotic complications (p = 0.009). High activity BB phenotype did not occur in lupus. Lipid parameters and arylesterase activity were within normal range in both groups. No significant correlation was found between paraoxonase activity and disease activity index, dose of corticosteroid therapy, CRP and anti-oxLDL level. Arylesterase activity did not differ in lupus and control groups., Conclusions: Present results suggest that other mechanisms, e.g. antibodies, genetic factors, alteration in the distribution of HDL-subfractions or ensyme abnormalities in HDL remodelling may stand at the background of reduced paraoxonase activity in lupus.

Introduction: Antiphospholipid syndrome is a multi-organ autoimmune disorder, characterized by arterial and venous thrombotic events, and a well-defined group of recurrent foetal wasteage due to pathologic antibodies against phospholipids and protein co-factors. Antiphospholipid antibodies can be formed in primary antiphospholipid syndrome, but also in other conditions, most often in systemic lupus erythematosus. This may modify the outcome of lupus increasing the risk for thrombotic complications. Less is known about the outcome in primary antiphospholipid syndrome, whether it may precede the development of systemic lupus., Aims: Authors hereby describe the case of four patients with primary antiphospholipid syndrome in whom the disease progressed to systemic lupus erythematosus., Results: Lupus followed the primary antiphospholipid syndrome within around a three-year period. It was indicated by the appearance of different antinuclear autoantibodies and clinical complications, such as polyarthritis, nephritis and hematologic disturbances. All of the patients presented cerebrovascular accident as the thrombotic manifestation. All but one, were around forty years old, and had a milder form of lupus. Symptoms of the antiphospholipid syndrome determined the outcome. On the other hand, a typical lupus developed in the youngest patient., Conclusions: According to present cases antiphospholipid syndrome may be considered as the initiative phase of SLE, but APS being a separate entity also may associate to lupus. Present observations indicate the importance of follow-up the patients with APS by immunologic respect. Future prospective, multi-centre studies with larger number of cases are needed to provide further evidence on the fact that patients with APS may acquire other autoimmune disorder.

Systemic lupus erythematosus (SLE) is a multifactorial polysystemic autoimmune disorder. Although life expectance in SLE has been improved by adequate immune suppressive therapy, the importance of chronic renal failure has not been reduced. Among late complications of the disease accelerated atherosclerosis attempts increasing attention. Dyslipoproteinemia and increased concentration of lipoproteins are important risk factors of atherosclerotic cardiovascular complication in SLE. Serum lipid parameters of 50 patients with lupus were examined in the present work. Thirty patients had histologically proven lupus nephritis (LN+), while the other group did not have renal involvement (LN-). Serum triglyceride, total cholesterol, LDL-C and apolipoprotein B (apoB) concentrations were significantly higher in the lupus nephritis (LN+) group. On the other hand, HDL-C and apoAI levels were also elevated in patients with LN. As a consequence of that, LDL-C/HDL-C and the apoB/apoAI ratios did not differ between patients with or without kidney involvement. This concluded the authors to measure the concentration of lipoprotein (a) in SLE patients, as Lp(a) is known to be an independent risk factor of atherosclerosis. Results indicated a significantly increased Lp(a) concentration in patients with lupus nephritis as compared to the LN- group. All but 2 patients without kidney involvement had lower than 100 mg/L Lp(a) concentration, while 27% of patients with lupus nephritis has an Lp(a) level between 100-300 mg/L. Further more, Lp(a) concentration was higher than 300 mg/L in 13% of the LN+ group. In a good correlation of these observations patients with nephritis suffered more frequently from deep venous thrombosis and ischaemic heart disease. The frequencies of hypertension and non-insulin dependent diabetes mellitus were slightly elevated in patients with nephritis. Present results suggest the importance of elevated lipoprotein (a) concentration in patients with lupus nephritis, further increasing the risk of athero-thrombotic cardiovascular complications.