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Management of Systemic Lupus Erythematosus

In a retrospective cohort study of renal biopsy-proven thrombotic
microangiopathy LN, plasmapheresis treatment had higher rate of
complete/partial remission compared with control (non-plasmapheresis)
(77.8 vs 11.1%, p=0.018). No AEs were observed. 77, level II-2

In a pre-post study of refractory SLE patients with sub-phenotypes of
thrombotic thrombocytopenic purpura (TTP), myasthenia gravis and
APS, PE as add-on treatment to corticosteroids and immunosuppressive
agents significantly decreased SLEDAI. However, 21% patients
experienced PE-related major AEs (catheter infections, bleeding and
hypotension). 78, level II-3

The BSR 2018 guidelines recommends the use of PE in SLE with
21
TTP. Meanwhile in the 2020 Chinese Guidelines for the Diagnosis
and Treatment of Systemic Lupus Erythematosus, PE can be
considered in patients with severe or refractory SLE. 67

• PE is considered in patients with severe or refractory SLE, in
particular those with TTP.

ii) Intravenous immunoglobulin
Intravenous immunoglobulin (IVIG) is a blood product derived from
the plasma of a large pool of healthy donors. The indication of its use
as immunomodulator has expanded to treat various autoimmune and
inflammatory diseases. There is limited evidence on IVIG treatment in
SLE.

In a non-randomised controlled trial in LN patients, IVIG was not
significantly different to CYC or AZA in achieving partial or complete
renal remission following induction therapy. However, it had lower
infection rate. No leukopenia, amenorrhoea or osteoporosis were
observed. 79, level II-1

• In SLE, the use of IVIG maybe considered in: 21; 50; 67
 severe refractory SLE including haematological flare, TTP and the
catastrophic variant of APS
 active SLE with concomitant infection

Thromboembolic events are delayed AEs of immunoglobulin treatment
with incidence rate up to 2% when given in high dose. 80, level III It
occurs because of hyperviscosity especially in patients having risk
factors including advanced age, previous thromboembolic diseases,
bedridden, diabetes mellitus, hypertension, dyslipidaemia and those

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