Granulomatosis With Poyangiitis (Wegeners) (GPA)

Your Path

  • Granulomatosis with poyangiitis (Wegeners) (GPA)

Sites

  • Kidneys
  • Skin
  • Eyes
  • Nasal mucosa/upper respiratory tract, lungs - w/25% GPA affecting respiratory tract only (limited GPA)
  • Peripheral nerves

Etiology

  • Small-to-medium vessel vasculitis

Risk Factors

  • Men = women
  • Average age 40-60

Symptoms

  • Skin erruption
  • F, c, sweats, fatigue
  • Recurrent nasal or sinus congestion, nasal d/c, bleeding
  • Cough, wheezing, SOB, DOE, hemoptysis
  • Ear pain, discharge, decreased hearing
  • Myalgias, arthralgias
  • Peripheral nerve involvement--> extremity numbness, pain, and/or weakness
  • Eye pain, redness, bulging, ophthalmoplegia
  • Lab evidence glomerulonepthritis
  • Often delays in dx as GPA is unusual→ treatedfor more common infectious or inflammatory processes (e.g. sinusitis, bronchitis, pneumonia, otitis)

Physical Exam Findings

  • Nose: nasal mucosal erythema, edema, discharge or ulcer, nasal septum perforation, saddle nose deformity
  • Ear: cartilage redness, swelling, pain, senorineural or conductive hearing loss, middle ear effusion
  • Pulmonary: focal lung findings; findings c/w ILD
  • Skin: papable purpura (vasculitis)
  • Eyes: red eye (scleritis, uveitis), proptosis, ophthalmoplegia
  • Joints: arthritis
  • Peripheral neuropathy related: decreased distal sensation, weakness, diminished reflexes

Tests

Links