ABSTRACT
Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small-
and medium-sized vessels. It is characterized by a constant association with asthma
and eosinophilia, and by the presence of anti-myeloperoxidase (MPO) anti-neutrophil
cytoplasmic antibodies (ANCA) in ~40% of the patients. Vasculitis typically develops
in a previously asthmatic and eosinophilic middle-aged patient and most frequently
involves the peripheral nerves and skin. Other organs, however, may be affected and
must be screened for vasculitis, especially those associated with a poorer prognosis,
such as the heart, kidney, and gastrointestinal tract, as assessed by the recently
revised Five-Factor Score (FFS). Overall survival of CSS patients is excellent, but
relapses are not uncommon and require maintenance or steroid-sparing therapies, depending
on the original FFS-based prognosis at diagnosis. All patients require corticosteroids,
often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide)
and maintenance therapy (azathioprine)], for those with poorer prognoses. Recent insights,
especially concerning clinical differences associated with ANCA status, showed that
CSS patients might constitute a heterogeneous group, both clinically and pathogenically.
Future therapies might reflect these differences more strongly.
KEYWORDS
Churg-Strauss syndrome - vasculitis - ANCA - steroids - immunosuppressants
REFERENCES
loic.guillevin@cch.aphp.fr
