Systemic Lupus Erythematosus

• LUPUS is otherwise known as Systemic Lupus Erythematosus (SLE)
• Lupus affects women with childbearing age group with 9 times more often than men
• It can affect children then it’s known as Juvenile SLE
• Lupus can be different from person to person.
• It is also known as the “disease of 1000 faces” as it can manifest in 1000 ways in thousand different persons
• This year the LUPUS DAY theme was –“LUPUS KNOWS NO BOUNDARY” that means lupus can affect at any organ of the body starting from the skin, hair to blood, kidney, brain and heart.
• All lupus patients manifest differently
• It is not always true that in a said LUPUS patient will have all the manifestation of lupus.

• Basically lupus is an autoimmune disease.
• This means the immune system of the own body attacks the healthy cells and tissues.
• This attack produces inflammation, which comes from the Latin word “inflammare” (light on fire).
• People affected with lupus, inflammation is extremely variable and can occur in almost any tissue of the body extend from skin, hair, joints to blood, brain and kidney.
• Exact cause of this autoimmunity is not yet known but gene and environment plays a role in pathogenesis of the disease.

• This disease mostly affects young women during reproductive age group (20-45 yrs. of age)
• Nine out of ten people with lupus are women. Less common in male person.
• It is found that about 1 in 1000 people get lupus.
• It doesn’t pass down through families.
• Chemicals from cigarette smoke or a virus in the environment might be the reasons for lupus.
• It is not an infectious disease or contagious disease
• It’s not true that if a mother is affecting with lupus then the child must develop lupus

“Lupus knows No boundary” “Lupus is a disease of 1000 faces” “Lupus is a treatable disease” “NO TWO LUPUS PATIENTS ARE ALIKE”

• Lupus can have wide range of symptoms
• Symptoms can vary person to person
• One person can have only fatigue or extreme tiredness and pain in the joints whereas another person can have kidney involvement
• Most common symptoms are easy tiredness, fever and skin rash
• Lupus tends to worst in first five years of onset the disease
• Always watchful for the urine abnormality in a lupus patient
• Some of the symptoms are:
   Fatigue (mostly tiredness)
   Hair loss
   Prolong fever
   Photosensitivity rash-reddish discoloration over the sun exposed area
   Rash over the malar area
   Mouth ulcers over the hard palate-mostly painless, usually unnoticed by the patient
   Joint pain and swelling (Arthritis)
   Protein in the urine (more froth in the urine)
   Swelling over the foot
   Pain in the chest while taking a deep breath in (pleurisy)
   Muscle pain and weakness
   Psychosis, depression and frequent fits
   Low hemoglobin and/or low platelet count
• NOTE: one lupus person need not have all the symptoms of lupus

How is lupus diagnosed?

• As Lupus can have varied presentation from person to person so sometime it may be difficult in diagnosis, often misdiagnosed as Tuberculosis.
• You should consult a rheumatologist if you are diagnosed or suspected as LUPUS by a physicians
• A detail history and Complete physical examinations should be done
• Complete examination starting from scalp, hair, mouth cavity, joints to lung, brain and heart
• Along with routine blood test and urine examination special immunological investigation are mandatory to reach a diagnosis of lupus

• Routine Blood test
   Complete blood count (CBC): For Anemia, Low platelet count, Low TWBC count
   Liver Function test: To look for transaminases-raised SGOT or SGPT,Billurubin level ( hemolysis)
   Kidney function test-Serum Creatinine
   ESR/CRP-For acute phase reactant marker
• A good Urine Routine and Microscopic Examination
• 60-75% of lupus patient have kidney involvement and treatment changes depending on the type of kidney disease
• Special Immunological Investigation
   ANA test
   ANA profile or ENA test
   Anti Ds DNA Antibody test
   C3 and C4 level
   Antiphospholipid antibody profile test (Sometime)
   Lupus anticoagulant (Sometime)

Anti-Nuclear Antibody (ANA) Test

• This is the most important blood test (also known as the ANA) and is done to confirm the diagnosis lupus
• ANA positivity occurs in 98% cases and negative occurs only in 2% of cases of lupus.
• If ANA test is negative then lupus extreme unlikely.
• If ANA test comes positive in the presence of lupus symptoms then there is strong possibility of lupus
• Sometime people with other disease and older individual can have ANA positivity
• ANA test should be done in IIF(Indirect Immunofluroscence) method not in the ELISA method
• This ANA test gives two results:
  o the titre
  o the pattern
• ANA includes number of patterns including homogenous, speckled, nucleolar, centromere, rim enhancing.

Anti-Double Standard DNA (anti-dsDNA):

• The antibodies which attacks DNA, are measured in this test.
• This is done by ELISA method
• This is a very specific test for lupus.

Extractable Nuclear Antigen (ENA) panel:

• In this test, antibodies to 7-15 proteins in the body are measured.
• Here the antibodies include: anti-Ro (also called anti-SSA), anti-La (also called anti-SSB), anti-Sm, anti-RNP, anti-Jo-1, anti-Scl70, and anti-centromere.

Kidney Biopsy:

• Sometime your concerned rheumatologist may ask for the kidney biopsy
• It is needed when there is protein (more than 1gram/24 hr urine) or/and RBC in the urine
• It is warranted when there is increase in Serum Creatinine or Kidney failure
• Kidney biopsy will help you in two ways for the further treatment:
  • Biopsy will tell you type of kidney involvement (TypeI/ TypeII/ TypeIII/ Type IV/ TypeV/ TypeVI) and treatment changes according to type of involvement
  • Biopsy will also tell you how much disease is active and already damage (Chronic).
  • Kidney biopsy should be done after proper counseling and taking consent from the patient or relatives

• Lupus has no effect on fertility
• The chances of getting pregnant for lupus patient is same as normal people
• If Lupus patient has recurrent pregnancy loss then Anti-Phospholipid Antibody test should be done

Key points:

• Women with lupus should plan the pregnancy. Before getting pregnant It is best to make sure the disease is under good control for at least 6 months.
• Certain medications are there that should be avoided during pregnancy. Discussion about all medications with your doctor should be there.
• Family planning should be discussed with rheumatologist because lupus can affect a pregnancy in many ways.