Connective Tissue Diseases - SLE

The protein collagen makes up 30% of the body's connective tissue. Connective Tissue (CT) diseases are autoimmune diseases that attack the connective tissues and are characterized by inflammation of the skin, blood vessels, joints and other organs where connective tissues are abundant. CT diseases are also referred to as Collagen Vascular diseases. The prototypical connective tissue disease is Systemic Lupus Erythematous (SLE).

Systemic Lupus Erythematosus

SLE may present in a similar fashion to RA with symptoms of a chronic, inflammatory polyarthritis associated with systemic symptoms. Young women are frequently affected. SLE may be distinguished from RA by characteristic skin findings including photosensitivity and a malar (facial) "butterfly" rash. Joint inflammation occurs affecting small joints in a symmetrical fashion, however deformity is rare.

Classification criteria for SLE

"SOAP BRAIN MD": Any person having 4 or more of the 11 criteria is considered to have SLE.
  • S = serositis
  • O = oral ulcers
  • A = arthritis
  • P = photosensitivity
  • B = blood disorder
  • R = renal disease
  • A = ANA positive
  • I = immunological disorder
  • N = neurological disease
  • M = malar rash
  • D = discoid rash

The Classification Criteria for SLE are summarized on the ACR webpage.

Review the case below by working through the various headings on the left.

History
Physical Examination
Rash
Mouth ulcers
Laboratory
History

A 36-year old female is seen by her dermatologist for a facial rash worse since a holiday in Cuba three months ago. She mentions that she has also noticed some mouth ulcers that appear every 4-6 weeks and that her hairdresser recently commented on some patchy hair loss. A detailed review of systems reveals episodes of hand and foot pain over the past year interfering with her job as a court reporter. She is stiff in the morning for approximately 90 minutes.