Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect many parts of the body. It is more common in women and certain ethnic groups. Genetic and environmental factors contribute to its development by causing the immune system to attack the body's own tissues and organs. Symptoms can include joint pain, rashes, fatigue, and organ involvement. Diagnosis is based on clinical criteria and the presence of autoantibodies. Treatment involves managing symptoms with medications such as corticosteroids, antimalarials, and immunosuppressants. Lifestyle changes can also help control the disease.

1. SYSTEMIC LUPUS
ERYTHROMATOSUS
1
BY
PRATYUSHA BANDARU

2. INTRODUCTION
• Systemic lupus erythematosus (SLE) is the prototypic
multisystem autoimmune disorder with a broad spectrum
of clinical presentations encompassing almost all organs
and tissues
• The extreme heterogeneity of the disease has
led some investigators to propose that SLE represents a
syndrome rather than a single disease
2

3. EPIDEMIOLOGY
• Prevalence rates in lupus are estimated to be as high as 51 per 100
000 people in the USA
• SLE affects about one in 2000 people, five times more women
than men in india
• The incidence of lupus has nearly tripled in the last 40 years,
mainly due to improved diagnosis of mild disease
• Women are affected nine times more frequently than men and
African American and Latin American
• Mestizos are affected much more frequently than Caucasians, and
have higher disease morbidity
• Sixty-five percent of patients with SLE have disease onset
between the ages of16 and 55 years, 20% present before age 16,
and 15% after the age of 55
3

4. AETIOLOGY
• The aetiology of SLE includes both genetic and
environmental components with female sex strongly
influencing pathogenesis
• These factors lead to an irreversible break in immunological
tolerance manifested by immune responses against
endogenous nuclear antigens
4

5. AETIOLOGY(CONTINUES….)
Genetic factors
• Siblings of SLE patients are approximately 30 times more likely to
develop SLE compared with individuals without an affected sibling
Environmental factors
• Environmental triggers of SLE include ultraviolet light, demethylating
drugs, and infectious or endogenous viruses or viral-like elements
• Barr virus (EBV) has been identified as a possible factor in the
development of lupus
5

6. AETIOLOGY(CONTINUES….)
Epigenetic effects
• The risk for SLE may be influenced by epigenetic effects such as DNA
methylation and post-translational modifications of histones, which can
be either inherited or environmentally modified
• Epigenetics refers to inherited changes in gene expression caused by
mechanisms other than DNA base sequence changes
Hormonal factors
• In murine models, addition of oestrogen or prolactin can lead to an
autoimmune phenotype with an increase in mature high-affinity
autoreactive B cells
6

7. AETIOLOGY(CONTINUES….)
7

8. Signs and symptoms that suggest systemic
lupus include:
• Painful or swollen joints
• Fingertips and/or toes become pale or purple from the cold or stress
• Sores in the mouth or nose
• Low blood count
• Red rash or color change on the face, across the cheek or bridge of nose
• Unexplained fever for several days
• Chest pain associated with breathing
• Protein in the urine
• Extreme fatigue — feeling tired all the time
• Sensitivity to the sun
• Depression, trouble thinking, and/or memory problems
• Unusual hair loss, mainly on the scalp
8

9. DIAGNOSIS
9

10. CLASSIFICATION CRITERIA FOR DIAGNOSIS
• The Systemic Lupus International Collaborating Clinics (SLICC)
Classification Criteria*
they are classified based on two criteria they are
immunological critera
clinical criteria
10

11. Clinical criteria
• Acute cutaneous lupus erythematosus (including “butterfly rash“)
• Chronic cutaneous lupus erythematosus (e.g., localized or generalized discoid
lupus erythematosus)
• Oral ulcers (on palate and/or nose)
• Non-scarring alopecia
• Synovitis (≥ 2 joints) or tenderness on palpation (≥ 2 joints) and morning
stiffness (≥ 30 min)
• Serositis (pleurisy or pericardial pain for more than 1 day)
• Renal involvement (single urine: protein/creatinine ratio or 24-hour urine
protein, >0.5 g)
• Neurological involvement (e.g., seizures, psychosis, myelitis)
• Hemolytic anemia
• Leukopenia (<4000/μL) or lymphopenia (<1000/μL)
• Thrombocytopenia (<100 000/μL) 11

12. Immunological criteria
• ANA level above laboratory reference range
• Anti-dsDNA antibodies
• Anti-Sm antibodies
• Antiphospholipid antibodies (anticardiolipin and anti- β 2-
glycoprotein I [IgA-, IgG- or IgM-] antibodies; false-positive VDRL
[Venereal Disease Research Laboratory] test)
• Low complement (C3, C4, or CH50)
• Direct Coombs test (in the absence of hemolytic anemia)
12

13. Investigations in suspected systemic lupus erythematosus (SLE) and
monitoring after diagnosis
•Screening laboratory tests*1
•Erythrocyte sedimentation rate
•Blood count, differential blood count
•Creatinine
•Urinary status and sediment
•Antinuclear antibodies (ANA) (HEp-2 cell test with fluorescence pattern)
13

14. Further laboratory tests after positive screening*1 (particularly in case
of positive ANA)*1
•Further differentiation of ANA (particularly anti-Sm, -Ro/SSA, -La/SSB, -
U1RNP antibodies, etc.)
•Anti-dsDNA antibodies (ELISA; confirmation by radioimmunoassay or
immunofluorescence test with Crithidia luciliae)
•Complement C3, C4
•Antiphospholipid antibodies, lupus anticoagulant
•Glomerular filtration rate; 24-hour urine (if urine protein positive),
alternatively: protein/creatinine ratio in single urine sample; investigation
for dysmorphic erythrocytes in sediment
•Liver enzymes; lactate dehydrogenase; creatine kinase in presence of
muscular symptoms
•Further laboratory tests depending on clinical symptoms
•Screening for comorbidities
•Assessment of vaccination status
14

15. 15
Effect of lupus on different organs

16. Effect on blood
• aemia: low hemoglobin or red blood cells
• thrombosis: excess blood clotting
Effect on gastro intestinal system
• Esophageal Disorders in Lupus - dysphagia,GERD
• Digestive Difficulties - diarrhea,vomiting,nausea
• Ulcerative Colitis – ulcers in lining of colon and rectum
• Crohn’s Disease - inflammation of digestive system
• Peritonitis - inflammation of peritonium
• Ascites - accumulation of fluid
• Pancreatitis - inflammation
• Liver complications - hepatic vasculitis jaundice
• peptic ulcers
16

17. Effect of lupus on kidneys
• Lupus Nephritis-Inflammation of the nephrons
• end-stage renal disease (ESRD)
Effect of lupus on skin
• Chronic cutaneous (discoid) -lupus-disk-shaped, round lesions
• Subacute cutaneous lupus-red scaly skin lesions with distinct edges
• Acute cutaneous lupus-occurs when systemic lupus is active
17

18. Other skin problems
• Calcinosis
• Cutaneous vasculitis lesions
• Hair loss
• Raynaud’s phenomenon
effect of lupus on musculoskeletal system
• Inflammation
• Lupus Arthritis
• Lupus Myositis
• Drug-Induced Muscle Weakness
18

19. Effect on Central Nervous System (CNS)
• Headaches
• Confusion
• Fatigue
• Depression
• Seizures
• Strokes
• Vision problems
• Mood swings
• Difficulty concentrating
19

20. Effect on Peripheral Nervous System (PNS)
• Vision problems
• Facial pain
• Ringing in the ears
• Dizziness
• Drooping of an eyelid
• Carpel tunnel syndrome
Effect on somatic nervous system
• Numbness
• Burning
• Tingling
• Mental confusion
20

21. Lupus Treatments
• While there is no cure for lupus, early diagnosis
and treatment can help in managing the symptoms
and lessening the chance of permanent damage to
organs or tissues.
• Because lupus is different for every person,
treatments and medications are prescribed based on
individual needs.
• For mild cases of lupus, medicines may include
over-the-counter pain relievers and anti-
inflammatory medicines.
• For more severe lupus, or when internal organs are21

22. Lupus Treatments
There are many categories of drugs physicians use to treat lupus. However,
the U.S. Food and Drug Administration or “FDA” has approved only a few
specifically for lupus, which include:
• Corticosteroids, including prednisone, prednisolone, methylprednisolone,
and hydrocortisone
• Antimalarials, such as hydroxychloroquine (Plaquenil®) and chloroquine
• The monoclonal antibody belimumab (Benlysta®)
• Acthar (repository corticotropin injection), which contains a naturally
ocurring hormone called ACTH (adrenocorticotropic hormone)
• Aspirin
22

23. TREATMENT PLAN
Once you have been diagnosed with lupus, your doctor will develop a
treatment plan based on your age, symptoms, general health, and
lifestyle. The goals of any treatment plan are to:
 Reduce inflammation caused by lupus
 Suppress your overactive immune system
 Prevent flares, and treat them when they occur
 Control symptoms like joint pain and fatigue
 Minimize damage to organs
23

24. 24
TREATMENT PLAN (CONTINUES……)
People with lupus often require other drugs to treat conditions commonly
seen with the disease. Examples include:
 Diuretics for fluid retention
 Antihypertensive drugs for high blood pressure
 Anticonvulsants for seizure disorders
 Antibiotics for infections

25. Alternative medicine
Sometimes alternative or complementary medicine may benefit people with
lupus.
Complementary and alternative treatments for lupus include:
Dehydroepiandrosterone (DHEA).
Supplements containing this hormone have been shown to reduce the dose of
steroids needed to stabilize symptoms in some people who have lupus.
Fish oil.
•Fish oil supplements contain omega-3 fatty acids that may be beneficial for
people with lupus.
•Side effects of fish oil supplements can include nausea, belching and a fishy
taste in the mouth.
Vitamin D.
• There is some evidence to suggest that people with lupus may benefit from
supplemental vitamin D.
25

26. Lifestyle and home remedies
Take steps to care for your body if you have lupus. Simple measures can help
you prevent lupus flares and, should they occur, better cope with the signs and
symptoms you experience. Try to:
•See your doctor regularly
•Get adequate rest
•Be sun smart
•Get regular exercise.
•Don't smoke
•Eat a healthy diet
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27. REFERENCES
• Systemic lupus erythematosus
Jessica J Manson* and Anisur Rahman, Orphanet Journal of Rare Diseases2006, 1:6
• The Diagnosis and Treatment of Systemic Lupus Erythematosus
Annegret KuhnProf. MBA,Gisela Bonsmann, Dr. Hans-Joachim Anders, Prof., Peter
Herzer, Prof.,Klaus Tenbrock, PD Dr., and Matthias Schneider
Dtsch Arztebl International 2015 Jun; 112(25): 423–432.
• http://www.lupusresearchinstitute.org/lupus-news/other
• http://www.lupusresearchinstitute.org/lupus-facts/lupus-fact-sheet
• http://www.lupusresearchinstitute.org/lupus-facts
• http://www.lupus.org/answers/entry/blood-disorders
• http://www.lupus.org/answers/entry/how-is-lupus-treated
• https://www.google.co.in/search?q=discoid+rash&client=firefox-
b&biw=1366&bih=657&source=lnms&tbm=isch&sa=X&sqi=2&ved=0ahUKEwiUudP
c4rXPAhUGRI8KHbZjA8kQ_AUIBigB#tbm=isch&q=raynaud%27s+phenomenon&img
rc=_sIKDij7DmXTUM%3A
• https://www.google.co.in/search?q=discoid+rash&client=firefox-
b&biw=1366&bih=657&source=lnms&tbm=isch&sa=X&sqi=2&ved=0ahUKEwiUudP
c4rXPAhUGRI8KHbZjA8kQ_AUIBigB#tbm=isch&q=calcinosis&imgrc=zHGOKDcBka3l
9M%3A
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