Systemic Lupus Erythematosus (SLE) 4.78/5 (40)

Last updated: 29 / 03 / 22
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SLE

Lupus often attacks young women, less often among children. Pxhere, CC 0




Systemic Lupus Erythematosus (SLE) / Lupus

ICD-10 codes: M 32.9 (unspecific SLE), L93.0 (diskoid lupus)

Definition

Lupus occurs in at least four different types:

  • Systemic lupus erythematosus (SLE) is the most extensive disease and is most discussed on these pages. This type is often meant when people talk about Lupus
  • Skin Lupus attacks the skin with various forms of eczema, but not the nervous system or internal organs
  • Drug-triggered Lupus
  • Neonatal Lupus can occur in newborns, but goes away on its own within a few weeks

Systemic Lupus Erythematosus (SLE) is the most well-known and common diagnosis among the Systemic connective tissue diseases which are rheumatic diseases. The symptoms, blood and urine tests are important for the diagnosis. SLE is often more imprecisely referred to as "Lupus".

Disease Cause

The cause of Lupus's disease 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. Rheumatic inflammation occurs in one or more organs. In this way, SLE can involve 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 one Autoimmune disease

Occurrence of lupus

SLE is about ten times more common in women than men. A Norwegian study shows that approx. 150 people become ill with lupus annually in Norway, and that we have approximately 2.600 women with SLE in Norway (reference: Lerang K, 2012).

Heredity

Heredity occurs, but it is still rare for more people in the immediate family to have SLE. Even among single twins (they are genetically identical) it is only in 25% risk that both get SLE

  • Among siblings, the incidence is estimated at 2%, which means that 98% does not get the disease even if a sister or brother is ill
Lupusfoto.jpg

Photo: Butterfly butterfly at Lupus. By Doctor Internet. CC BY SA 4.0

Symptoms of lupus

SLE can have very different symptoms and vary greatly from person to person. Early symptoms:

Further assessment and research can reveal a number of more or less typical findings:

Blood

Anemia (low blood percentage) may have various associated causes related to SLE

  • Aplastic anemia
  • Hemolytic anemia (Autoimmune)
    • Microangiopathic (Schistocytes in blood smears). complications: TTP, DIC, CAPS, Medicines
  • Iron deficiency
  • Chronic rheumatic inflammation (inflammation)
  • Drug Side Effects (Sendoxan, Plaquenil, Mycophenolate, NSAIDs)
  • The spleen is too large (splenomegaly)
  • Kidney failure

Antifosfolipid antibodies occurs in approx. 40% with SLE

  • Antifosfolipid syndrome (Antiphospholipid antibody, blood clots or multiple related spontaneous abortions) occur in approx. 10% with SLE
  • Lupus anticoagulant
  • Anti-cardiolipin antibody
  • Beta-2-Glycoprotein Antibody

Leukocytopenia (low white blood cell count)

  • Neutropenia (low number of neutrophil leukocytes)
    • Virus
    • Medication
    • Splenomegaly
  • Lymphopenia (low number of lymphocytes)
    • Active SLE

Thromboctopenia (low platelet count)

Pancytopenia (low number of red and white blood cells and lack of platelets). Investigation with biopsy (tissue sample) from bone marrow is required

Heart

Skin

Lupus rash. Department of Internal Medicine, Konkuk University Medical Center, Seoul, Korea. CC-BY-NC 3.0

Joint pain

Joint pain is often in the fingers which can also swell (Arthritis)

  • Occurrence: 60-100%
  • Types of joint damage
    • Joint and tendonitis (arthritis tendinitis) is common (70-80%)
    • Jaccoud arthritis causes skewed joints without X-rays showing skeletal damage (not erosive) occurrence is approximately 5%
    • Skeletal injury (erosive arthritis)  can be similar RA (rheumatoid arthritis) and attack approx. 5%

Liver disease

Liver disease is not among the most common complications of SLE

  • It is under discussion whether elevated liver enzymes (in blood tests) are due to Lupus inflammation ("Lupus hepatitis") or overlap with autoimmune hepatitis which occurs more frequently at SLE
  • Azathioprine, Methotrexate and other drugs can cause side effects from the liver
  • A latent ("sleeping") infection with Hepatitis B virus can flare up during SLE treatment

Lungs

Pulmonary Membrane-inflammation (Pleurisy) is not uncommon

Mouth ulcers

Mouth ulcers occur at SLE

Nervous System

Brain and spinal cord can be attacked

Kidneys

An early symptom of that kidneys can be attacked is that the urine is foaming

  • Kidney inflammation is often not felt until the legs swell
    • The cause of such swelling is the accumulation of "water" (edema)
    • The swelling of 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 will early reveal signs of renal insufficiency and should always be done by investigation and control
  • Renal inflammation of SLE may also lead to high blood pressure and increased risk of blood clots
  • More about kidneys at SLE can be read on a separate page here (in Danish)

Eye symptoms

The eyes can be attacked by SLE, but relatively rarely. For symptoms from eyes, a number of causes should be considered:

Sun, solarium and lupus / SLE

Among those who have SSA or SSB antibodies There is a great risk of getting a significant sun eczema. It can actually be serious if one is unlucky. The rash can start a few days after the influence of sun and persist for several months

  • Experience suggests that sun eczema or worsening of the disease varies individually by SLE. Some with SSA or SSB antibodies never get sun eczema and also tolerate the sun without relapse of the disease. Some respond immediately to light (ultraviolet radiation, UVA and UVB), while others tolerate most. One does not know why there are differences, but generally caution is recommended with sunlight. In general, strong sun protection is recommended, for example, sunscreen with a factor of 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 og Azathioprine there is a slight increased risk of skin cancer in the long term. Sunbathing is a significant contributing factor that should be limited
  • Some medications can trigger severe sun rash. Among these are Plaquenil, Methotrexate, Bactrim and Trimetoprim Sulfa.
  • When one is little in the sun, Vitamin D production in the body is low. This applies especially to dark-skinned and those with SLE that protect themselves well. Low vitamin D may aggravate the lupus disease. One can measure the vitamin D level in a blood sample. Vitamin D tablets or suitable diet will correct a deficiency condition and prevent problems

Drug-triggered Lupus

It is known that drugs can trigger Lupus (SLE) or lupus-like disease. In total, about XNUM different drugs are suspected.

  • TNF inhibitors. In recent years, TNF inhibitors have infliximab (Rixarthon, Inflectra, Remicade), adalimumab (Humira) and etanercept (Benepali, Enbrel) taken over as the most common causes of drug-triggered lupus
  • It is known from the past that SLE can be triggered by hydralazine used in hard-regulated blood pressure
  • Procaine amide or Amiodarone hydrochloride (Cordarone) against heart rhythm disorders

The symptoms often come after the triggering drug has been used for several months to some years. The symptoms can be mild and unspecific, for example limited to joint- or muscle aches. Thus, the diagnosis can be difficult. In blood samples, SLE- Antibodies (anti-histones are rare but most typical) and other SLE signs. Unlike other SLE, the symptoms of drug-triggered lupus will recur after the trigger drug is removed. If symptoms continue, another SLE must be suspected.

Medical investigation for suspected lupus

The disease history (see above) is important in combination with blood and urine tests:

  • Erythrocyte Sedimantation Rate (ESR) is increased while CRP remains almost normal
  • Number of white blood cells (leukocytes), red blood cells (erythrocytes) and platelets (platelets) should be measured in blood samples. They may be too low at SLE
  • Urin test must be checked for protein and red blood cells
  • Blood pressure measurements are made
  • Antibody samples ("Rheuma samples" in blood) include ANA test. If this test is positive (ie suspicious of disease), the laboratory will proceed with examination of ANA subgroups. These may show typical SLE signs (anti-DNA, anti-C1q, anti-Sm and other subgroups). There are high rashes that are crucial, because low titers also occur in other diseases and among healthy persons
  • Investigation by the physician consider whether increased body fluid (edema) is present. Joints, heart and lungs are also examined. The skin is evaluated for rash (eczema) on the body, face and scalp

Keywords by investigation of SLE here

The referral to a specialist at lupus

Diagnosis

The diagnosis is based on a combination of symptoms and examination findings from two or more organs (skin + joints are the most common manifestations and detected in two out of three with SLE). In addition, the results of laboratory tests, including typical ones, are assessed.Antibodies”(ANA, with subgroups: DNA, SSA, Sm or RNP and antiphospholipid antibody). The prerequisite is that the effects cannot be explained by other illness. The diagnosis can be made independent of "criteria" which are intended for classification by research, but the criteria are still useful as a checklist.

Classification criteria (EULAR / ACR, SLICC, ACR)

Incorrect diagnosis? (Symptoms similar, differential diagnoses)

Disease (recurrence) at lupus

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 symptoms and examination findings that the individual's disease started with.

  • Reduced 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: The blood-lowering reaction (SR) is high, low CRP (nevertheless increases somewhat if arthritis or inflamed pulmonary membranes (pleurisy), heart bag inflammation (pericarditis) or joint inflammation (arthritis) are present). number), s-creatinine (increases if kidney function is reduced), anti-DNA (antibody can increase), anti-C1q (high in 50% of cases, but not specific: see also HUVS), C3 and C4 (low complement factors). Urine containing protein and blood suggests kidney inflammation

More detailed information about the investigation and follow-up of SLE (most relevant for doctors) can be found here

Treatment

Treatment of SLE is individually different, depending on how the disease attacks the individual and is usually a specialist task. Among the commonly used drugs are:

Treat to Target (EULAR recommendations)

Before the treatment begins, a goal should be set for what one wants to achieve (“Treat to target”). The goal may be lower disease activity or disease remission (no disease activity), absence of organ infection (kidneys, skin, nervous system, lungs, heart) and / or better quality of life.

  • After a certain time (3-6 months) is evaluated whether the treatment goal has been reached

Vplease read about the drug treatment of SLE here (in Danish)

Treatment of renal inflammation / lupusnephritis here (in Danish)

Contraception at SLE

  • Estrogen-containing oral contraceptives (common oral contraceptives) should not be used if the blood samples have shown "Antiphospholipid antibodies": Lupus anticoagulant, antibody to 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 causes (previous thrombosis, smoking, Factor V Leiden mutation, etc.) (reference: Sammaritano LR, 2014)
  • Gestagen preparations can usually be used ("minipille", p-rod, hormone helix, p-syringe, emergency contraception)
  • You can read more about the pregnancy page here

Pregnancy / Pregnancy in SLE

  • Most people who get SLE are women between the ages of 20 and 29. About one in 1000 younger (fertile) women has SLE. Thus, pregnancy at SLE is relevant for many. Unfortunately, there is an increased risk of complications for both the pregnant woman and the fetus at SLE, but successful pregnancies are still, with some exceptions, possible to carry out.
  • Systemic lupus and pregnancy are described on separate page here (in Danish). A summary:

Diet by SLE

Systemic lupus contributes to increased risk of Atherosclerosis (atherosclerosis). In the process, heart attack and stroke may occur. At SLE, optimal drug treatment is important, but also risk factors must be reduced as high cholesterol (via diet and drugs), smoking, poorly regulated diabetes (diabetes) and overweight. High intake of multi-unsaturated fatty acids via "Mediterranean diet" reduces the risk of atherosclerosis.

A combination of several immunosuppressive drugs (eg Prednisolone, Methotrexate, MabThera, Remicade / Remsima / Inflectra) 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.

To live better with lupus

One can even contribute to a better prognosis at SLE. Here are nine tips:

  1. Avoid sunbathing (especially sunburn) and use sun protection
    • Ultraviolet light is one of the most common triggering causes of recurrence and aggravation (please see "Sun, solarium and lupus / SLE" above on this page)
  2. Avoid infections. Infections increase the risk of recurrence of SLE
    • Ta vaccines against pneumococci (pneumonia) every 7-10 years and annual flu vaccine. These are "dead vaccines" that are usually well tolerated. A small (theoretical) risk of SLE disease getting worse from vaccines is more than abolished by the fact that infections are reduced
    • If you are using immunosuppressive drugs (such as Imurel, Metotrexate or high doses of prednisolone), "live vaccines" should be avoided, since one can get sick from the vaccine.
    • Please read more about vaccines in rheumatic disease here
  3. Ensure adequate rest
  4. Be physically active
  5. Do not use tobacco and avoid passive smoking
  6. Treatment of high cholesterol is important
    • Diet and a cholesterol-lowering agent may be important in reducing the risk of stroke and heart attack in the long term
  7. Use the medication as recommended
  8. Women with SLE should be monitored with cervical cell samples.
  9. Contact your doctor / rheumatologist if you feel increasing signs of disease

Investigation and follow-up

Keywords for medical journal writing at SLE

SLEDAI, scores of disease activity

Rights at SLE (Norsk Revmatikerforbund)

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)

Large Medical Encyclopedia

Literature

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