This document discusses systemic lupus erythematosus (SLE), an autoimmune disease where the immune system attacks the body's own tissues and organs. It causes inflammation and damage to many different body systems. SLE is more common in women and typically presents between ages 15-25. Symptoms can include rashes, joint pain, fatigue, and organ involvement. Diagnosis involves evaluating symptoms, signs, and antibody tests. Treatments include medications like NSAIDs, antimalarials, steroids, and immunosuppressants to reduce symptoms and prevent organ damage. Complications can affect many organs but most commonly involve the kidneys, heart, and lungs. With treatment, 5-year survival rates are over 85%.

3. Definition
Pathophysiology
Causes
Signs and Symptoms
Diagnosis
Treatment
Complications
Prognosis

4.  SLE is a chronic autoimmune disease in
which the body immune system induce
auto-antibodies against the DNA which
can affect almost any organ system
 Systemic (more than one organ can be
affected)
 Lupus (autoanitbody against DNA)
 Erythematosus (skin rash)

5.  The type of reaction in this autoimmune
disease is Type III Hypersensitivity Reaction
(immune-complex).
 Extracellullar double-stranded DNA occurs
mainly in the form of nucleosomes, which are
fragments of chromatin that cells release
when they undergo apoptosis. Pathogenic
anti-ds-DNA autoantibodies in the patient
with lupus bind to nucleosomes and gets
deposit on different sites and induce the
inflammatory reaction

6.  Ultraviolet (UV) radiation
(sun exposure) exacerbates
 the lesions of SLE. Can cause autoimmune
disorders by increase in cell death and/or a
decrease in the rate of dead cell clearance
 Cigarette smoking has been shown
to be associated
 with the development of SLE.

7.  Drugs such as procainamide and
hydralazine can inducean SLE-like disease,
although typically glomerulonephritisdoes
not develop.
 Sex hormones in the SLE 10 times
more common in women during
reproductive
years than in men of similar ages but only 2 to
3 times
more common in women during childhood or
after the age of 65.

9.  • Type I interferons.
 patients with SLE also produce abnormally large
 amounts of IFN-α.
 • TLR signals(found dendritic cells)
 TLRs that recognize DNA and RNA, notably the
DNArecognizing
 TLR9 and the RNA-recognizing TLR7, produce
 signals that activate B cells specific for self nuclear
 antigens.(found B lymphocytes Plasmacytoid
dendritic cells)
 • Failure of B cell tolerance
 patients with SLE suggest the presence of defects
in both
 central and peripheral tolerance.

11.  Immunologically the disease is associated
with an enormous number of auto antibodies
basically including (ANAs).
 Fundamental defect in SLE is a failure to
maintain self-tolerance ,leading to
production of large number of auto
antibodies .
 Mechanism by which the auto antibodies
can damage tissues :
I. Direct damage to tissues
II. Immune complex deposit

12. Triggering :

13.  Auto antibodies in lupus :
I. ANAs
II. Auto antibodies against ds-DNA
III. RO
IV. LA(lupus anticoagulant )
V. Sm

14.  Symptoms vary from person to person, and may come and go.
Almost everyone with SLE has joint pain and swelling. Some
develop arthritis. The joints of the fingers, hands, wrists, and knees
are often affected.
 Other common symptoms include:
 Chest pain when taking a deep breath
 Fatigue
 Fever with no other cause
 General discomfort, uneasiness, or ill feeling (malaise)
 Hair loss
 Mouth sores
 Sensitivity to sunlight
 Skin rash -- a "butterfly" rash in about half people with SLE. The
rash is most often seen over the cheeks and bridge of the nose, but
can be widespread. It gets worse in sunlight.
 Swollen lymph nodes

15.  Other symptoms depend on which part of the
body is affected:
 Brain and nervous system: headaches, numbness,
tingling, seizures, vision problems, personality
changes
 Digestive tract: abdominal pain, nausea, and
vomiting
 Heart: abnormal heart rhythms (arrhythmias)
 Lung: coughing up blood and difficulty breathing
 Skin: patchy skin color, fingers that change color
when cold (Raynaud's phenomenon)
 Kidney: swelling in the legs, weight gain
 Hematologic (eg, cytopenias such as leukopenia,
lymphopenia, anemia, or thrombocytopenia)

17.  To be diagnosed with lupus , you must
have 4 out of 11 common signs of the
diseases .
Your doctor will do a physical exam and
listen to your chest . An abnormal sound
called a heart friction rub or pleural
friction rub may be heard . A nervous
system exam will also be done .

18. Tests used to diagnose SLE may include :
 Antibody tests including antinuclear
antibody (ANA) panel
 CBC
 Chest X-ray
 Kidney biopsy
 urinalysis

19. You may also have other tests to learn more
about your condition , some of these are :
Antithyroglobulin antibody
Antithyroid antibody
Complement components (C3 and C4)
Coombs test- direct
Cryoglobulins
ESR
Kidney function blood tests
Liver function blood tests
Rheumatoid factor
Antiphospholipid antibodies

20.  Almost 90% of all cases occur in women
 Overall, SLE affects women eight times more
often than it does in men
 At age 30 years, the ratio of women to men is 10:1
 The ratio at age 65 years, the ratio appears to be
about 3:1
 The prevalence rate among women between age 15
and 64 years is 1 in 700 women
 Symptoms usually appears between ages 15 and
25 years
 The prevalence in the general population is about
1 in 1000

21.  Mild forms of the disease may be treated
with:
 NSAIDs for joint symptoms and pleurisy,
after talking with your doctor
 Corticosteroid creams for skin rashes
 A drug also used to treat malaria
(hydroxychloroquine) and low-dose
corticosteroids for skin and arthritis
symptoms

22.  Treatments for more severe SLE may
include:
 High-dose corticosteroids
 Cytotoxic drugs

23.  Systemic lupus erythematosus (SLE) can
cause systemic complications throughout
the body.
 COMPLICATIONS OF THE BLOOD
 Almost 85% of patients with SLE
experience problems associated with
abnormalities in the blood.
 Anemia. About half of patients with SLE
are anemic.

24.  The effects on blood vessels have also
been associated with confusion,
headaches, and seizures. Leg ulcers can
also develop.
 Patients with APS who become pregnant
have a high incidence of pregnancy loss,
especially in the late term.

25.  Heart disease is a primary cause of death in
lupus patients. The immune response in SLE
can cause chronic inflammation and other
damaging effects that can cause significant
injury to the arteries and tissues associated
with the circulation and the heart. In addition,
SLE treatments (particularly corticosteroids)
affect cholesterol, weight, and other factors that
can also affect the heart.

26.  About 45% of patients with SLE suffer
gastrointestinal problems including
nausea, weight loss, mild abdominal pain
 and diarrhea.
 Severe inflammation causes cramping,
vomiting, diarrhea, and rarely intestinal
perforation which is life threatening.

27.  Inflamed blood vessels in the eye can
reduce blood supply in the retina,
resulting in degeneration of nerve cells
and a risk of hemorrhage in the retina.
 The most common symptoms are cotton-
wool-like spots on the retina.

28.  SLE affects the lungs in about 60% of patients:
 Inflammation of the membrane lining the lung (pleurisy) is
the most common problem, which can cause shortness of
breath and coughing.
 In some cases, fluid accumulates, a condition called pleural
effusion.
 Inflammation of the lung tissue itself is called lupus
pneumonitis. It can be caused by infections or by the SLE
inflammatory process. Symptoms are the same in both cases:
fever, chest pain, labored breathing, and coughing. Rarely,
lupus pneumonitis becomes chronic and causes scarring in
the lungs, which reduces their ability to deliver oxygen to the
blood.
 A very serious and rare condition called pulmonary
hypertension occurs when high pressure develops as a result
of damage to the blood vessels of the lungs.

29.  The kidneys are a crucial battleground in
SLE because it is here that the debris left
over from the immune attacks is most likely
to be deposited. Also, the immune response
can also attack different parts of the kidney
causing damage. About 50% of patients with
SLE exhibit inflammation of the kidneys
(called lupus nephritis).This condition occurs
in different forms and can vary from mild to
severe. Poor kidney function and kidney
failure may result from this damage.

30.  Serious complications occur eventually in
about 30% of patients. If kidney injury
develops, it almost always occurs within
10 years of the onset of SLE, rarely after
that.

31.  In 1950:
• 5 yr survival rate in case of lupus nephritis
was 0%
 Recently:
• 5 yr survival 85%
• 10 yr survival 73%

32.  Poor prognostic indicators
• Delay in treatment of more than 5 months from
onset of nephritis
• Young age at onset of nephritis
• Black racial background
• Hypertension
• Nephrotic syndrome
• Renal biopsy findings showing diffuse lupus
nephritis or high chronicity index