Treatment of SLE (Systemic Lupus Erythematosus) 3.5/5 (4)

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Treatment of lupus

Treatment of Lupus usually lasts for at least 2-3 years after the disease has come under control

Definition

Choice of medicines for Lupus / SLE depends on how the disease attacks the individual organs. Treatment of SLE is a specialist task.

Before treatment starts

It is important to be well informed of the disease, the treatment goal, and about side effects that may occur

  • Infection risk increases during treatment
    • Other causes of reduced immune system are mapped (history of blood and white blood cell count, immunoglobulin / IgG)
    • Vaccines for protection against flu and pneumococci (pneumonia) are relevant
    • Latent infections like Tuberculosis (Tbc), hepatitis B og HIV excluded by blood tests and measures against Opportunistic infections may be necessary
  • Pregnancy Wish must be taken into account when choosing medications
  • Disease activity and possible organ damage are recorded for subsequent comparison in order to assess the effect of treatment
  • Other risk factors considered

Duration of treatment

Duration of treatment by SLE is dependent on the course of the disease in each individual

It is controversial when other disease modifying treatment should be terminated

  • Some recommend quitting (discontinuing) 2-3 years after the illness has completely subsided
    • Choosing a longer duration of treatment reduces the risk of recurrence of the disease, but the immunosuppressive treatment burdens the body over a longer period of time, which increases the risk of infections.
    • Hydroxychlorokine (Plaquenil) is usually kept longer

Treatment of kidney manifestation by SLE, please read here

Corticosteroids (Cortisone)

  • Methyl prednisolone (SoluMedrol) is given intravenously if necessary (250-500mg / day) for up to three consecutive days in the event of threatening organ damage such as visual disturbance or other symptoms of critical blood circulation.
    • ECG and measurement of potassium and calcium in the blood should be done ahead of time, since heart-rhythm disorders can occur
  • Prednisone most often at high disease activity is given from the onset of disease (often 0,5mg / kg / day) or after methyl-prednisolone (see above). The dose is gradually reduced.
    • Over time, the dose should be reduced to a minimum and preferably terminated completely

Hydroxychlorine (Plaquenil)

Hydroxychloroquine reduces the risk of recurrence of the disease

  • The drug has a beneficial effect on the outlook (prognosis)
  • May reduce blood clot risk (Thromboembolism)
  • Can be used during pregnancy and lactation
  • The usual dose is 200 mg tablets 1-2 times a day, depending on body weight. In the long term, the dose should be below 5mg / kg / day. Lower dose in reduced kidney function
  • A rheumatologist should start and end the treatment
  • Ophthalmic surgery is important during treatment because of the risk of eye damage (retinopathy) at high total dose over time
    • After 5 years of use, ophthalmologist is recommended at least once a year
    • After any 20 years of use of Plaquenil at the usual dose, the risk of eye complications is over 10%
    • An increased risk of eye side-effect is present if retina or macular disease is present in advance
    • The ophthalmologist should include Electro-retinography (ERG) or optical coherence tomography (OCT) (reference: Marmor_MF, 2016)
  • Keratitis
    • Inflammation of cornea / cornea) with pain and eye discomfort, is possible in the initial phase
  • Should be avoided by Psoriasis (may cause more eczema) and kidney failure (danger of overdose)
  • Avoid direct sunlight (can trigger pigment changes and sunburn)
  • Please read more about Plaquenil here

Sendoxan (Cyclophosphamide)

  • Intravenous treatment or more rarely as tablets. Used for kidney inflammation in SLE (Glomerulonephritis, WHO class IV-V or severe class III), severe serositis or lupus in the CNS (brain or spinal cord)
  • After a few months (most often 3-6 months) treatment with Sendoxan one goes to another immunosuppressive treatment with azathioprine (Imurel), mycophenolate (CellCept, Myfortic, Myfenax) or methotrexate (reference: Morris HK, 2013)
  • Sendoxan treatment is described in more detail here

Azathioprine (Azathioprine)

Imurel is usually dosed 2mg / kg / day, in one or two doses daily

  • Checked in advance TPMTgenotype in blood sample since reduced tolerance occurs in certain genotypes
  • The correct dose is measured by 6-TGN in blood sample (reference values: 2,0 - 6,0). Some respond quickly to azathioprine with "drug fever" or DRESS syndrome, leukocytopenia (low white blood cell count, most often neutrophil leukocytes) or high liver enzymes and must stop treatment. Leukocytopenia occurs at too high a dose and may be difficult to distinguish from SLE activity (which may also cause leukocytopenia)
  • Imurel can be used during pregnancy. Doses in excess of 100mg / day should be avoided during breastfeeding, although no damage has been demonstrated because the drug passes into breast milk.
  • More about azathioprine here

Mycophenolate Mofetil (CellCept, Myfenax, Myfortic)

Tablets that have a good effect on renal manifestations and in some cases are used as induction treatment instead of Sendoxan or as maintenance treatment

Methotrexate (Ebetrex, Metex, Methorexate)

Methotrexate is an alternative to azathioprine for the treatment of SLE

  • Often chosen for joint inflammation (Arthritis)
  • The dose is 10-25mg /week, given as a weekly dose in the form of tablets or injection (the same dosage as for arthritis (Rheumatoid arthritis, RA)
  • Supplemented with Folic acid
  • Caution in renal impairment
  • Not used in known liver disease (check Hepatitisantibody)
  • If pregnancy Methotrexate should not be used in the coming months. The drug must be discontinued at least three months before pregnancy
  • Regular blood tests (Hemoglobin, Leukocytes, Thrombocytes, ALT, Creatinine) are recommended
  • Please read more about Metotreksat here

Belimumab (Benlysta)

Benlysta is an approved Biological medicine for the treatment of severe SLE

  • It is given as "add on" therapy when the disease activity is difficult to control
  • Approximately one in three has effect and one in three has to end because of intolerance / side effects
  • Dosed day 0 - 14 - XNUM, then every 28. week
  • Infusions (10mg / kg) are given over one hour in hospitals, initially (2 first doses) often on bed post, then daytime or outpatient clinic. The patient is observed one hour after the end of the infusion
  • Mechanism of action:
    • Benlysta is a fully human immunoglobulin that binds soluble B-lymphocyte stimulator (BLyS / BAFF) in the immune system. Thus, hyperactive, aggressive CD20 + B lymphocytes can go into apoptosis
  • Please read more about Belimumab here

Rituals (MabThera /Rituxan)

Rituximab is Biological drug and an alternative to established treatment in the event of serious illness.

Rituximab is used for antibody-dependent low platelet counts (thrombocytopenia) or severe spinal cord attack (myelitis). Some have an effect, while others do not (reference: Yusof Md, 2017)

  • Before treatment, measurement of Lymphocyte sub-populations (CD19 + B cells), hepatitis-B antibodies and immunoglobulins in the blood may be relevant
  • Dosage most often as in arthritis (Rheumatoid arthritis) (initial 1000mg iv twice at two week intervals), or "lymphoma protocol": 4 infusions, each dose is 375mg / m2 body surface = approx. 600mg at 1 week intervals
  • Treatment pause with azathioprine (Imurel), mycophenolate (CellCept), methotrexate or similar is considered individually
  • Upon follow-up
    • Immunoglobulins (IgG) are checked in blood samples
    • CD19 + B cells remain suppressed for ca. 12 months, but need not be measured regularly
    • CNS side effect (nervous system): progressive multifocal leucoencephalopathy (PML) is observed, but unclear relationship with ritual simab. Due to reactivation of latent JC polyoma virus
  • Many sheep pneumocystisprophylaxis with Bactrim (1X tablet daily or two tablets two to three days weekly)
  • Consider vaccines against pneumococci and influenza
  • Hepatitis B vaccine whose risk of infection

Please read more about ritual abortions here

ACE inhibitors or AT-II antagonists

High blood pressure or proteinuria is often supplemented with enalapril (Renitec), Capropril or other ACE inhibitors or AT-II inhibitors (check the risk of adverse reactions and www.interaksjoner.no ).

  • ACE inhibitors are contraindicated / should not be used pregnancy
    • Trandate applicable to high blood pressure in pregnancy

Statins

There is generally an increased risk of atherosclerosis at SLE.

  • Prevention with cholesterol-lowering statins should be considered individually

Smoking

It has been shown that smoking causes poorer medical prognosis / living prospects at SLE

Vaccines

Dead vaccines ”

  • Against influenza and pneumococci (pneumonia) are "dead" vaccines and unproblematic to give. These are often recommended before beginning immunosuppressive therapy, especially in Sendoxan or MabThera / Rixarthon.
  • Hepatitis B vaccine: experience is lacking
  • HPV. With SLE, increased cervical cancer (cervical uterus) is suspected, which may be due to HPV (human papilloma) virus. HPV vaccine can be assessed
  • "Live Vaccines" are discouraged by immunosuppressive treatment
  • However, some cases of outbreak of Lupus following vaccination have been reported
  • Please read more about vaccines and rheumatic disease here

Birth control pills

"IUS" Hormon spiral

  • Gestagen coil (eg, Mirena) can be used for SLE
  • Hormonal vaginal ring (Nuvaring) is not recommended due to increased disease and thrombosis risk

Stem cell / bone marrow transplantation

In very severe cases of SLE, autologous stem cell therapy has been attempted as treatment for SLE. The method is comparable to Stem cell therapy for systemic sclerosis (scleroderma).

  • The results have not been particularly good at Lupus. Significant problems are relapse of the disease in many cases, and deaths related to treatment have been reported (reference: Illei GG, 2011)
  • Transplant of mesenchymal stem Cells (MSC) is an opportunity that is being tested (reference: Collin SE, 2013)

Link to Medicines, BINDEVEVSYSKDOMMER.no

Link to pregnancy, BINDEVEVSSYKDOMMER.no


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