Systemic lupus erythematosus. Disease outcome in patients with a disease duration of at least 10 years: second evaluation

Abstract

Data related to the disease course of patients with systemic lupus erythematosus (SLE) with special attention to the persistence of disease activity in the long term are scarce. At this moment reliable figures are only known about the survival rate as a measure of outcome. The aim of this multicenter study was to describe the outcome of SLE patients with a disease duration of greater than 10 y. Outcome parameters were two disease activity-scoring systems (SLEDAI and ECLAM), the end organ damage (SLICC/ACR damage index) and treatment. Our results are derived from 187 SLE patients followed at 10 different centres in Europe over a period of 1 y. Serious clinical signs or exacerbations, defined by the occurrence or detoriation of already existing symptoms of renal and cerebral nervous systems were observed in 2-11% of the patients, seizures and psychosis in 3%, proteinuria in 11% and an increase in serum creatinine in 5% of the patients. No change took place in the overall damage index. Yet, the disease course in most patients was characterized by periods of tiredness (42-60%), arthritis (20-25%), skin involvement such as malar rash (32-40%), migraine (15-20%), anaemia (15%) and leucopenia (17-19%). Summarizing these results it is shown that patients, still under care after such a long time of having this disease, do have a disease that is far from extinguished.

Disease activity and damage accrual during the early disease course in a multinational inception cohort of patients with systemic lupus erythematosus

“…Patient characteristics (89% female, age 35 years, diagnostic delay 28 months) corresponded well with those in similarly designed (observational multicentre cohort) studies. 8,9,14,[22][23][24] Similarly, the clinical burden of disease at diagnosis (6.5 ACR criteria, median SLEDAI > 10) was comparable to that seen in the Caucasian LUMINA patient cohort (n ¼ 71) (six ACR criteria and Systemic Lupus Activity Measure (SLAM) 8.5). Disease activity and damage accrual during the early disease course J Nossent et al…”

“…This is in accordance with findings that disease flares remain a clinical challenge even after 15-20 years of disease. 9,14 The overall flare rate of 23 per 100 patient years is at the lower end of reported flare rates, 37,38 suggesting that both flare frequency and severity may be declining. Not achieving an early disease remission was associated with higer relapse rates and higher subsequent SLEDAI scores, indicating that the best window of therapeutic opportunity is in the first year of disease.…”

“…Also, different approaches to diagnosis and management cannot be wholly excluded; however, all centres have a long-standing research involvement in similar collaborative efforts. 8,9,14 The collection of annual summary data may have underestimated disease activity, as patients with minor flares, other complications and related changes in medical treatment may not all have been referred to the study centre. Finally, the limited follow-up naturally precludes longerterm predictions.…”

“…Using an existing infrastructure for SLE-related research, 8,9,14 14 rheumatology centres agreed in 1999 to assemble data on disease presentation in new onset SLE patients seen over a period of 5 years and provide follow-up data. After a core data set was defined, case record forms were developed and distributed.…”

Anämie bei entzündlich-rheumatischen Erkrankungen – Begleitsymptom oder behandlungsbedürftige Manifestation?

“…Die H ä ufi gkeit und Auspr ä gung der An ä -mie variiert bei dieser Erkrankung sehr stark in Abh ä ngigkeit vom untersuchten Patientengut. Nach einem Krankheitsverlauf von mehr als zehn Jahren waren in einer gro ß en europ ä ischen SLE-Kohorte lediglich 15 % an ä misch [34] , w ä hrend in einer hochselektierten Kohorte mit Vorliegen eines speziellen Fc γ -Rezeptor-Polymorphismus 84 % der Patienten eine An ä mie hatten [19] . In der amerikanischen LUMINA-Kohorte hingegen (longitudinale Untersuchung von 613 SLE-Patienten mit ≤ 5 Jahren Krankheitsdauer) waren bei Studieneinschluss 37,6 % der Patienten an ä misch.…”