Serum β2-microglobulin level is a useful indicator of disease activity and hemophagocytic syndrome complication in systemic lupus erythematosus and adult-onset Still’s disease

Abstract

This study demonstrates whether serum β2-microglobulin (β2-MG) level can be an indicator of the status of systemic lupus erythematosus (SLE) and adult-onset Still's disease (AOSD), and development of hemophagocytic syndrome (HPS) complication. Serum β2-MG level was compared between the active and inactive statuses of SLE and AOSD in hospitalized patients. Active status was defined as a state for which a therapy was introduced. Serum β2-MG level was also compared between patients with and without HPS complication. HPS was diagnosed on the basis of clinical and pathological findings. Laboratory markers of HPS including peripheral blood cell counts and levels of serum lactate dehydrogenase (LDH), serum ferritin, plasma fibrin/fibrinogen degradation product (FDP), and plasma D-dimer were examined to determine their correlations with serum β2-MG level. Sixteen SLE and seven AOSD patients (all females, aged 39.0 ± 16.4) were included. The serum β2-MG level was high in the active status of underlying diseases and decreased significantly after the therapy (3.5 ± 1.4 vs. 2.1 ± 0.8 mg/L, p < 0.001). Among patients with active status, the β2-MG level was higher in patients with HPS (two with SLE and three with AOSD) than in patients without HPS (4.9 ± 1.8 vs. 3.3 ± 1.4 mg/L, p < 0.05). Serum β2-MG level significantly correlated with the levels of serum LDH (r(s) = 0.42, p < 0.05), plasma FDP (r(s) = 0.58, p < 0.05), and plasma D-dimer (r(s) = 0.77, p < 0.01). Serum β2-MG level would be a useful indicator of disease activity and development of HPS complication in patients with SLE and AOSD.

Rediscovering Beta-2 Microglobulin As a Biomarker across the Spectrum of Kidney Diseases

“…During normal cell turn over, it is released into the body fluids. Pathologies with high cell turnover, such as hemato-oncological conditions, and rheumatologic diseases are associated with higher serum β 2 M levels (268, 269). As many of these conditions may be associated with the subsequent development of CKD (270–272), we will briefly review some of these associations.…”

“…Higher serum levels are seen in patients with systemic lupus erythematosus and adult-onset Still disease, especially in those with active diseases and hemophagocytic syndrome (268, 301). After therapy, the serum β 2 M level decreased significantly (268). Urinary β 2 M has been shown to correlate with overall and renal disease activity scores and proteinuria (164).…”

Regulatory effects of four ginsenoside monomers in humoral immunity of systemic lupus erythematosus

“…4B) increased after addition of LPS, while this increase was inhibited in the presence of the GS monomers. As β2M levels are a useful indicator of disease activity in SLE (19,20), RT-qPCR was used to detect the mRNA expression of β2M, revealing that GS monomers caused a significant reduction of the LPS-stimulated β2M expression (Fig. 4C).…”

Urine β2-microglobulin is associated with clinical disease activity and renal involvement in female patients with systemic lupus erythematosus

“…Lupus activity was classified on the basis of Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores: no activity (SLEDAI ¼ 0), mild activity (SLEDAI ¼ 1-5), moderate activity (SLEDAI ¼ 6-10), high activity (SLEDAI ¼ [11][12][13][14][15][16][17][18][19], and very high activity (SLEDAI ! 20).…”