systemic%20lupus%20erythematosus%20(pediatric)
SYSTEMIC LUPUS ERYTHEMATOSUS (PEDIATRIC)
Systemic lupus erythematosus is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against self-antigens and immune-complex formation.
It can be suspected when ≥2 organ systems are involved.
It is predominantly diagnosed in females of childbearing age, rarely diagnosed before 8 years old.
Clinical presentation varies in different patients and the disease activity varies over time in a single patient. Majority of patients have arthralgia of the hand.

Principles of Therapy

Patients require individualized therapy depending on disease manifestations, activity & severity

Goals of therapy

  • Control disease manifestations
  • Allow the child to have a good quality of life w/o major exacerbations
  • Prevent serious organ damage that adversely affects function or life span
  • Prevent adverse effects of the drugs used

Pharmacotherapy

Corticosteroids

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
  • Used as initial therapy for severe discoid lupus erythematosus or lupus vasculitis
  • Low-dose corticosteroids may be added to Hydroxychloroquine for fatigue & fever
  • High-dose corticosteroids are necessary for refractory manifestations of SLE & for severe organ systems’ manifestations esp CNS, renal & hematologic manifestations
    • Patients w/ milder forms of renal manifestations (eg mesangial glomerulonephritis & focal proliferative glomerulonephritis) may respond to corticosteroids
    • Show improved survival in patients w/ severe forms of SLE nephritis
    • May also be useful in severe, life-threatening thrombocytopenia & hemolytic anemia
    • May also be useful in pleuritis or pericarditis
  • Decreases inflammation by suppression of the immune system
    • Decreases lymphocyte volume & activity, PMN migration, capillary permeability
    • High doses over periods >2-3 wk, suppress adrenal function
  • Corticosteroid use should be tapered, & if possible withdrawn, as soon as desired response is observed (control of inflammatory manifestations) to avoid toxicity
Topical Corticosteroids
  • Helpful for discoid lesions esp on the scalp
  • Use a less potent steroid on the face because it is more prone to atrophy
Parenteral Corticosteroids
  • Pulse therapy w/ IV corticosteroids in combination w/ immunosuppressive therapy is recommended for ClassIII/IV SLE patients w/ confirmed glomerulonephritis
Hydroxychloroquine
  • Used for skin & joint manifestations
    • Useful for patients w/ skin disease that are unresponsive to topical corticosteroids & in patients w/ arthritis that does not respond to NSAID
  • 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/IV SLE patients w/ nephritis
    • Patients w/ continuing treatment w/ Hydroxychloroquine showed less renal damage as compared to those on placebo

Immunosuppressants

Choice of immunosuppressant will depend on nature & severity of disease manifestation

  • 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 w/ corticosteroids allows lower doses of immunosuppressants
Azathioprine
  • May be used as the initial immunosuppressant for SLE nephritis
  • Antagonizes purine metabolism & inhibits synthesis of DNA, RNA & proteins
  • An alternative to Cyclophosphamide & a steroid-sparing agent in nonrenal disease
  • May decrease proliferation of immune cells, resulting in lower autoimmune activity
Belimumab
  • The first US FDA-approved biologic therapy for the treatment of SLE
  • A human monoclonal antibody that binds to soluble B-lymphocyte stimulator (BLyS/BAFF) to reduce disease activity
  •  Used for active, autoantibody-positive SLE patients currently on standard therapy
  • Not recommended for patients w/ severe active lupus nephritis or severe active CNS disease
  • Studies have shown that patients on Belimumab therapy experienced reduced risk of severe flares, improved health-related quality of life & reduced steroid use as compared to those on placebo
Cyclophosphamide
  • Used as the initial immunosuppressant for SLE nephritis
    • Concomitant use w/ Prednisone is the standard treatment for lupus nephritis as it helps preserve renal function
  • Useful for severe SLE w/ involvement of different organ systems (eg severe CNS involvement, vasculitis or lupus nephritis)
  • As an alkylating agent, may involve cross-linking of DNA, which may interfere w/ growth of normal & neoplastic cells
Intravenous immune globulin (IVIg)
  • Used as immunosuppression in serious SLE flares
  • Neutralizes circulating myelin antibodies through anti-idiotypic antibodies
    • Down-regulates proinflammatory cytokines, including interferon-gamma
    • Blocks receptors on macrophages, suppresses inducer T & B cells & augments suppressor T cells
    • Blocks complement cascade, promotes remyelination & may increase colony stimulating factor Immunoglobulin G
Methotrexate
  • May be used as the initial immunosuppressant for severe arthritis
  • Blocks purine synthesis, increases anti-inflammatory adenosine concentration at sites of inflammation
Nonsteroidal anti-inflammatory drugs (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 w/ SLE should apply sunscreen w/ at least an SPF of 15 to prevent dermal or systemic disease flares upon exposure to ultraviolet light



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