Systemic Lupus
Erythematosus A431
Manduul
Sandagsuren
Bat-Ider
Introduction
• SLE is a multisystem autoimmune disease associated with genetic and
environmental risk factors.
• SLE is most common in women and those from non‐white ethnic backgrounds.
• Lupus nephritis occurs in up to 50% of SLE patients.
• SLE patients should receive pre‐pregnancy counselling to ensure optimal
disease control and drug therapy before conception.
• Premature cardiovascular disease is an increasing cause of death in SLE
patients.
Etiology
Clinical manifestation
General features
Fatigue is common. It may be associated with depression or fibromyalgia
secondary to SLE, hypo- thyroidism (often autoimmune in nature), anaemia,
pulmonary or cardiovascular problems. Other constitutional symptoms of active
disease include fever, malaise, anorexia, lymphadenopathy and weight loss.
normochromic normocytic anaemia
Mucocutaneous manifestation
● Mouth ulcers
● Alopecia
● Butterfly or malar rash
● Photosensitivity
● Nasal or vaginal ulcers
Musculoskeletal manifestations
Generalized arthalgia with morning stiffness (no swelling)
Non erosive arthritis with joint tenderness and swelling
Jacoud’s arthropathy (10–35%)
Myalgia (secondary myopathy)
Avascular necrosis, infection
Risk of osteoporosis
Renal manifestation
Early nephritis is often asymptomatic, so regular urinalysis for protein, blood and
casts is essential. Renal biopsy is helpful for assessing the severity, nature, extent
and reversibility of the involvement and is an important guide to treatment and
prognosis.
Nervous system manifestations
● Aseptic meningitis ● Acute inflammatory
● Cerebrovascular disease demyelinating
● Demyelinating syndrome
polyradiculoneuropathy
● Headache (including migraine and
● (Guillain–Barré syndrome)
benign intracranial hypertension)
● Autonomic disorder
● Movement disorders (including chorea)
● Myelopathy ● Mononeuropathy (single or
● Seizure disorders multiplex)
● Acute confusional state ● Myasthenia gravis
● Anxiety disorder ● Neuropathy, cranial
● Plexopathy
● Polyneuropathy
Diagnostic tests
❏ CBC
❏ Urinalysis, serum creatinine
❏ GFR
❏ Antinuclear antibody, anti‐dsDNA
❏ Anti Sm
❏ Anti‐extractable nuclear antigens tests
❏ Complex of anticardiolipin antibodies
❏ Beta‐2‐glycoprotein
Treatment
Goals of therapy:
● Control disease manifestation
● Allow child to have a good quality of life without major exacerbations
● Prevents serious organ damage that adversely affects function or lifespan
● Prevent adverse effects of the drugs
Pharmacotherapy- Corticosteroids
- Immnosuppressants
- NSAIDs
- Sunscreen
Oral corticosteroids
● Patients w/ mild SLE do not normally require use of systemic corticosteroids
but there are patients who has low quality of life if not given low-dose
corticosteroids
● Lowest possible dose should be used for maintenance therapy
● High-dose corticosteroids are necessary for refractory manifestations of SLE
& for severe organ systems' manifestations especially CNS, renal &
hematologic manifestations
● Decreases inflammation by suppression of the immune system
Topical corticosteroids
● Helpful for discoid lesions especially on the scalp
● Use a less potent steroid on the face because it is more prone to atrophy
Parenteral corticosteroids
● Pulse therapy with IV corticosteroids in combination with immunosuppressive
therapy is recommended for Class IIl and IV SLE patients with confirmed
glomerulonephritis
HYDROXYCHLOROQUINE
● Used for skin & joint manifestations
● Also used for preventing flares & other constitutional symptoms
● Inhibits chemotaxis of eosinophils & locomotion of neutrophils & impairs
complement-dependent antigen-antibody reactions
● Recommended as background treatment for Class III and IV SLE patients
with nephritis
IMMUNOSUPPRESSANTS
● These agents act as immunosuppressive, cytotoxic & anti-inflammatory
agents
In the treatment of severe CNS & severe glomerulonephritis,
thrombocytopenia & hemolytic anemia, high dose glucocorticoids &
immunosuppressantS are used
● Concomitant use with corticosteroids allows lower doses of
immunosuppressants
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[Link] Immune Globulin (IVig)
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NSAIDS
● These drugs provide symptomatic relief of fever, arthritis & mild serositis
Inhibit inflammatory reactions & pain by decreasing prostaglandin synthesis
SLE patients have a high incidence of NSAID-induced hepatotoxicity
SUNSCREEN
● Patients with SLE should apply sunscreen with at least an SPF of 15 to
prevent dermal or systemic disease flares upon exposure to ultraviolet light
Treatment of non-renal SLE—recommended drugs with respective grading of recommendation. aPL, antiphospholipid
antibodies; AZA, azathioprine; BEL, belimumab; BILAG: British Isles Lupus Assessment Group disease activity index; CNIs,
calcineurin inhibitors; CYC, cyclophosphamide; GC, glucocorticoids; HCQ, hydroxychloroquine; IM, intramuscular; MMF,
mycophenolate mofetil; MTX, methotrexate; Pre, prednisone; PO, per os; RTX, rituximab; PLTs: Platelets; SLEDAI, Systemic
Lupus Erythematosus Disease Activity Index.
Thank you for your attention :)