Systemic Lupus Erythematosus (SLE)

1. Case 2
Presenter: Layla Almzraq

2. The Case
• A 40 years-old women present with a butterfly rash on
her face.
• She does not use any medication.
• Other symptoms include arthralgia, alopecia and fatigue.
• There is no fever.

3. Learning Objectives:
1. Which diagnosis is the most likely in view of the clinical
presentation?
2. Describe the extent to which other organs can be involved in this
disorder?
3. Which laboratory test would you order to establish the diagnosis
in this case, and to mentor the involvement of other organs than
the skin and joint?
4. What initial therapeutic measures do you advice?
5. Suppose there is no involvement of other organs initially. What
advice would you give the patient regarding check-up visits?

4. Learning Objectives:
1. Which diagnosis is the most likely in view of the clinical
presentation?
2. Describe the extent to which other organs can be involved in this
disorder?
3. Which laboratory test would you order to establish the diagnosis
in this case, and to mentor the involvement of other organs than
the skin and joint?
4. What initial therapeutic measures do you advice?
5. Suppose there is no involvement of other organs initially. What
advice would you give the patient regarding check-up visits?

5. The most likely diagnosis
• The most likely diagnosis is SLE (
Systemic Lupus Erythematosus)
Why?
• Because SLE is an inflammatory,
multisystem autoimmune disorder.
• Characterized by arthralgia and
butterfly rash.
Which is consistent with the case.

6. The most likely diagnosis cont…
 Is Inflammatory autoimmune disorder that can affect multiple
systems like brain, heart, lungs, liver, kidneys, blood vessels,
several joints, the overall nervous system and the skin.
 Etiology:
 Unknown. However, some etiological factors may trigger
autoimmune response to a variety of tissue components. For
example:
 Sex hormone (mostly in female>>estrogen).
 UVR (in induce damage to DNA << enhance autoimmune).
 Drugs induce SLE (e.x hydrazine and procainamide).

7. The most likely diagnosis cont…

8. Learning Objectives:
1. Which diagnosis is the most likely in view of the clinical
presentation?
2. Describe the extent to which other organs can be involved in this
disorder?
3. Which laboratory test would you order to establish the diagnosis
in this case, and to mentor the involvement of other organs than
the skin and joint?
4. What initial therapeutic measures do you advice?
5. Suppose there is no involvement of other organs initially. What
advice would you give the patient regarding check-up visits?

9. Other organs can be involved in SLE
• SLE affect many body systems , so the patients may present with
any of the following manifestations:
Constitutional (eg, fatigue, fever, arthralgia, weight changes).
Musculoskeletal (eg, arthralgia, arthropathy, myalgia, frank
arthritis, avascular necrosis).
Dermatologic (eg, malar rash, photosensitivity, discoid lupus).
Renal (eg, acute or chronic renal failure, acute nephritic disease).

10. Other organs can be involved in SLE cont…
• Neuropsychiatric (eg, seizure, psychosis).
• Pulmonary (eg, pleurisy, pleural effusion, pneumonitis,
pulmonary hypertension, interstitial lung disease).
• Gastrointestinal (eg, nausea, dyspepsia, abdominal pain).
• Cardiac (eg, pericarditis, myocarditis).
• Hematologic (eg, cytopenias such as leukopenia,
lymphopenia, anemia, or thrombocytopenia).

11. Learning Objectives:
1. Which diagnosis is the most likely in view of the clinical
presentation?
2. Describe the extent to which other organs can be involved in this
disorder?
3. Which laboratory test would you order to establish the diagnosis
in this case, and to mentor the involvement of other organs than
the skin and joint?
4. What initial therapeutic measures do you advice?
5. Suppose there is no involvement of other organs initially. What
advice would you give the patient regarding check-up visits?

12. Laboratory test
 CBC: show a leucopenia, lymphopenia and/or thrombocytopenia.
Anaemia of chronic disease or autoimmune haemolytic anaemia also
occurs.
 ESR is raised and CRP is usually normal but may be high.
 Urea and creatinine only rise when renal disease is advanced. Low
serum albumin or high urine albumin/creatinine ratio are earlier
indicators of lupus nephritis.
 Autoantibodies: many different autoantibodies may be present in SLE
but the most significant are ANA, anti-dsDNA, anti-Ro, anti-Sm and anti-
La. Antiphospholipid antibodies are present in 25–40%.

13. Laboratory test
 Serum complement C3 and C4 levels are often reduced during active
disease.
 Histology: Characteristic histological and immunofluorescent abnormalities
deposition of IgG and complement) are seen in biopsies from the kidney and
skin.
 Diagnostic imaging:
 CT scans of the brain sometimes show infarcts or haemorrhage with
evidence of cerebral atrophy.
 MRI can detect lesions in white matter which are not seen on CT. However, it
can be very difficult to distinguish true vasculitis from small thrombi.

14. Learning Objectives:
1. Which diagnosis is the most likely in view of the clinical
presentation?
2. Describe the extent to which other organs can be involved in this
disorder?
3. Which laboratory test would you order to establish the diagnosis
in this case, and to mentor the involvement of other organs than
the skin and joint?
4. What initial therapeutic measures do you advice?
5. Suppose there is no involvement of other organs initially. What
advice would you give the patient regarding check-up visits?

15. Treatment of SLE
 General measures:
• The disease and its management should be discussed with the patient.
• Patients advised to avoid excessive exposure to sunlight + reduce
cardiovascular risk factors.

16. Treatment of SLE
 Symptomatic treatment:
• Many patients do not need treatment with corticosteroid or
immunosuppressive agents. Arthralgia, arthritis, fever and serositis all
respond well to standard doses of NSAIDs.
• Topical corticosteroids are effective and widely used in cutaneous
lupus.
• Antimalarial drugs (chloroquine or hydroxychloroquine) help mild
skin disease, fatigue and arthralgias that cannot be controlled with
NSAIDs but patients require regular eye checks.

17. Treatment of SLE
 Corticosteroids and immunosuppressive drugs:
• Short courses of oral corticosteroids are useful in treating severe conditions.
• Renal or cerebral disease must be treated with high dose oral corticosteroids.
• Cyclophosphamide was most commonly used to achieve remission in severe
forms of lupus but is being replaced by mycophenolate mofetil, which has
fewer side-effects.
• Newer agents, which target cells or cytokines in the immune system, these
include: Rituximab(anti-CD20) and belimumab, both monoclonal antibodies
acting against B lymphocytes.

18. Learning Objectives:
1. Which diagnosis is the most likely in view of the clinical
presentation?
2. Describe the extent to which other organs can be involved in this
disorder?
3. Which laboratory test would you order to establish the diagnosis
in this case, and to mentor the involvement of other organs than
the skin and joint?
4. What initial therapeutic measures do you advice?
5. Suppose there is no involvement of other organs initially. What
advice would you give the patient regarding check-up visits?

19. Advice to the patient with no other organs
involvement
 The disease and its management will be discussed with the patient.
 Particularly the effect upon the patient’s lifestyle e.g: debility due to
fatigue.
 Patients are advised to: Avoid excessive exposure to sunlight.
 Periodic follow up and blood tests are required for:
1. Detecting signs and symptoms of new organ-system involvement in
the patient.
2. Monitoring response and adverse reactions to therapies. (steroid side
effects).
Usual protocol is visits are arranged every 3
months (quarterly visits) at least 4 visits
every year.

20. References
• Kumar and Clark's clinical medicine 9th Edition. Chapter 18

21. Thank you
