Churg-Strauss syndrome (CSS)



Background

  • ANCA - positive granulomatous small-vessel vasculitis of unknown aetiology
  • M=F

Clinical

  • Prodromal "allergic" phase
  • Recurrent sinusitis (75%)
  • Asthma - often "late" onset (and or patchy pulmonary infiltrates)
    • But pulmonary haemorrhage rare, cavitation rare (common in Wegners granulomatosis)
  • Vacsulitic mono- or poly- neuropathy (later CNS vasculitis)
  • Cardiac involvement late (arrhtyhmia, AMI, valvular disease, CCF)
  • Rash on extensor surfaces (purpuric, eosinophilic infiltration)
  • GI and renal vasculitis relatively rare (as opposed to PAN)
  • Migratory arthralgia may herald vasculitic phase

Differential Dx

  • Eosinophilic disorders
    • leukemia
    • eosinophilic fasciitis
    • Löffler syndrome
  • Vasculitis
    • Wegener granulomatosis
    • Microscopic polyangiitis
    • Polyarteritis nodosa (PAN)
    • Goodpasture syndrome.

Labs

  • Eosinophilia (>10%) (eospinophilic infiltration of most organs)
  • Anaemia "of chronic desease", raised infalmmatory markers
  • Positive Rheumatoid factor and anti-nuclear antibodies
  • Complement normal

Treatment

  • Steroids (± Cyclophosphamide)
  • Maintenance Azothioprine or MTX

Content by Dr. Íomhar O' Sullivan 26/01/2012. Last reviewedDr. ÍOS 19/01/15.