Connective Tissue Diseases

Connective Tissue Diseases

 

Several autoimmune connective tissue diseases may present with similar features.  These include systemic lupus erythematosus (SLE), Sjögren syndrome, mixed connective tissue disease (MCTD), scleroderma (SS), inflammatory myopathies (polymyositis/dermatomyositis [PM/DM]) and undifferentiated connective tissue disease (UCTD)

Epidemiology

  • Incidence – ranges from 15-50/100,000 depending on disease
  • Age – onset 15-40 years with peak onset 20-30 years
  • Sex – F>M (6-10:1)

Pathophysiology

  • Circulating antigen-antibody complexes affect a variety of organs
  • Multisystem disease presentation; major blood vessels are the predominant target

Clinical Presentation

  • Musculoskeletal – arthralgias, arthritis, synovitis, myopathy
  • Dermatologic – skin rashes, Raynaud phenomena, photosensitivity
  • Cardiopulmonary – pleuritis, pericarditis, fibrosis
  • Renal – proteinuria, glomerulonephritis
  • Otorhinolaryngologic – Sicca syndrome,  oral ulcers
  • Gastrointestinal – gastroesophageal reflux disease (GERD)
  • Neurologic – seizures, encephalopathy
  • Hematologic – cytopenias (involving neutrophils, erythrocytes and platelets)

Treatment

  • Anti-inflammatory based and includes glucocorticosteroids and other drugs such as methotrexate and mycophenolate

See Also