Churg-Strauss syndrome (CSS)
- ANCA - positive granulomatous small-vessel vasculitis of unknown aetiology
- M=F
Differential Dx
- Eosinophilic disorders
- leukemia
- eosinophilic fasciitis
- Löffler syndrome
- Vasculitis
- Wegener granulomatosis
- Microscopic polyangiitis
- Polyarteritis nodosa (PAN)
- Goodpasture syndrome.
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
Labs
- Eosinophilia (>10%) (eospinophilic infiltration of most organs)
- Anaremia "of chronic desease", raised infalmmatory markers
- Positive Rheumatoid factor and anti-nuclear antibodies
- Complement normal
Treatment
- Steroids (+/- Cyclophosphamide)
- Maintenance Azothioprine or MTX
