guidelines published by the British Society for Rheumatology will help
rheumatologists and other health care professionals working with patients with
systemic lupus erythematosus (SLE)in the United Kingdom to treat them
- The British
Society of Rheumatology has published guidelines for the treatment of
systemic lupus erythematosus (SLE), an
- The treatment
differs for mild, moderate and severe cases
should be kept at a minimum with hydroxychloroquine being the preferred
drug for long-term maintenance.
. An executive summary of the guidelines was published in Rheumatology
Systemic lupus erythematosus
(SLE) is an
autoimmune disease that causes skin rash and can affect multiple organs
including the kidneys, joints and the brain. The disease was in the news
recently when the singer and actress Selena Gomez underwent a kidney transplant
following failure of her kidneys due to SLE.
‘The British Society of Rheumatology has published guidelines for the treatment of systemic lupus erythematosus that can be used for patients in the United Kingdom.’
The diagnosis of SLE is
based on clinical features and is confirmed by the presence of autoantibodies
like antinuclear antibody (ANA) which is positive in around 95% cases,
anti-dsDNA antibodies, and anti-Smith (Sm) antibodies, and low complement
The treatment of SLE
depends on its severity - SLE may be mild, moderate or severe, or may occur as
flares. The following is a summary of the guidelines issued by the Royal
Society of Rheumatology for its treatment.
Mild SLE usually
involves the skin and mucous membranes, joints or the pleura (tissue covering
the lungs), but is not associated with life-threatening organ involvement. The
symptoms may include fatigue, rash on the cheeks, diffuse hair loss, mouth
ulcers, joint or muscle pain, and a platelet count of between 50 and 149 × 109
Drugs that can be used
for the treatment of mild SLE include:
- Initial treatment
can be with local corticosteroid, oral prednisolone ≤20 mg daily for 1-2 weeks, or intramuscular or intraarticular (injection
into the joint) injection of methyl-prednisolone in a dose ranging from 80
- In addition, the
disease-modifying drugs hydroxychloroquine and /or methotrexate
and/or short courses of NSAIDs (non-steroidal painkillers like ibuprofen
and diclofenac) may be used.
- Low dose
prednisolone (less than or equal to 7.5 mg/day), hydroxychloroquine and/or
methotrexate may be used for maintenance treatment. Corticosteroids may be
used locally on the skin or can be injected into the joints for arthritis.
Protection Factor (SPF) sunscreen along with avoiding sunlight is advised
to prevent skin lesions.
Moderate SLE is
characterized by fever, rash up to 2/9 body surface area, cutaneous vasculitis
(inflammation of the blood vessels of the skin), hair loss with scalp
inflammation, liver or joint disease, inflammation of the pleura or pericardium
(the tissues surrounding the lungs and the heart, respectively) and a platelet
count of 25-49 × 109
/l. Moderate SLE requires the following drugs:
- Corticosteroids in the form of higher doses of prednisolone (up to 0.5 mg/kg/day), or the use
of intramuscular or intravenous injections of methylprednisolone.
- Methotrexate, azathioprine, mycophenolate mofetil,
ciclosporin or other calcineurin inhibitors may be used in addition to
hydroxychloroquine for patients with skin or joint disease, vasculitis or low
For cases that do not respond to the above treatments,
belimumab or rituximab may be tried out.
- Maintenance treatment is with prednisolone,
hydroxychloroquine, and azathioprine, methotrexate, mycophenolate mofetil or
Severe SLE is a
life-threatening condition that can affect vital organs including the kidneys
and the brain. The patient may present with rash involving more than 2/9 body
surface area, muscle pain, severe inflammation of the pleura and/or pericardium
with fluid accumulation, fluid accumulation in the abdomen, involvement of the
intestines, brain, spinal cord, and optic nerve, and an extremely low platelet
count of less than 25 × 109
/l. The presence of infection should be
ruled out in these patients.
methylprednisolone or high-dose oral prednisolone (up to 1 mg/kg/day) may
be required as initial treatment.
drugs that may be used include mycophenolate mofetil and ciclosporin for
lupus nephritis and refractory severe non-kidney disease in addition to
the corticosteroid treatment in addition to
- The biologic
therapies belimumab or rituximab may be used for patients who have failed
to respond to the immunosuppressive drugs or cannot use them due to side
immunoglobulin, plasmapheresis (removal of antibodies from the plasma) may
be required for patients with low blood counts not responding to
treatment, thrombotic thrombocytopenic purpura (due to low platelet
levels), rapidly deteriorating acute confusional state and the
catastrophic variant of APS (antiphospholipid syndrome).
may be needed for patients with thrombotic manifestations
treatment is with prednisolone and azathioprine, mycophenolate mofetil or
cyclosporine, along with hydroxychloroquine.
The treatment for flares
depends on the severity of the underlying disease. Most drugs except
hydroxychloroquine can be eventually stopped once the patient achieves stable
Gordon C et al. The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults: Executive Summary. Rheumatology, Volume 57, Issue 1, 1 January 2018, Pages 14-18, https://doi.org/10.1093/rheumatology/kex291