Lege Artis Medicinae

[Analysis of short-term and long-term survival and causes of death in patients with systemic lupus erythematosus]

TARR Tünde, KISS Emese, SZEGEDI Gyula, ZEHER Margit

MARCH 20, 2011

Lege Artis Medicinae - 2011;21(03)

[INTRODUCTION - In systemic lupus erythematosus (SLE), both short-term and long-term survival rates have improved worldwide. We analysed retrospectively the short-term and long-term survival data and causes of death at a single center. These data were compared with previous survival data recorded at the same centre and published in international studies. PATIENTS AND METHOD - The data of 550 patients with SLE were analysed between 1970 and 2009. We examined the effect of clinical symptoms, age, severity and onset of the disease and the applied immunosuppressive treatment on survival, using the Kaplan-Meier method. RESULTS - Survival rates at 5, 10, 15 and 20 years after the diagnosis were 98%, 94%, 90% and 89%, respectively. Late onset, neuropsychiatric symptoms and severe SLE were found to be prognostic factors. Manifestations affecting other organs and the applied immunosuppressive therapy did not influence survival rates. During the study period, 57 out of the 550 patients (10.4%) died. The main causes of death were cardiovascular complications (50.9%), infections (21%), and malignancies (12.3%). CONCLUSIONS - Our results show that among patients with SLE, it is mostly longterm survival that has increased, owing to the close control of patients. The increase in cardiovascular mortality highlights the importance of regular screening.]



Further articles in this publication

Lege Artis Medicinae

[“Home Birth should not be Legalized, it is Legal” – An Interview with Ágnes Geréb]


Lege Artis Medicinae

[Gustav and Alma Mahler ]


Lege Artis Medicinae

[An Abundance of New Knowledge – Csaba Farsang: The Handbook of Hypertension]

RÁCZ Károly

Lege Artis Medicinae

[Three Roles: – Chekhov as the Author, the Physician and the Patient]

KISS László

Lege Artis Medicinae

[ECG artefacts]


[The recognition of ECG artefacts is becoming increasingly important for physicians working in the field of internal medicine. At the same time, however, very little information about artefacts is published in either articles or textbooks. The authors provide a summary of the generation, types and recognition of ECG artefacts. The aim of the article is to draw the attention of clinicians to the dangers of this increasingly common phenomenon. Unrecognised artefacts can often prompt further (unnecessary) investigations and may result in establishing wrong diagnosis as well as erroneous treatment decisions.]

All articles in the issue

Related contents

Clinical Oncology

[Clinical role of multigenic prognostic tests in breast cancer therapy]


[Current clinical practice for breast cancer originates in “evidence based medicine”. In this, each tumor receives a therapy optimal for a given patient population - which might not be optimal for each individual patient. Multigenic tests determining expression of a set of genes can provide additional support in this decision process. Two such tests (MammaPrint and Prosigna) have already received FDA clearance. A number of additional test are commercially available (IHC4, Oncotype DX, EndoPredict, BCI). A common property of these assays is their utility in estrogen receptor positive early breast cancer. The main clinical problem answered by them is the necessity of adjuvant chemotherapy. To date, no reliable algorithm has been identifi ed capable to pinpoint the most effective chemotherapy combination for a given patient. Furthermore, there is no trustworthy test for triple negative breast cancer. The assays utilize different technologies (immunohistochemistry, gene chips, RT-PCR) and a discrepant list of genes - these result in discordance of the predictions for the individual patient. Despite these shortcomings, multigenic tests quickly gained foothold in breast cancer therapy decision process. Their utility is supported by the cost reduction for the health care providers by lowering the number of patients eligible for chemotherapy.]

Hypertension and nephrology

[Hungarian Vasculitis Registry – results of the first five years]

HARIS Ágnes, TISLÉR András, ONDRIK Zoltán, FILE Ibolya, MÁTYUS János, ZSARGÓ Eszter, DEÁK György, AMBRUS Csaba

[Launching the Hungarian Vasculitis Registry aimed to collect information about prevalence and outcome of our patients with ANCA-associated vasculitis, and treatment protocols of the disease. The on-line data collection has been developing dynamically since its initiation five years ago, presently 278 patients’ files are available. Patients’ mean age is 58.2±14.5 years, 62% are women; their disease is associated with c-ANCA positivity in 51% and p-ANCA in 49%. At diagnosis GFR was 24.6±21.6 ml/min/1,73 m2, that time 29%, during the total follow up 39% of the registered subjects needed dialysis. Renal replacement therapy could be discontinued in 23% of them. In cases with focal histological changes, also with upper respiratory tract and skin involvement dialysis was significantly less frequently necessary, which underlines the importance of early diagnosis. In induction therapy steroid was administered for 94% of the patients, 85% of them got cyclophosphamide, 59% was treated by plasmapheresis, 11% got rituximab. Maintenance treat ment contained steroid in 80%, per os cyclophosphamide in 23%, parenteral cyclophosphamide in 22%, furthermore 40% of the patients got azathioprin, 8 subjects got mycophenolate and 6 got methotrexate. Median follow up was 30 months (IQR 6-78), during which period 20% of the patients died, 5% got kidney transplantation, and 5% were lost to follow up. Median survival was 14.8 years, five years survival was 85%, and ten years survival was 70%. Long term survival in patients with c-ANCA vasculitis seemed better comparing to p-ANCA vasculitis, but when correcting by age this difference disappeared. Predictors of death were age and dialysis dependent renal failure. Relapses developed in 27% of patients, 28% of them presented in the first year, 21% suffered it after five years of care. Collected data by the Hungarian Vasculitis Registry shows our society’s successful professional activity. Our results are comparable to the published data in the literature, yet there are several areas in our care where further improvements are warranted in order to increase our patient’s survival and quality of life.]

Hungarian Immunology

[Long-term follow-up of a large Hungarian lupus patient population]


[INTRODUCTION - The authors give a report about their experiences obtained by long-term follow-up of a large Hungarian lupus patient population. They also compare their results with literature data. PATIENTS AND METHODS - Clinical and laboratory characteristics of 800 SLE patients followed at this institution were analysed. Issues of diagnosis, therapy and outcome measures were also discussed. RESULTS AND DISCUSSION - Results indicate that the incidence and prevalence of SLE are increasing. Recently, milder cases are also recognised. Early diagnosis makes possible an early adequate therapy. Partly due to these the survival improved, and the mortality reduced. The compliance of patients also improved. The authors suggest the importance of regular medical follow-up. Despite of that, the clinical presentations of SLE have not been changed the disease became milder. Considering the longer survival in lupus, chronic complications, such as vascular diseases, osteoporosis and cancer are suspected to appear more frequently. On the other hand, there is a small group of patients still being in risk, as they fail to respond conventional immunosuppressive therapy. These patients die within a short period due to progression of SLE. This indicates the importance of research work on the pathogenesis of SLE. Based on the results of basic research novell immune modulator modalities should be developed.]

Clinical Neuroscience

Posterior reversible encephalopathy syndrome as an initial manifestation of systemic lupus erythematosus

AYAS Özözen Zeynep, ÖCAL Öncel Ruhsen, GÜNDOGDU Aksoy Asli

Posterior reversible encephalopathy syndrome (PRES) is a disorder which is diagnosed with its characteristic clinical and radiological findings, typically resolves with treatment. The prevalence of PRES in systemic lupus erythematosus (SLE) patients is not exactly known. A systemic disorder frequently appears as a presenting symptom in SLE. However, in rare cases, the disease starts with a neurological manifestation. Here we report a 35-year-old woman presenting with a headache and blurred vision. She had neurologic symptoms and cerebral lesions on magnetic resonance imaging (MRI) suggesting PRES. The patient was diagnosed with SLE during the etiological investigation of PRES. In this article, we aimed to emphasize that PRES as an initial presentation of SLE.

Hungarian Immunology

[SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus]

SALLAI Krisztina, NAGY Eszter, GERGELY Péter

[OBJECTIVE - To assess the relation between clinical features and the presence of SS-A(Ro) and SS-B(La) autoantibodies in systemic lupus erythematosus. PATIENTS - The data of 200 patients with definite systemic lupus erythematosus were analysed. SSA( Ro) and SS-B(La) antibodies were assessed by enzyme immunoassay. RESULTS - 40.5% of systemic lupus erythematosus' patients were SS-A(Ro) and/or SS-B(La) antibody positive (’positive group’); the majority of such patients displayed both antibodies, 16.5% had SSA( Ro) antibodies alone, while only 2% has SS-B(La) antibodies alone. There were no differences in the occurrence of arthritis, secondary antiphospholipid syndrome and hematologic manifestations between the positive and negative groups; serositis was more common in the positive group. Skin manifestations, in particular subacute cutaneous lupus erythematosus and urticaria vasculitis were more frequent in the positive group, while kidney and central nervous system involvation, in particular severe forms were less frequent. Secondary Sjögren's syndrome occurred exclusively in antibody positive patients. Sm, RNP and Scl-70 antibodies were more frequently found in the positive group. CONCLUSIONS - The presence of SS-A(Ro) and/or SS-B(La) antibodies in systemic lupus erythematosus has some prognostic significance; in antibody-positive patients there is an increased risk for skin lesions (in particular subacute cutaneous lupus erythematosus and urticaria vasculitis) and secondary Sjögren’s syndrome and a decreased risk for severe nephritis or central nervous system involvement.]