By Dr.P.W.G Chamika
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare
systemic necrotizing vasculitis affecting small and medium sized vessels.
This multisystem disease is typically characterized by 3 phases which
include prodromal phase, eosinophilic phase and a vasculitic phase.
American College of Rheumatology (ACR), states ≥4/6 criteria are needed
for the diagnosis: peripheral eosinophilia (> 10%), paranasal sinusitis,
pulmonary infiltrates, histological evidence of vasculitis with extravascular
eosinophils, and mononeuritis multiplex or polyneuropathy. Asthma and
lung disease are the cardinal clinical features of EGPA. Neurological
involvement in EGPA is rare.
This is a unique case of a 31 year old patient presenting
with bilateral spastic paraparesis with bladder and autonomic involvement,
who was initially diagnosed as having transverse myelitis, on the tenth day
following an appendectomy, later being diagnosed as having EGPA with
CNS vasculitis. This is an atypical presentation of EGPA with CNS, gut and
dermatological involvement at the diagnosis without lung or upper airway
involvement. ANCA was negative. Remission induced with
cyclophosphamide and methylprednisolone pulse therapy. Maintenance
therapy was continued with azathioprine.
CNS vasculitis due to EGPA is rare. EGPA may not always
follow the typical triphasic pattern of the disease. Prompt identification of
the disease with atypical presentations and early use of cytotoxic therapy
would improve the outcome.
Key words: EGPA, Churg Strauss, CNS vasculitis, Case report, appendectomy, Sri lanka