systemic%20lupus%20erythematosus
SYSTEMIC LUPUS ERYTHEMATOSUS
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 diagnosed with a single organ involvement such as lupus nephritis.
It is predominantly diagnosed in females of childbearing age.
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.

Systemic%20lupus%20erythematosus Management

Follow-Up and Monitoring

Lifelong monitoring is required for systemic lupus erythematosus (SLE) patients. Frequency of visits depends on disease activity, severity and extent, response to treatment, type of treatment and monitoring of toxicity
  • The most important tool in the management of SLE is careful, frequent clinical and laboratory evaluation to detect disease flares, appearance of infections and to tailor management based on patient response
    • Do active surveillance of opportunistic infections
    • Adjust current therapy (eg corticosteroids and immunosuppressants) according to the severity of organ involvement during SLE flares 
    • Patients with severe SLE, complications, comorbidities and drug toxicity will require more frequent follow-up
  • Monitoring during clinic visit should include history-taking, physical exam and lab tests (eg CBC, platelet count, creatinine measurement, urinalysis, renal and liver function tests, immunology, ECG, imaging) 
    • For active SLE disease, monitoring can be done every 1-3 months after diagnosis or flare while for low/no disease activity or with stable treatment course, monitoring can be every 6-12 months
  • Physicians may be aided by at least 1 of the following several global or body system-specific indices developed for long-term observation of SLE: BILAG, SLEDAI, ECLAM and PGA
  • Results of laboratory tests that may precede a disease flare:
    • Decrease in serum complement levels
    • Increase in anti-dsDNA
    • Increase in erythrocyte sedimentation rate (ESR)
    • Decrease in hemoglobin level, leukocyte or platelet counts
    • Increase in creatine phosphokinase (CPK) levels
    • Appearance of microscopic hematuria or proteinuria

Toxicity

  • Patients on long-term glucocorticoids should be monitored for:
    • Electrolyte, glucose and lipid levels to identify metabolic conditions
    • Bone densitometry to identify osteoporosis and monitor response to treatment
  • Patients on Hydroxychloroquine should have ophthalmological screening (eg visual fields examination and/or spectral domain-optical coherence tomography) performed at baseline, after 5 years, and then annually in those with no risk factors for retinal toxicity
  • Patients on immunosuppressants should be monitored for hematologic, liver and renal toxicity, and occurrence of infection

Hospital Admission

  • Hospital admission is advised for those:
    • With life-threatening or serious organ system involvement
    • Requiring high-dose IV corticosteroids or chemotherapeutic/immunosuppressant drugs (eg Cyclophosphamide, Mycophenolic acid) 
    • With severe hypertension
    • With unexplained fever to rule out sepsis

Expert Referral

  • Establish good working relationship amongst the primary care physician, rheumatologist, nurse, pharmacist, other healthcare providers, the patient and his/her family
    • Team approach will allow for earlier identification of disease flares and medication toxicity
  • Patient will initially present to a primary care physician who is responsible for:
    • Recognizing the signs and symptoms of SLE in their patients
    • Making an initial diagnosis
    • Referring patient to a rheumatologist and participating in further SLE diagnosis, management and monitoring
  • The rheumatologist is the integral part of the medical team managing an SLE patient
  • Referral to a rheumatologist is indicated for:
    • Confirmation of diagnosis
    • Management plan for patient
    • Periodic evaluation of disease activity and severity
    • Management of uncontrolled disease
    • Management of organ involvement or life-threatening disease
    • Prevention and/or management of treatment toxicities
    • Special situations (eg pregnancy, antiphospholipid syndrome, life-threatening disease, concomitant infection and surgery)
  • Multidisciplinary care involves referral of SLE patients to a psychologist, psychiatrist, physical and/or occupational therapist, ophthalmologist, dermatologist, nephrologist, cardiologist, orthopedic surgeon and other specialists when necessary
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