- 1 Definition
- 2 Disease Cause
- 3 Occurrence
- 4 Symptoms
- 5 Examinations
- 6 Diagnosis
- 7 Similar conditions, differential diagnoses
- 8 Pregnancy / pregnancy in SLE
- 9 Diet by SLE
- 10 Ten tips for living better with lupus
- 11 Sun, solarium and lupus / SLE
- 12 Treatment
- 13 Useful links for more information
- 14 Guidelines and recommendations
- 15 Literature
Systemic lupus erythematosus (SLE, "lupus") is a typical rheumatic connective tissue diseases where the own immune system becomes too active and by mistake can attack almost any of the body's own organs (autoimmune disease). The disease most often starts among young adults and older children (juvenile lupus), and over 90% are women. Feeling sick, rashes and joint inflammation (Arthritis) are typical symptoms. Blood tests show signs of significant immunological disturbances, and the production of rheumatism tests/antibodies is evident in blood tests (reference: Fava A, 2019). Lupus occurs in several different types: Systemic lupus erythematosus (SLE) is the most widespread disease and is the most discussed in these pages. Skin Lupus attacks the skin with various forms of eczema, but internal organs. Drug-induced Lupus. due to bivrfication of a drug and Neonatal Lupus can occur in newborns, but passes on its own within a few weeks-months. New drugs mean that the prognosis for SLE has improved significantly in recent years.
The cause of Lupus is unknown, but once the disease has started, the immune system becomes overactive. The immune system then attacks the body's own organs by mistake. A rheumatic inflammation occurs in one or more organs. In this way, SLE can involve the skin, joints, kidneys, heart, lungs, nervous system and blood cells (number of red and white blood cells, as well as platelets). SLE is thus a typical Autoimmune disease. Hereditary cases do occur, but it is still rare for several close relatives to have SLE. Even among identical twins (they are genetically identical), there is only a 20-40% risk that both will get SLE. Among siblings, the incidence is calculated at 2%, which means that 98% will not get the disease even if a sister or brother is ill. The heritability from mother to child is correspondingly low.
A Norwegian study shows that approx. 150 people become ill with lupus annually in Norway (incidence). SLE is approximately ten times more common among women than men, and we have approximately 2.600 women with SLE in Norway (prevalence) (reference: Lerang K, 2012). The 25-34 age group is particularly vulnerable. SLE rarely begins after the age of 50. The proportion of SLE that occurs before adulthood is 20-30%, then most often in the teenage years, but rare genetic variants can begin earlier. The incidence is higher among people originally from non-European countries, particularly Asia and Africa. Black Americans are approx. twice as frequently attacked as Caucasians, and Asians have approx. 30% increased risk compared to Caucasians (reference: Somers EC, 2014). These exposed ethnicities generally have an earlier disease onset and a more severe course (reference: Lim SS, Arthritis Rheum 2009). SLE in children (juvenile lupus) is described on a separate page.
SLE is characterized by individual, different disease courses. Damage to the skin, mucous membranes, joints, internal organs, blood and nervous system occurs to varying degrees, but damage to organs accumulates over time if the disease is not stopped (reference Dörner T, 2019).
- Chest pain: Pneumonia (pleurisy), inflammation of the pericardium (pericarditis).
- Eczema, either on the body, on the face (cheeks) or on the fingers.
- Hair loss (alopecia)
- Mouth ulcers
- Night sweats
- Decreased appetite
- Joint pain and joint swelling (arthritis)
- Weight Loss
Later and more serious symptoms include:
- Inflammation of the pericardium and pleura; Pericarditis and pericardial fluid, pleureitis/pleural fluid.
- Bleeding due to low platelet count (thrombocytopenia)
- Paleness and lethargy due to a low number of red blood cells (anaemia)
- Infections due to low white blood cell count (leukopenia)
- Renal inflammation (glomerulonephritis)
- Pulmonary hemorrhage (diffuse alveolar), (rarely)
- Neuropsychiatric symptoms (psychosis, epileptic seizures)
- Skeletal damage where bone tissue dies (necrosis)
Exhaustion/Fatigue is common in SLE is fatigue. The degree of fatigue is independent of disease activity and treatment, but may be partially related to pain, depression and psychosocial conditions.
Medical history should be comprehensive and attempt to register all relevant symptoms (see above). Central is the content of the classification criteria (see below), which can be a good starting point. One can map signs of hair loss, new mouth ulcers, eczema (location and spread), symptoms from the lungs, heart, joints and nervous system. Swelling (edema) in the legs or in the face is often a sign of kidney inflammation. History of blood clots (thromboembolism), miscarriages, stillbirths and recurrent fevers is also requested.
Clinical a general examination is carried out which may include hair, mouth, skin, joints, heart, lungs, blood pressure and neurological assessment for relevant symptoms. In active SLE, swollen lymph nodes are common (reactive lymphadenopathy).
Blood tests; Routine testing may include CRP, lowering reaction (SR), hemoglobin, white blood cells (leukocytes with differential counts), platelets (thrombocytes), electrolytes, IgG, glucose, liver, kidney and thyroid function tests, CK, albumin, as well antibody (see more below) ANA, anti-DNA, complement C3, C4 and urin stick.
- Anemia (low hemoglobin/blood percentage) is common and most often for several reasons. Can be caused by iron deficiency, inflammation, hemolysis (increased bilirubin, reticulocytes and LD, as well as decreased haptoglobin. - Leukopenia (low number of white blood cells) may appear as isolated lymphocytopenia and/or granulocytopenia (signs of activity). - Thrombocytopenia (few platelets) (< 100.000 x 109/L). Thrombocytopenia in SLE is common, but bleeding is rare. There are two types of thrombocytopenia; One that follows disease activity and will often require advanced treatment. The second type is moderate and there is often no need for treatment. Pancytopenia (low numbers of red and white blood cells and a lack of platelets) can occur in SLE, but it is unusual for all cell lines to have low numbers. Evans syndrome (Autohemolysis + thrombocytopenia). (reference: Velo-Garcia A, 2016). -Reduction reaction (SR): elevated in 90% with active disease. -Polyclonal hyper-gammaglobulinaemia detected by serum electrophoresis. High level of gammaglobulinemia correlates with SR but not with C-reactive protein (CRP): usually normal if not infection. SLE-related active peripheral arthritis or serositis (pleuritis or pericarditis) may nevertheless increase SR. -Complement may be reduced (CH50, C3 and C4) with increased amounts of split products (C3d and c-activation product) and TCC (terminal complement complex), southern in active disease.
Immunological investigations: -ANA is detectable in over 90% (high sensitivity), but is seen in a number of other conditions and among healthy people (low specificity). -Anti-ds (native) DNA: in high levels, especially if both the ELAISA and IF tests are "positive". SLE without anti-ds DNA rarely has nephritis. –Other antibodies in SLE: anti-Sm (Smith) (sensitivity 10%, but high specificity), anti-RNP (also consider MCTD), anti-SSA/SSB (consider subacute cutaneous lupus og Sjøgren's syndrome). Rheumatoid Factors (RF): If also positive anti-CCP consider “rhupus” (overlap against Arthritis, RA). –Antiphospholipid antibodies (Lupus anticoagulant, Anti-cardiolipin, anti-(beta-2-glycoprotein) is seen in 30%: In the event of blood clots or miscarriages, Antifosfolipid syndrome (ApLs) available.
Urine sticks; should always be done to rule out signs of new inflammation (glomerulonephritis).
Imaging. -Joints, skeleton and tendons: Ultrasound, MRI, X-ray or CT. -Lungs: X-ray or CT. -The heart: Ultrasound/echocardiography.
Heart Inflammation of the heart bag (Pericarditis) is the most common cardiac complication (in 11-55%). A typical symptom is chest pain that improves when sitting or standing, worsens when lying down. Damage to the heart valves (Liebmann-Sachs endocarditis) are rarer, but can attack both the inner part of the heart (endocardium) and heart valves (most commonly mitral valves) in 10-30%. Endocarditis is diagnosed by ECG, ultrasound/echocardiography: trans-thoracic 10%, trans-oesophageal 30% and MRI examinations. Cardiac enzymes (troponins, CKMB) are usually normal. The heart muscle may become inflamed (myocarditis) in 10-15%. Symptoms of myocarditis are heart failure. In the blood, troponin and creatine kinase (CK, CKMB) are elevated. Pulmonary hypertension is a rare complication with elevated pressure in the pulmonary circulation, which in the long run can also lead to heart failure. In the longer term, the risk of developing cardiovascular disease is due to artherosclerosis approximately twice as large in SLE compared to the general population (reference: Schoenfeldt SR, 2012). One can feel chest pain when straining (angina pectoris) (reference; Miner JJ, 2014).
Skin and mucous membranes. Rash either on the cheek and above the nose ("Butterflies Eczema "). Psoriasis-like scaly rash can be seen on the scalp with patchy hair loss or on the body in the form of discoid lupus. Skin symptoms are common and are seen in 20% at onset, 50-70% later in the course (reference: Sontheimer RD 1996). Sun rash: Rash on sun-exposed areas is typical. The rash can have a different shape and intensity and occurs in approx. 60% with SLE. Blonde, blue-eyed people have little pigment in their skin and are most exposed. The antibody SSA (Ro) is a predisposing factor. Chilblain Eczema are red-blue changes on the hands and occur in 20% of people with SLE. Lupus profundus is a form of fatty tissue inflammation (Panniculitis) under the skin. Lupus tumidus is red eczema (erythematous plaques), most often on the body, less often on the arms and legs. Lupus Pernio is not related to Lupus, but is a form of Sarcoidosis in the skin. Mouth ulcers: In the mouth, mucous membranes can be attacked in 25-45%, so that mouth ulcers occur. The ulcers can be very different in size, location and shape. Lips, gums, the inside of the cheeks and palate can be attacked. Similar conditionsr that are not associated with lupus are atopic eczema, flushing, contact dermatitis, roseacea og seborrhea.
Joint. Joint pain, often in the fingers, which can also swell (Arthritis). Occurrence: 60-100%, joint and tendon inflammation (arthritis-tendinitis) is common (70-80%). Jaccoud arthritis causes crooked joints without X-rays showing skeletal damage (not erosive) the incidence is approx.5%. Skeletal damage (erosive arthritis) may resemble RA (rheumatoid arthritis) and attacks approx. 5%.
Liver disease. Liver disease is not among the most common complications of SLE, but elevated liver enzymes (in blood tests) can be due to Lupus inflammation ("Lupus hepatitis") or overlap with autoimmune hepatitis which occurs more frequently in SLE than in the general population. Azathioprine, Methotrexate and drugs can cause side effects from the liver. A latent ("sleeping") infection with Hepatitis B virus can flare up during immunosuppressive SLE treatment.
Lungs can be attacked in the form of inflammation of the pleura (pleurisy). Symptoms are breathing-dependent pain, usually at the same time as other signs of disease activity. Blood tests may show elevated CRP, which is otherwise not common in SLE, and an increased blood-lowering reaction. Lung infections (bacterial pneumonia) occur more frequently than in the general population. Please see Opportunistic infections. Interstitial Lung Disease (FIRE) and pneumonitis (rheumatic inflammation/inflammation, not infection) is the soul. Pulmonary Hypertension (PAH) is rare and can be caused by combinations of heart valve failure, blood clot/lungembolism, especially at the same time Antifosfolipid syndrome and in case of interstitial lung disease (reference: Parabu A, 2009). Pulmonary haemorrhage / kapillaritt is also rare (2-4%) (reference: Ednalino C, 2015). "Shrinking lungs" can be seen in up to 10% and cause the diaphragm to be high (diaphragm dysfunction, myopathy), but are often without symptoms. Drug side effects in the lungs are rare: Cyclophosphamide (Sendoxane), methotrexate, TNF inhibitor (Literature: Allen D, 2012).
Mouth ulcers occurs as part of the disease in SLE. The sores do not always follow the activity of the lupus disease. You will find a separate page about mouth ulcers in general here .
Nervous System. The brain and spinal cord can be attacked in the form of so-called "brain lupus" (CNS lupus). One detects changes in various tests / examinations. Most neuropsychiatric manifestations occur early in the course of SLE. However, several of the symptoms are so common that in SLE they can only be related to the disease in approximately 30% of cases (Hanly JG, 2020). In CNS lupus, antibodies are often detected in a blood test and in spinal fluid (spinal fluid): Anti-ribosomal P og Antifosfolipid antibody is associated with neuropsychiatric SLE. MRI of the brain recommended in case of suspicion of obvious neurological changes, convulsions, chronic cognitive impairment and at Antifosfolipid syndrome (reference: Sarbu N, 2015). However, MR changes are not easy to interpret (Reference: Kim KW, 2008) and MRI is normal at approx. 50%, even if neuropsychiatric lupus is present. Electro encephalogram (EEG); about. 80% with active CNS lupus have pathological changes (slow waves, focal changes). Fatigue, which is common in SLE, is not defined as CNS lupus. Devic's syndrome consists of optic neuritis and transverse myelitis. –Sinus vein thrombosis seen by Antifosfolipid syndrome and causes forehead headache, vision changes, neurological symptoms, possibly epilepsy. The diagnosis is made by cerebral MRI combined with venous MR angiography. Transverse myelitis is a very serious complication in the spinal cord that quickly causes paralysis. You can find more about SLE in the central nervous system in a separate chapter.
Kidneys. The kidneys are often attacked in SLE, especially among those who have high levels of anti-DNA in the blood. One problem is that kidney inflammation is often not noticed until the legs swell. The cause of such swelling is the accumulation of "water" (edema). The swelling in the legs occurs because the blood loses egg white (protein) via inflamed kidneys. After days-weeks, the protein level in the blood becomes too low. A urine test in the form of a urine dipstick, which can be done at any doctor's office, will be able to reveal proteins in the urine early on and should always be done during investigation and control. An early but uncertain symptom that the kidneys may be attacked is that the urine may foam. Kidney inflammation in SLE can also cause high blood pressure and an increased risk of blood clots. For reliable detection of kidney inflammation and to determine the degree of severity/treatment, a tissue sample (biopsy) is often necessary. The procedure rarely has complications. Bleeding is seen in about 2%, which usually stops on its own. It is common to be observed for 24 hours after biopsy. Results of the tissue samples can be divided into different "classes" (References; Weening JJ. J Am Soc Nephrol 2004. Almaani S, Meara A, 2017). More about kidneys at SLE can be read on a separate page here .
Eyes. Sight can be affected in SLE, but relatively rarely. Tolosa Hunt syndrome causes severe eye pain. Reduced blood circulation, especially in Antifosfolipid syndrome, can cause eye damage with double vision and blood clots in the form of Sinus vein thrombosis with headache, especially behind eyes. The medicine Plaquenil (hydroxychloroquine) can cause damage to the retina (maculopathy) after several years of treatment. Ophthalmological examinations after 5 years of using Plaquenil are therefore recommended regularly (annually) (reference: Marble M, 2011). Retina (Retina) injuries ("Lupus retinopathy") with "cotton wool» changes, bleeding, swelling / oedema, a rare complication of lupus. Visual hallucinations are experienced in nerve/CNS manifestations. Dry eyes (Sicca) may be secondary Sjögren's syndrome (reference: Palejwala NV, 2012).
The diagnosis of SLE is based on a combination of medical history, symptoms and examination findings (see above) from two or more organs. Skin + joints are the most common manifestations and are detected in two out of three people with SLE. In addition, the results of laboratory tests, including typical "Antibodies” (ANA, with the subgroups: DNA, SSA, Sm or RNP and antiphospholipid antibody). The condition is that the results cannot be explained by another disease.
The SLE diagnosis in an early stage can be difficult due to individually different symptoms, even if the diagnosis is the same. Some make their debut with general manifestations, while others have an onset of disease where only one organ is attacked, for example in isolation thrombocytopenia or kidney inflammation. A third group debuts with multiorgan disease.
The diagnosis can be made independently of “criteria" which are intended for classification by research, but the criteria are still useful as a checklist.
Classification criteria (EULAR / ACR, SLICC, ACR)
The criteria are used for research into SLE. It is not a requirement that they should be raised in order to make the SLE diagnosis in clinical practice.
|2019 EULAR/ACR classification criteria (Aringer M, 2019)|
|Everyone should have ANA in blood test) with titer of at least 80. Total score of at least 10 is required for SLE|
|Manifestations Nephritis (kidney inflammation)||Score|
|Class III/IV (severe nephritis)||10|
|Sm or DsDNA||6|
|Pericardial fluid / Pleural fluid increased||5|
|Skin and mucous membranes|
|Acute Cutaneous Lupus (ACLE)||6|
|Subacute cutaneous or discoid lupus||4|
|Alopecia (without scarring)||2|
|CNS (Central Nervous System)|
|Delirium (disturbed consciousness)||2|
|Complement C3 and C4 low||4|
|Complement C3 or C4 low||3|
|Antiphospholipid antibody (lupus anticoagulant, cardiolipin or β2-glycoprotein)||2|
Similar conditions, differential diagnoses
- Arthritis (joint inflammation) can be reminiscent of Rheumatoid arthritis (RA) or psoriatic arthritis
- Cytopenia (low blood cell count) drug-induced, bone marrow disease (all three cell lines).
- Tiredness : infection, diabetes, low metabolism (hypothyroidism), hyper-parathyroidism, depression, chronic fatigue syndrome (CFS, ME).
- Fever of unknown cause: Opportunistic or tropicalinfection. Sweet's syndrome, autoinflammatory disease, Adult Stills.
- GVHD Graft-vs-Host Disease (rejection reaction) after transplantation
- Hemolytic anemia, autoimmune
- Hepatitis, autoimmune
- Skin: rosacea, Dermatomyositis, hypersensitivity reaction, Sweet's syndrome
- Lymph nodes: Infections, Lymphoma, Leukemia
- Medication side effect
- Mouth ulcers: Methotrexate-side effect, fungal infection
- Kidneys: Urinary tract infection, ANCA.-vasculitis
- Thromboembolism (blood clot): Primary Antifosfolipid syndrome, Leiden mutation, lack of protein S or protein C. Myxoma, endocarditis. Idiopathic.
- Sarcoidosis (skin symptoms, swollen lymph nodes, joint symptoms)
- Still's disease in adulthood (adult Still's) with fever, swollen lymph nodes and rash
It has been shown that several different drugs can trigger lupus-like disease. When the drug is stopped, the disease usually returns. The recent years have Biological treatment with TNF inhibitors such as infliximab (Rixarthon, Inflectra, Remicade), adalimumab (Humira) and etanercept (Benepali, Enbrel) have taken over as the most common causes of drug-induced lupus. In addition, approx. 25% during anti-TNF treatment get ANA antibodies in the blood, but very few get lupus (reference: Williams GL, 2009). Procainamide or Amiodarone hydrochloride (Cordarone) for heart rhythm disturbances and minocycline (antibiotic). The symptoms often appear after the triggering drug has been used for several months to a few years. The symptoms can be mild and unspecific, for example limited to joints or muscle aches. Thus, the diagnosis can be difficult. In blood tests, results are seen in ANA (anti-nuclear antibody) at 75-100%. SLE Antibodies (anti-histones are rarer (15%), but most typically, anti-DNA is more common) (reference: Picardo S, 2020). Unlike other SLE, the symptoms of drug-triggered lupus will completely go away after the triggering drug is removed. If symptoms persist, other SLE must be suspected. The risk of relapse is 30-50% if the same drug is used again (references: Ramos-Casals M, 2007; Ruby RL, 2019, Arnaud L, 2019).
Pregnancy / pregnancy in SLE
- Estrogen-containing birth control pills (regular birth control pills) should not be used if the blood tests have shown “Antifosfolipid antibodies”: Lupus anticoagulant, antibody against cardiolipin or beta-2 glycoprotein, or when the SLE disease is in an active phase. Similarly, if there is a risk of blood clots for other reasons (previous blood clots, smoking, Factor V Leiden mutation and more) (reference: Sammaritano LR, 2014).
- Progestagen preparations can usually be used ("mini-pill", birth control stick, hormonal coil, birth control syringe, emergency contraception/attack pill).
- You can read more about the pregnancy page here
Most women who are newly diagnosed with SLE are of childbearing age. Thus, pregnancy with SLE is relevant for many. Successful pregnancies are, with some exceptions, possible. There is, however, an increased risk of complications for both the pregnant woman and the fetus with SLE, so that close follow-up may be relevant.
- It is important that the disease before pregnancy is in a quiet phase for at least the last 6 months.
- One must not use drugs that can harm the fetus.
- Some make the mistake of ending treatment with important drugs, without these being replaced by drugs that can be used during pregnancy (Prednisolone, Plaquenil and Imurel can be used by pregnant women) and thus risk the SLE disease flaring up, which is particularly unfavourable.
- One should discuss a planned pregnancy with SLE with a rheumatologist well in advance, preferably more than three months in advance.
- Systemic lupus and pregnancy are described on own page here.
- You can read more about pregnancy and medication in rheumatic disease here
Diet by SLE
It has not been shown that special diet is the cause of SLE and special diet or dietary supplements do not usually affect the course of the disease. Meanwhile, SLE is a risk factor for later development of Atherosclerosis (atherosclerosis). Early prevention of heart attacks and strokes can therefore be important. In addition to good SLE treatment, so that the inflammation is under control, one should consider reducing high cholesterol (via diet and drugs), stop smoking, ensure well-regulated diabetes and avoid obesity. When using predisolone and other cortisone preparations, prevention of osteoporosis/fragility important. Sufficient calcium and vitamin D via food and supplements are recommended.
A combination of several immunosuppressive drugs (eg prednisolone, methotrexate, mycophenolate og biological drugs increases the risk of infection. Avoid foods that may contain bacteria. This can be raw meat, raw fish (sushi), raw eggs, unpasteurized cheese, milk or unwashed vegetables. General information about diet in rheumatic disease is described in a separate chapter.
Ten tips for living better with lupus
- Avoid sunbathing (especially sunburn) and use sun protection. Ultraviolet light is among the most common triggers for relapses and exacerbations (please see “Sun, solarium and lupus / SLE” on this page).
- Reduce the risk of infections. Infections increase the risk of recurrence of SLE. Toe vaccines against pneumococci (pneumonia) every 7-10 years and annual flu vaccine. These are "dead vaccines" which are usually well tolerated. A small (theoretical) risk of the SLE disease becoming worse from vaccines is more than canceled out by the fact that infections are reduced. If you use immunosuppressive drugs (such as Imurel, Methotrexate, mycofneolate/CellCept or high doses of Prednisolone), "live vaccines" should be avoided, since one can then get sick from the vaccine. Please read more about vaccines in rheumatic disease here.
- Ensure adequate rest.
- Be physically active.
- Do not use tobacco and avoid passive smoking.
- Treatment of high cholesterol is important. Diet and a cholesterol-lowering agent can be important in reducing the risk of stroke and heart attack in the long term
- Use the medication as recommended
- Women with SLE should be checked with cervical smears. Cervical cancer may be slightly increased in SLE (reference: Raposo A, 2016).
- If you use prednisolone, you should prevent osteoporosis/osteoporosis.
- Contact the doctor/rheumatologist if you feel increasing signs of illness.
Sun, solarium and lupus / SLE
Among those who have SSA or SSB antibodies there is a high risk of getting significant sun eczema. It can actually be serious if you are unlucky. The rash can start a few days after exposure to the sun and persist for several months. Experience suggests that solar eczema or worsening of the disease varies individually in SLE. Some people with SSA or SSB antibodies never get sun eczema and can tolerate the sun without relapse of the disease. Some react immediately to light (ultraviolet radiation, UVA and UVB), while others tolerate most. It is not known why there are differences, but in general caution with sunlight is recommended. In general, strong sun protection is recommended, for example sunscreen with factor 55 or more.
Both sun and solarium can damage the cells in and under the skin. Damaged cells are taken care of by our immune system which is then stimulated and more active. It can lead to the production of several antibodies (anti-DNA and others) suspected of being important for the development of SLE. Thus, sun can trigger or worsen SLE (reference Lehmann P, Homey B. .2009).
By using immunosuppressive medications like methotrexate, mycophenolate og Azathioprine a slightly increased risk of skin cancer has been demonstrated in the long term. Sunbathing is a significant contributing factor that should be limited. Some medications can trigger severe sun eczema. Among these are Plaquenil, Bactrim and Trimethoprim Sulfa.
When one is little in the sun, Vitamin D production in the body be low. This applies especially to dark-skinned people and those with SLE who protect themselves well. Low vitamin D can worsen the lupus disease. You can measure the vitamin D level in a blood test. Vitamin D tablets or a suitable diet will correct a deficiency and prevent problems.
Treatment of SLE is individually different, depending on how the disease attacks the individual and is adapted to the course of the disease and tolerance for drugs along the way. The treatment is usually managed by a medical specialist.
Sun exposure and especially sunburn should be avoided because disease activity may increase, a relapse may occur or the drugs contribute to skin damage, including an increased risk of skin cancer. Ensure vitamin D substitution. Sun factor 70 is recommended, factor 30 is too low.
Infections increases the risk of disease activity and should be avoided as far as possible. Preventive measures such as vaccines (influenza, pneumococci, papillomavirus, meningococci, hepatitis) are important, but also to avoid exposure to the risk of infection (backpackers in Asia, Africa, etc.). Corticosteroids such as prednisolone, especially in high doses (>15mg/day) increase the risk of infection and are used for strict indications. Carefully consider the benefits of treating the mildest disease manifestations.
Osteoporosis. Prevention is important if you use prednisolone or other cortisone for more than a few weeks.
Atherosclerosis. In the longer term, there is an increased risk of atherosclerosis. One should avoid obesity and smoking. High cholesterol should be treated. Statins are the first choice. One should strive to keep cholesterol in the blood well below 5 mmol/l and/or an LDL level below 3 mmo1/L. Hydroxychloroquine (Plaquenil) can also have a beneficial effect.
treat to target. Before starting, you should set goals that must be reached if the treatment works as intended ("treat to target"). Realistic treatment goals can be the absence of clinical disease activity (remission according to the doctor's assessment) and prednisolone ≤ 5mg/day. One can start from individual manifestations or disease activity (see also section above) measured by composite score: APPENDIX (British Isles Lupus Assessment Group) , SLEDAI (Systemic Lupus Erythematosus Disease Activity Index). In lupus nephritis, the treatment goal is to achieve a complete renal response. Because the kidney disease can persist despite a good clinical treatment effect, some recommend a new kidney biopsy and histological/immunopathological treatment goals after 1-2 years of treatment (Parody I, 2020). Although newer drugs and combination treatments have significantly improved the outcome, unfortunately we are still far from achieving the desired treatment goal in most patients with lupus nephritis (please see under Prognosis below). By reaching the treatment goals, you will also prevent organ damage, reduce cardiovascular comorbidity, improve the patient's quality of life and improve the mortality rate (Yang Z, 2022).
Among the drugs that are often used against SLE are:
- Prednisone (a cortisone). One wants the shortest possible treatment and with the lowest possible dose to reduce side effects. When the disease has been symptom-free on Prednisolone 5mg or less for one year, treatment can in some cases be discontinued. However, one must be aware that the risk of relapse is then four times increased (reference: Mathian A, 2020)
- Plaquenil (hydroxychlorokine)
- Imurel (azathioprine)
- CellCept (mycophenolate)
- Benlysta (belimumab)
- Sendoxan (cyclophosphamide)
Relapse, disease flare-up
Disease relapse (recurrence). In the case of relapse of SLE, symptoms and examination findings differ from person to person, but a general rule is that they are similar to the symptoms and examination findings with which the disease in the individual started. Impaired general condition with fatigue, fever/night sweats, hair loss, joint pain, swollen joints, eczema, kidney inflammation or mouth ulcers occur. Signs of disease activity in blood and urine: Blood lowering reaction (SR) is high, low CRP Cell counts (low numbers), s-creatinine (increases if kidney function is reduced), anti-DNA (antibody may increase), Complement factors C3 and C4 may be lower . Urine containing protein and blood indicates kidney inflammation.
General recommendations for SLE treatment have been drawn up by, among others, EULAR. Reference is made to guidelines in published literature (Fanpouirakis A, 2019) (Thomas Dörner, Richard Furie, 2019) (Fanouriakis A, 2020) and the supervisor of Norwegian rheumatology association/medical association.
Useful links for more information
- Keywords for medical examination and medical evaluation of SLE here
- Link to FSS you will find here (Deacon home hospital)
- Keywords that help you write a medical journal at SLE here
- SLEDAI, scores of disease activity
- Rights at SLE (Norsk Revmatikerforbund)
- Large Medical Encyclopedia
Guidelines and recommendations
- EULAR: Fanpouirakis A, 2019 (Management 2019)
- EULAR: Fanouriakis A, 2020 (managemnet lupus-nephritis)
- EULAR: Andreoli L, 2017 (Assisted fertilization)
- EULAR: Götestam Skorpen, 2015 (medicines for singing and breastfeeding)
- ACR: Hahn BH, 2012 (Lupus nephritis)
- British: Gordon C, 2018 (Management)
- Norwegian Rheumatological Association / Medical Association (supervisor)
- Thomore Dörner, Richard Furie, Lancet 2019
- Mug CC, 2018
- Norby GE, 2010 (Nephritic)
- Jordan N, 2016 (Newer Treatment Methods)
- Magro-Checa C, 2016 (neuropsychiatric lupus)
- Ünlü 0, 2016 (αPL antibody at SLE)
- Lerang K, 2012 (incidence and prevalence in Norway)
- Lerang K, 2014 (survival)
- Androli L, 2017 (EULAR recommendations for planning pregnancy)
- Yuen HK, 2014. Management of fatigue in SLE