Systemic Lupus Erythematosis

Question 1

What is SLE?

Answer 1

• Systemic lupus erythematosus (SLE) is a
  chronic inflammatory disease of
  unknown cause that can affect virtually
  every organ
• Response to nuclear and cytoplasmic
  antigens
• Requires regular clinical visits and labs
  to monitor and assess

Question 2

Etiology- Pathophysiology of SLE

Answer 2

• Defect in apoptosis, increased cell death, and disturbance of immune tolerance
• directly or indirectly from antibody formation and then immune complexes (IC) form
• T cells: CD8 cytotoxicity, T regulatory, B cell help, migration
  and adhesion all affected

Question 3

Clinical Presentation of SLE

Answer 3

• Presentation and course: highly variable
• Classic: Fever, joint pain and rash in females of childbearing age.
• Other symptoms: Constitutional, musculoskeletal, dermatologic,
  renal, Neuropsychiatric, pulmonary, GI, cardiac, and hematologic.
  Multisystem disease, often with a relapsing-remitting course.
• Disease Flares: Photosensitive rash, non-erosive polyarthritis,
  serositis, and fatigue are common manifestations of disease flares

Question 4

Constitutional sx of SLE

Answer 4

• Fatigue, fever, arthralgia, weight changes are the most common
• Can get fever due to active SLE, infection, or reactions to meds also

Question 5

Musculoskeletal sx of SLE

Answer 5

• Joint pain, arthralgia , myalgia, arthritis (nonerosive, asymmetrical
  possibly and pain disproportionate to swelling)
• PIP, MCP and wrist arthritis most common MSK finding
• 90% of patients experience arthralgias and arthritis

Question 6

Derm sx of SLE

Answer 6

• Lupus diagnostic criteria: Malar rash, photosensitivity, and discoid lupus
• Others not always related: raynaud, urticaria, telangiectasias, bullous
  lesions, panniculitis, vasculitic purpura, livedo reticularis

Question 7

Most commonly involved visceral organ in SLE

Answer 7

Renal

Question 8

_____ is the most common cardiac features of SLR

Answer 8

Pericarditis

Question 9

Cardiac sx seen with SLE

Answer 9

• Heart Failure and Chest pain need to be carefully assessed
• Pericarditis is the most common cardiac features
• Rubs and signs of effusions may be found
• Systolic murmurs are reported in up to 70% of cases
• Libman-Sacks endocarditis with possible digital infarcts and
  splinter hemorrhages

Question 10

Neuro sx seen with SLE

Answer 10

• Most common is HA, altered mood also
• Seizures and psychosis are included in the diagnostic criteria
• Can have 19 other types, but the above are more common

Question 11

Renal sx seen with SLE

Answer 11

• Most commonly involved visceral organ in SLE***
• Glomerular disease is usually asymptomatic and only clinically
  evident in 50% of SLE patients

Question 12

Pulmonary sx seen with SLE

Answer 12

• Pleurisy, effusion, pneumonitis, HTN, interstitial lung diseases
• Chronic steroids increase risk of atypical infections
• Rarely: hemoptysis from alveolar hemorrhage (life threatening)

Question 13

Hematologic sx seen with SLE

Answer 13

• History of multiple cytopenias
• Can be medication caused
• Lymphopenia is common in SLE
• Recurrent early miscarriages clues in on Lupus or APS

Question 14

Ophthalmologic sx seen with SLE

Answer 14

• Not a common 1st complaint, but needs to be checked
• Fundoscopic exam is important
• Medication side effects (ie-hydroxychloroquine can cause
  maculopathy)
• retinal vasculitis can cause blindness

Question 15

Other linked Dz to SLE

Answer 15

• Immunodeficiencies
• Antiphospholipid syndrome
• Fibromyalgia
• Osteonecrosis
• Infections
• Other autoimmune diseases
• SLE is all inclusive
• Malignancies

Question 16

Evaluate all needed areas at each
clinical visits for SLE:

Answer 16

• Depending on system affected: echo,
  PFT’s, CT scans, Biopsies, CXR, MRI, LP,
  arthrocentesis, etc.
• Frequency of exams change depending
  on disease state. Stable=6 month
  follow up; Mild/mod disease=3-4
  months…
• Flares: Mild, Moderate or Severe.

Question 17

Labs for SLE (done at every visit)

Answer 17

• CBC , ESR, CRP, UA with urinary sediment,
  Protein:creatinine ratio, serum creatinine, eGFR,
  anti-dsDNA, Complement levels (C3 and C4)
• (Don’t usually repeat ANA or other specific antibodies
  besides the dsDNA)

Question 18

Nonpharmacologic management of SLE

Answer 18

• Sun Protection
• Diet and nutrition-
• vitamins
• balanced diet
• Exercise
• Many consultations
• Stop smoking
• Immunizations
• Treating comorbid conditions
• Avoid specific meds (ie
  TMP-SMX)
• Contraception during active
  disease

Question 19

Pharmacologic Therapies for SLE

Answer 19

• Mainstay: Hydroxychloroquine (aka- Plaquinil) or
  chloroquine unless contraindicated
• Mild manifestations:
• NSAIDS or short potency immunosuppression meds
  beyond the mainstay and short course steroids
• Moderate Lupus:
• Mainstay plus short term therapy of 5-15mg
  prednisone, taper after above drug takes effect.
• Consider immunosuppressive agent (ie-methotrexate,
  azathioprine, etc) to control also

Question 20

Pharmacologic Therapies for Severe or Life Threatening manifestations of SLE:

Answer 20

• Intensive immunosuppressive therapy
• Short period of high dose systemic glucocorticoids (IV) sometimes with or without other immunosuppressive agents.
• Watch length of time on the meds d/t side effects!!!
• Once controlled, can back off on meds to maintain
  control on less intense treatment

Question 21

Potential Complications of SLE

Answer 21

• CNS
• Renal disease
• Infection due to immunosuppression
• Cardiovascular disease
• Treatment complications
• Malignancies
• Rarely- remission is achieved