management

Last edited 12/2021 and last reviewed 12/2021

The main aim of lupus treatment is to manage acute periods of potentially life threatening ill health, minimize the risk of flares during periods of relative stability and controlling the less life-threatening, but often incapacitating day to day symptoms (1).

Seek expert advice

The choice of treatment is largely dependent on the severity of the disease and the site of organ involvement (2).

  • treatment of non organ specific symptoms such as fatigue and chronic pain can be challenging since the causes are multifactorial and no specific therapies exist.
    • good  overall control of lupus may improve these symptoms to a certain extent
    • exercise programmes have been shown to be useful in fatigue without causing disease flares (3)
  • other general measures include:
    • mild disease may respond to rest, NSAID's (see notes below),
    • life style changes e.g. - avoid sitting in direct sunlight and to use physical protection from the sun (e.g. long sleeves, hats and sun-protective clothing), avoidance of oestrogens e.g. in contraceptive pill
    • topical agents e.g. - simple measures such as the use of sunscreens
    • management of co-morbidities e.g - prompt or prophylactic treatment of infection
  • management of patients without major organ involvement
    • mild - moderate disease is managed by
      • oral corticosteroids at low-moderate doses
        • for mild disease, low doses (e.g. 5-10 mg daily) are often sufficient
        • for moderate disease the dose can be increased to 0.5 mg/kg
      • antimalarial therapy
        • hydroxychloroquine (HCQ) (up to 6.5 mg/kg daily) - is useful in the treatment of mucocutaneous disease, serositis and fatigue
    • symptoms not controlled by above mentioned methods require:
      • higher-dose of steroids
        • the therapeutic aim should be to maximise benefit while minimising steroid-related side effects
      • steroid-sparing agents
        • azathioprine (AZA) (1–3 mg/kg) - commonly used,
        • methotrexate - beneficial in patients with inflammatory arthritis
        • sulfasalazine - is usually avoided (due to its association with drug induced lupus)
  • management of lupus with major organ involvement
    • the main aim in this group is rapid suppression of inflammation to prevent irreversible damage.
    • available therapeutic options include
      • high-dose intravenous (IV) methylprednisolone
      • immunosuppressant therapies - cyclophosphamide (CYC), mycophenolate mofetil (MMF)
      • biological therapies - rituximab, belimumab (6)
  • at all times, careful monitoring is required for treatment induced side effects. Associated joint and skin disease may require the addition of antimalarials e.g. chloroquine derivatives. Dialysis may be used if there is renal failure. Anticoagulants are used if the lupus anticoagulant is present
  • monitoring of levels of anti-dsDNA and treatment with steroids as soon as there is a significant rise in this marker prevents relapse in most cases, without increasing the cumulative dose of steroid given

Notes:

  • new approaches that target both immune cells and cytokine pathways important in SLE show promise as treatment targets (4):
    • B-cell depletion with rituximab treatment can improve clinical manifestations of SLE, indicating that B cells are crucial not only for the development of SLE but also for continued activity of established disease
    • anti-TNF therapy experience with infliximab (and etanercept) suggests significant benefit for rapidly reducing inflammation and possible long-term effects on proteinuria, despite the transient occurrence of autoantibodies
    • NICE have suggested that belimumab is recommended as an option as add-on treatment for active autoantibody-positive systemic lupus erythematosus in people with high disease activity despite standard treatment, only if:
      • high disease activity is defined as at least 1 serological biomarker (positive anti-double-stranded DNA or low complement) and a Safety of Estrogen in Lupus National Assessment - Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) score of greater than or equal to 10
      • treatment is continued beyond 24 weeks only if the SELENA-SLEDAI score has improved by 4 points or more
    • a systematic review concluded (7):
      • at the FDA-approved dose of 10 mg/kg, based on moderate to high-certainty data, belimumab was probably associated with a clinically meaningful efficacy benefit compared to placebo in participants with SLE at 52 weeks. Evidence related to harms is inconclusive and mostly of moderate to low-certainty evidence. More data are needed for the longer-term efficacy of belimumab
  • SLE and cardiovascular risk (4):
    • SLE should be considered a CHD-equivalent condition
    • aspirin in SLE
      • there is study evidence to suggest that the risk:benefit of low-dose aspirin would favour its use in all patients with SLE (5)
      • patients with SLE and any additional cardiovascular risk factor and patients who are positive for antiphospholipid antibodies or the lupus anticoagulant should be treated with low-dose aspirin
      • does not appear to be an additional benefit of aspirin in patients with the antiphospholipid syndrome already treated with warfarin

  • NSAID use in SLE (4):
    • all NSAIDs and selective COX-2 inhibitors can adversely affect renal function, promote fluid retention and exacerbate hypertension
    • newer selective COX-2 inhibitors have been associated with an excess of cardiovascular events which has led to the withdrawal of rofecoxib and significant changes to the labelling and use of celecoxib and etoricoxib
    • the use of non-selective (ns) NSAIDs/selective COX-2 inhibitors should be considered carefully in patients with SLE
      • continued requirement for nsNSAID/selective COX-2 inhibitor therapy in some patients may indicate that further adjustment to their disease-modifying anti-rheumatic drug (DMARD) therapy is indicated to control inflammation better
    • the nsNSAID/selective COX-2 inhibitor dose should be reviewed and the lowest effective dose should be used for the shortest period of time
    • the majority of patients on aspirin plus a NSAID will also require some form of gastroprotection

  • lupus nephritis
    • there is some evidence that oral mycophenolate may be an effective alternative to cyclophosphamide treatment in patients with lupus nephritis (2).

Reference:

Related pages:

management of lupus anticoagulant

steroid treatment in SLE

cytotoxic drugs

stem-cell transplantation in severe systemic SLE

Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)

Belimumab for systemic lupus erythematosus (SLE)