Systemic lupus erythematosus is a rare, chronic autoimmune disease that can affect multiple organ systems. It is characterized by hyperactivity of B lymphocytes and autoantibodies that can cause tissue damage. Common symptoms include arthritis, skin rashes, kidney issues, and fatigue. Diagnosis requires meeting certain criteria related to constitutional symptoms, skin and joint involvement, serological markers, and organ system dysfunction. Treatment focuses on managing symptoms and preventing disease flares and organ damage using medications like corticosteroids, immunosuppressants, and hydroxychloroquine. Prognosis has improved but risks of infection and accelerated cardiovascular disease remain higher than the general population.

1. Dr Habeeb
Resident
Medicine Unit-1
BMCH

2. Introduction
Systemic Lupus erythromatosus is a rare, autoimmune based chronic
inflammatory disease of un-known aetiology.
Maybe confined to the skin or may involve multiple organs system.
Some 90% of affected patients are female.
Peak age at onset is 20 to 30 years.
Lupus is associated with morbidity and a five-fold increase in mortality
mainly because of an increased risk of premature cardiovascular
disease.
Most common cause of death are infections and Renal Failure.

3. Pathophysiology
SLE is characterized by hyper-reactivity of B
lymphocytes, hypergammaglobulinaemia,
multiple autoantibodies, circulating immune
complexes, and complement activation (low C3 ,
C4).
Autoantibodies to self antigens, increased
apoptosis and impaired clearance of apoptotic
bodies play an important role. Tissue damage is
the result of immune complex deposition as well
as direct cellular injury.

4. History and Examination…..
1. Constitutional Symptoms
Patient may present with
symptoms such as Fever, Weight
loss , fatigue and malaise

5. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
Arthralgia is a common
symptom, occurring in 90% of
patients, and is often associated
with early morning stiffness,
joint deformity may occur but
erosion does not.

6. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
Raynaud’s associated with SLE
needs to be differentiated from
primary Raynaud’s, which is
common in healthy young
women.

7. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
4. Skin involvement
3 types of skin lesions:
Malar Rash classically butterfly
rash, Discoid Rash which is
scaring and may cause Alopecia
if on scalp & subacute cutaneous
lupus erythromatosus rash which
is migratory and non-scaring

8. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
4. Skin involvement
5. Kidney
Typical renal lesion is a
proliferative glomerulonephiritis
, characterized by haematuria,
proteinuria and casts on urine
microscopy.

9. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
4. Skin involvement
5. Kidney
6. Cardiovascular
Pericarditis, Myocarditis & less
commonly Libman–Sacks
endocarditis can occur. Increased
risk of stroke & MI due to
increased atherosclerosis.

10. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
4. Skin involvement
5. Kidney
6. Cardiovascular
7. Lungs
Lung involvement manifests as
pleurisy or pleural effusion.
Other features include
pneumonitis, atelectasis,
reduced lung volume and
pulmonary fibrosis.

11. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
4. Skin involvement
5. Kidney
6. Cardiovascular
7. Lungs
8. Neurological
Fatigue, headache and poor
concentration are common.
More specific features of
cerebral lupus include visual
hallucinations, chorea, organic
psychosis, transverse myelitis
and lymphocytic meningitis.

12. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
4. Skin involvement
5. Kidney
6. Cardiovascular
7. Lungs
8. Neurological
9. Hematological
Neutropenia, lymphopenia,
thrombocytopenia or
haemolytic anaemia may occur,
due to antibody mediated
destruction of peripheral blood
cells.

13. History and Examination…..
1. Constitutional Symptoms
2. Arthritis
3. Raynaud’s Phenomenon
4. Skin involvement
5. Kidney
6. Cardiovascular
7. Lungs
8. Neurological
9. Hematological
10.Gastrointestinal
Mouth ulcers may occur and may
or may not be painful.
Mesenteric vasculitis is a serious
complication, which can present
with abdominal pain, bowel
infarction or perforation.

15. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Fixed erythema, flat or raised over the malar
eminences, sparing the nasolabial folds

16. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Oral or nasopharyngeal ulceration, usually
painless

17. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Erythematous raised patches with adherent
keratotic scaling and follicular plugging;
atrophic scarring may occur in the older lesions

18. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Haemolytic anaemia, leucopaenia (<4000 mm),
lymphopaenia (<1500/mm),
thrombocytopaenia(<100,000/mm

19. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Persistent proteinuria greater than 0.5 gm/day
or greater than 3+ on dipstick
or Cellular casts

20. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Seizures or psychosis, other causes ruled out

21. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Skin rash on exposed areas as a result of
unusual reaction to sunlight

22. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Non-erosive arthritis involving two or more
peripheral joints, characterised by tenderness,
swelling or effusion

23. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Anti-DNA antibodies in abnormal titre or presence of
antibody to Sm antigen or positive antiphospholipid
antibodies

24. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
Pleuritis, by history of pleuritic pain or rub
heard by a physician or evidence of pleural
effusion on CXR or CT or Pericarditis by ECG
or evidence of pericardial effusion

25. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
A significant titre by ImmunoFlurescent
method in the absence of the other causes of
positive ANA

26. Diagnostic Criteria
1. Malar Rash
2. Oral Ulcers
3. Discoid Rash
4. E “Hematological”
5. Renal Involvement
6. Neurological
7. Photosensitivity
8. Arthritis
9. Immunological
10.Serositis
11.ANA
Mnemonic “Modern Paisa” to memorize the criteria
A significant titre by ImmunoFlurescent
method in the absence of the other causes of
positive ANA

27. Workup…

28. Workup…
 Blood CP Anemia,Leukopenia &
Thrombocytopenia
which characterize
active Lupus
Following biochemical investigations are routinely carried out:

29. Workup…
 Blood CP
Urine Analysis
Hematuria,
Proteinuria,
Casts
Following biochemical investigations are routinely carried out:

30. Workup…
 Blood CP
Urine Analysis
Creatinine
Routinely done to
assess Kidney
function
Following biochemical investigations are routinely carried out:

31. Workup…
 Blood CP
Urine Analysis
Creatinine
ESR
Routinely done to
assess Kidney
function
Following biochemical investigations are routinely carried out:

32. Workup…
These are repeated periodically (monthly or at longer intervals).
Immunology investigations
• ANA (not required to be repeated)
• Anti-dsDNA
• ENAs (Antibodies to extractable nuclear an(SSA), La (SSB), Smith etc.
• C3, C4
• Antiphospholipid antibodies
Others:
X-ray chest

33. Investigations Depending on Systemic Involvement
Workup…

35. Disease Activity Assessment
 Periodic CBC and urine analysis are mandatory.
 Active lupus is characterised by anaemia, leucopaenia,
lymphopaenia, and thrombocytopaenia
 Proteinuria and active urinary sediment point to active
glomerulonephritis.
 Rising anti-dsDNA titres and falling C3 C4 levels.

37. Treatment

38. Prevention
All patients with lupus erythematosus
should be counseled on photoprotection,
including protecting skin from sunlight
and avoiding sun exposure during peak
hours (i.e., between 10 AM and 2 PM).
Broad-spectrum sunscreen that contains
titanium, zinc, Mexoryl (L’Oreal), or
Helioplex
(Neutrogena) should be used whenever
patients are outdoors.
Photoprotective clothing, available from
multiple vendors, is useful for limiting sun
exposure.

39. Medium-potency topical corticosteroids
Use of triamcinolone 0.1% cream (Flutex) for lesions on the
head and neck up to 2 weeks
Foam or liquid- or lotion based corticosteroids for the scalp
lesions.
Intralesional corticosteroids may cause mild discomfort,
atrophy of the skin or subcutis, or stretch marks.
Topical calcineurin inhibitors such as pimecrolimus (Elidel) or
tacrolimus (Protopic) may be used for maintenance treatment
 Recurrent or refractory lesions require systemic treatment.
Treatment of Cutaneous Lupus Erythematosus

40. Antimalarials hydroxychloroquine (HCQ) are disease-
modifying agents that limit the progression of lupus.
Hydroxychloroquine at 200 mg daily for 2 weeks and then
increased to 400 mg daily.
Hydroxychloroquine exerts its effects within 2 to 3 months
of beginning treatment.
Treatment of Systemic Lupus Erythematosus

41. • Depending on end-organ involvement in SLE,
immunosuppression with systemic corticosteroids such as
prednisone at doses of 1 mg/kg/day is appropriate.
• Steroid-sparing drugs such as methotrexate , acitretin or
mycophenolate mofetil are added.
Treatment of Systemic Lupus Erythematosus cont……

42. Arthralgia can usually be managed with acetaminophen or
nonsteroidal antiinflammatory drugs (NSAIDs).
Hydroxychloroquine 200 mg twice daily can be added.
Methotrexate 7.5 to 25 mg PO once weekly with folic acid 1mg
daily .
Azathioprine (Imuran) 0.5 to 2 mg/kg with monitoring of CBC
& LFTs.
Low-dose glucocorticoids (prednisone 5–10 mg/day) may be
used as a bridge to steroid-sparing therapy and to treat
intermittent flares.
Treatment of Musculoskeletal Manifestations

43. Lupus Nephritis is one of the severe manifestation of renal
disease and Treatment should be coordinated with a
rheumatologist or nephrologist.
Class I disease no therapy.
 Class II can be treated with prednisone (20 mg/day for 6 weeks
to 3 months)
Class III and IV disease treated with prednisone(1 mg/kg/day)
for 6 weeks ,maintenance of 10 to 15 mg/day.
In addition cytotoxic therapy with Cyclophosphamide 0.5 to 1
g/m BSA monthly for 6 months and tapered to every 3 months
Class V disease can be treated with prednisone alone, similar to
class II disease
Treatment of Renal Manifestations

44. Neuropsychiatric Symptoms can range from mild to severe
headache, aseptic meningitis, neuropathy, myelopathy,
cognitive dysfunction, seizures, cerebritis, and stroke.
For seizures, antiepileptic therapy is used.
Lupus cerebritis and transverse myelitis are two of the more
serious manifestations that need to be treated emergently with
aggressive immunosuppression.
 Treatment includes high-dose corticosteroids and
cyclophosphamide, similar to treatment for lupus nephritis.
Treatment of Nervous System Manifestations

45. SPECIAL PROBLEMS
Fertility, Pregnancy, Contraception
Infections
SLE in Elderly

46. • Steroids, hydroxychloroquine, and azathioprine can be
continued during pregnancy.
• Cyclophosphamide, methotrexate, and mycophenolate
should be discontinued 3 to 6 months before conception.
• Cyclophosphamide therapy carries the risk of age
dependent ovarian failure.
• Intrauterine contraceptive devices should be avoided.
• Oestrogen-containing contraceptives should be avoided
with APS
Fertility, Pregnancy, Contraception

47. • Patients with SLE are particularly susceptible to infections
• Candida, Herpes, Salmonella & Mycobacteria and capsulated
organisms (Pneumococcus, Meningococcus and H.
influenzae).
• Increased susceptibility is due to low complement levels,
prednisolone (>20 mg/d), immunosuppressives, splenic
hypofunction.
• Prevention of infection with chemoprophylaxis and
vaccination is advised.
Infections

48. • SLE in the elderly is a milder disease.
• Characterized by insidious onset, longer duration of disease
• Lower incidence of renal, musculoskeletal, skin and
neurological manifestations.
SLE in Elderly

49. Drug-Induced SLE

50. • ANA, anti-histone antibodies, and LE cells are characteristic
features of drug induced SLE.
• Clinical features are mild; renal and neurological involvement
is rare.
• Symptoms resolve on withdrawal of the offending drug
• Antibody titres may continue to remain elevated for a long
time.
Drug-Induced SLE

51. Drug-Induced SLE
o Procainamide
o Quinidine
o Hydralazine
o Methyldopa
o Chlorpromazine
o Isoniazid
More commonly
Less common