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SYTEMIC LUPUS ERYTHEMATOUS,URTECERIYA
SYSTEMIC SCLERODERMA,ANAPHELACTIC SHOCK
A chronic inflammatory condition caused by an
autoimmune disease. An autoimmune disease
occurs when the body's tissues are attacked by
its own immune system. Patients with lupus
have unusual antibodies in their blood that are
targeted against their own body tissues.
Inate Susceptibility
•HLA type
•Immunoregulatory genes
•Hormonal levels
•Complement levels
Environmental Stimuli
•UV exposure
•Microbial response
•Medication
Autoimmune Proliferation
•Hyperactive B-cell/T-cell
activation
•High ratio of CDA;CD8 T-cells
•Defective immune complex
clearance
•Impaired tolerance
Autoantibody
Production
•Apoptosis and self
exposure
•Self-recognition
•Foreign-Ab cross
reaction
 fever, rash, arthritis, alopecia, and renal
involvement
Symptoms Occurrence
Achy joints (arthralgia) 95%
Fever more than 38 degrees C 90%
Rheumatoid arthritis 90%
Prolonged or extreme fatigue 81%
Skin rashes 74%
Anaemia 71%
Kindey involvement 50%
Pain in the chest on deep breathing
(pleurisy)
45%
Find out more about Asthma TreatmentSystemic Lupus Erythematosus
 Affects 85% of patients with SLE
 50% of patients have anemia
 Between 34 - 42% of patients have
antiphospholipid syndrome (APS)
 Patients who have APS have a high incidence
of blood clots
 Blood clotting puts patients at higher risk for
stroke and pulmonary embolism
• Heart disease is primary cause of death in lupus
patients
• Atherosclerosis, or plaque buildup in the arteries
• High blood pressure, most likely because of
kidney injury and corticosteroid treatments
• Heart failure
• Pericarditis, an inflammation of the tissue
surrounding the heart
• Myocarditis, an inflammation of the heart muscle
itself (rare)
 Affects 50% of patients
 Poor kidney function (mild) and kidney failure
(severe) may result from this damage.
 Serious complications occur in 30% of patients
 Infections (common)
 Gastrointestinal (45%)
 Joint, Muscle, Bone (Osteoporosis, Arthritis)
 Eye (5% temporary blindness)
 Difficult to diagnose
 No single diagnostic marker; identified
through a combination of clinal and laboratory
criteria
 Early diagnosis is important as it reduces
morbidity and mortality (lupus nephritis)
 4 of 11 clinical and laboratory criteria must be met
 Antinuclear antibody titer is the primary laboratory
test
 Antinuclear antibody titer of 1:40 and characteristic
multiorgan system involvement can be diagnosed
with systemic lupus erythematosus without
additional testing
 Patients with an antibody titer of 1:40 who fail to
meet full clinical criteria should undergo additional
testing: including tests for antibody to
doublestranded DNA antigen and antibody to Sm
nuclear antigen.
• No cure
• Treatment goal: relieve symptoms and protect organs by
decreasing inflammation and/or the level of autoimmune
activity in the body.
• Treatment options:
 Rest/Sleep
 Nonsteriodal anti-inflammatory drugs (taken with
medication to prevent ulcers)
 Corticosteriods (more potent than NSAIDs in reducing
inflammation)
 Hydroxychloroquine (anti-malarial)
 Cytotoxic drugs (immunosuppressive medications)
  Anaphylactic shock is a severe, potentially life-
threatening allergic reaction. It can occur
within seconds or minutes of exposure to
allergen.
 Skin: 90 %of episodes
⁻ Generalized hives.
⁻ Itching or flushing.
⁻ Swollen lips-tongue-uvula.
⁻ Per-orbital edema.
 Respiratory: 70 % of
episodes
⁻ Nasal discharge and
congestion.
⁻ Change in voice quality.
⁻ Sensation of throat closure
or choking.
⁻ Tridor or wheeze.
⁻ Shortness of breath and
cough.
 Gastrointestinal: 45% of
episodes
⁻ Nausea.
⁻ Vomiting.
⁻ Diarrhea.
⁻ Crampy abdominal pain.
 Cardiovascular: 45 % of
episodes
⁻ Hypotonia (collapse).
⁻ Syncope.
⁻ Dizziness.
⁻ Tachycardia.
⁻ hypotension.
 Serum or plasma total tryptase 
normal range 1 to 11.4 ng/mL
 Plasma histamine
We don’t rely on
laboratory
findings on such
cases only clinical
presentation
 Epinphrin
 IM 0.3mg , 1:1000
 IV 0.15mg , 1:10,000
 Antihestsmin
 Corticosteroid
 Bronchodilator
 Oxygen
 Fluids and vasopressors, why?
Ankylosing
spondylitis
It is a form of arthritis that is long-lasting (chronic) and most
often affects the spine.
It affects joints in the spine and the sacroilium in the pelvis,
causing eventualComplete fusion results in a complete rigidity of
the spine, a condition known as bamboospine
 AS is a systemic rheumatic disease and is one of the
seronegative spondyloarthropathies AS is a systemic
rheumatic disease and is one of the seronegative
spondyloarthropathies
 mild to severe back and buttock pain that is often
worse in the early morning hours.
-This pain usually gets better with
activity. Continued inflammation of the:
-ligaments,
-tendons,
-joint capsules (soft tissues surrounding the
joint),
-and joints of the spine
 -cause the spine to fuse together (ankylose) as the
joints and disc spaces are replaced by bone.
-leading to less motion in the neck and
low back
 a blood test for the HLA-B27 gene
 X-ray
-which show characteristic spinal changes
and sacroiliitis
 tomography and magnetic resonance imaging
of the sacroiliac joints
 Schober's test
-a useful clinical measure of flexion of the
lumbar spine performed during examination.
X-ray demonstrating in
ankylosing spondylitis
Treatment:-
No cure is known for AS!
treatments and medications are available to reduce
symptoms and pain.
Surgical Management:
may include OSTEOTOMY for marked deformities of
the hip/spine.
occasionally, hip or knee ARTHROPLASTY is used.
- if there is severe arthritis of those joints.

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systemic erythematous,urtecariya

  • 1. SYTEMIC LUPUS ERYTHEMATOUS,URTECERIYA SYSTEMIC SCLERODERMA,ANAPHELACTIC SHOCK
  • 2. A chronic inflammatory condition caused by an autoimmune disease. An autoimmune disease occurs when the body's tissues are attacked by its own immune system. Patients with lupus have unusual antibodies in their blood that are targeted against their own body tissues.
  • 3. Inate Susceptibility •HLA type •Immunoregulatory genes •Hormonal levels •Complement levels Environmental Stimuli •UV exposure •Microbial response •Medication Autoimmune Proliferation •Hyperactive B-cell/T-cell activation •High ratio of CDA;CD8 T-cells •Defective immune complex clearance •Impaired tolerance Autoantibody Production •Apoptosis and self exposure •Self-recognition •Foreign-Ab cross reaction
  • 4.  fever, rash, arthritis, alopecia, and renal involvement Symptoms Occurrence Achy joints (arthralgia) 95% Fever more than 38 degrees C 90% Rheumatoid arthritis 90% Prolonged or extreme fatigue 81% Skin rashes 74% Anaemia 71% Kindey involvement 50% Pain in the chest on deep breathing (pleurisy) 45%
  • 5. Find out more about Asthma TreatmentSystemic Lupus Erythematosus
  • 6.
  • 7.  Affects 85% of patients with SLE  50% of patients have anemia  Between 34 - 42% of patients have antiphospholipid syndrome (APS)  Patients who have APS have a high incidence of blood clots  Blood clotting puts patients at higher risk for stroke and pulmonary embolism
  • 8.
  • 9. • Heart disease is primary cause of death in lupus patients • Atherosclerosis, or plaque buildup in the arteries • High blood pressure, most likely because of kidney injury and corticosteroid treatments • Heart failure • Pericarditis, an inflammation of the tissue surrounding the heart • Myocarditis, an inflammation of the heart muscle itself (rare)
  • 10.  Affects 50% of patients  Poor kidney function (mild) and kidney failure (severe) may result from this damage.  Serious complications occur in 30% of patients
  • 11.  Infections (common)  Gastrointestinal (45%)  Joint, Muscle, Bone (Osteoporosis, Arthritis)  Eye (5% temporary blindness)
  • 12.  Difficult to diagnose  No single diagnostic marker; identified through a combination of clinal and laboratory criteria  Early diagnosis is important as it reduces morbidity and mortality (lupus nephritis)
  • 13.  4 of 11 clinical and laboratory criteria must be met  Antinuclear antibody titer is the primary laboratory test  Antinuclear antibody titer of 1:40 and characteristic multiorgan system involvement can be diagnosed with systemic lupus erythematosus without additional testing  Patients with an antibody titer of 1:40 who fail to meet full clinical criteria should undergo additional testing: including tests for antibody to doublestranded DNA antigen and antibody to Sm nuclear antigen.
  • 14. • No cure • Treatment goal: relieve symptoms and protect organs by decreasing inflammation and/or the level of autoimmune activity in the body. • Treatment options:  Rest/Sleep  Nonsteriodal anti-inflammatory drugs (taken with medication to prevent ulcers)  Corticosteriods (more potent than NSAIDs in reducing inflammation)  Hydroxychloroquine (anti-malarial)  Cytotoxic drugs (immunosuppressive medications)
  • 15.   Anaphylactic shock is a severe, potentially life- threatening allergic reaction. It can occur within seconds or minutes of exposure to allergen.
  • 16.
  • 17.  Skin: 90 %of episodes ⁻ Generalized hives. ⁻ Itching or flushing. ⁻ Swollen lips-tongue-uvula. ⁻ Per-orbital edema.  Respiratory: 70 % of episodes ⁻ Nasal discharge and congestion. ⁻ Change in voice quality. ⁻ Sensation of throat closure or choking. ⁻ Tridor or wheeze. ⁻ Shortness of breath and cough.  Gastrointestinal: 45% of episodes ⁻ Nausea. ⁻ Vomiting. ⁻ Diarrhea. ⁻ Crampy abdominal pain.  Cardiovascular: 45 % of episodes ⁻ Hypotonia (collapse). ⁻ Syncope. ⁻ Dizziness. ⁻ Tachycardia. ⁻ hypotension.
  • 18.  Serum or plasma total tryptase  normal range 1 to 11.4 ng/mL  Plasma histamine We don’t rely on laboratory findings on such cases only clinical presentation
  • 19.  Epinphrin  IM 0.3mg , 1:1000  IV 0.15mg , 1:10,000  Antihestsmin  Corticosteroid  Bronchodilator  Oxygen  Fluids and vasopressors, why?
  • 21. It is a form of arthritis that is long-lasting (chronic) and most often affects the spine. It affects joints in the spine and the sacroilium in the pelvis, causing eventualComplete fusion results in a complete rigidity of the spine, a condition known as bamboospine  AS is a systemic rheumatic disease and is one of the seronegative spondyloarthropathies AS is a systemic rheumatic disease and is one of the seronegative spondyloarthropathies
  • 22.  mild to severe back and buttock pain that is often worse in the early morning hours. -This pain usually gets better with activity. Continued inflammation of the: -ligaments, -tendons, -joint capsules (soft tissues surrounding the joint), -and joints of the spine  -cause the spine to fuse together (ankylose) as the joints and disc spaces are replaced by bone. -leading to less motion in the neck and low back
  • 23.  a blood test for the HLA-B27 gene  X-ray -which show characteristic spinal changes and sacroiliitis  tomography and magnetic resonance imaging of the sacroiliac joints  Schober's test -a useful clinical measure of flexion of the lumbar spine performed during examination.
  • 25. Treatment:- No cure is known for AS! treatments and medications are available to reduce symptoms and pain. Surgical Management: may include OSTEOTOMY for marked deformities of the hip/spine. occasionally, hip or knee ARTHROPLASTY is used. - if there is severe arthritis of those joints.

Editor's Notes

  1. Although the specific cause of SLE is unknown, multiple genetic predispositions and gene-environment interactions have been identified (see chart below). This complex situation perhaps explains the variable clinical manifestations in persons with SLE.
  2. http://emedicine.medscape.com/article/332244-overview#aw2aab6b2b5
  3. Can cause systemic complications
  4. Can cause systemic complications
  5. Can cause systemic complications
  6. Infections: streptococcal but also bacterial and fungal infections Gastrointestinal: nausea, weight loss, diarrhea
  7. http://www.aafp.org/afp/2003/1201/p2179.html
  8. http://emedicine.medscape.com/article/332244-overview#aw2aab6b2b5
  9. http://emedicine.medscape.com/article/332244-overview#aw2aab6b2b5