The majority of Swedish systemic lupus erythematosus patients are still affected by irreversible organ impairment: factors related to damage accrual in two regional cohorts

Abstract

Background Although the survival of patients with systemic lupus erythematosus (SLE) has improved, irreversible organ damage remains a critical concern. We aimed to characterize damage accrual and its clinical associations and causes of death in Swedish patients. Methods Accumulation of damage was evaluated in 543 consecutively recruited and well-characterized cases during 1998−2017. The Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI) was used to estimate damage. Results Organ damage (SDI ≥ 1) was observed in 59%, and extensive damage (SDI ≥ 3) in 25% of cases. SDI ≥ 1 was significantly associated with higher age at onset, SLE duration, the number of fulfilled SLICC criteria, neurologic disorder, antiphospholipid antibody syndrome (APS), hypertension, hyperlipidemia, depression and secondary Sjögren's syndrome (SS). In addition, SDI ≥ 3 was associated with serositis, renal and haematological disorders and interstitial lung disease. A multiple regression model identified not only well-known risk factors like APS, antihypertensives and corticosteroids, but pericarditis, haemolytic anaemia, lymphopenia and myositis as being linked to SDI. Malignancy, infection and cardiovascular disease were the leading causes of death. Conclusions After a mean SLE duration of 17 years, the majority of today's Swedish SLE patients have accrued damage. We confirm previous observations and report some novel findings regarding disease phenotypes and damage accrual.

Prediction of Damage Accrual in Systemic Lupus Erythematosus Using the Systemic Lupus International Collaborating Clinics Frailty Index

“…Second, our analysis assumed a constant rate of damage accrual throughout the follow‐up period and thus could not account for potential accelerations or decelerations in the average rate of change in SDI score over time. However, consistent with the results of previous studies conducted in a variety of different health care systems , we found a steady, linear rate of increase in mean SDI score during follow‐up, suggesting that our assumption about the constant rate of damage accrual among SLE patients is valid. Third, 277 patients (15.2% of the SLICC cohort) were excluded due to missing baseline or follow‐up data.…”

Systemic Lupus Erythematosus (SLE) Therapy: The Old and the New

“…For example, in lupus nephritis (LN), nearly 10% of the patients progress towards end-stage kidney disease. Persistent disease activity, comorbidities and drug toxicity significantly contribute to the risk of progressive irreversible damage accrual and increased mortality [ 5 , 6 ].…”

High genetic risk score is associated with early disease onset, damage accrual and decreased survival in systemic lupus erythematosus

“…Despite improved prognosis, the mortality rate stills exceeds that of the general population 4. Due to active inflammation, prolonged corticosteroid use, comorbidities and factors unrelated to SLE, organ damage accumulates in the majority of patients over time,1 5 6 with cardiovascular disease and renal failure being strong risk factors for premature mortality 4 7–9…”