Churg-Strauss syndrome (allergic granulomatosis)

Churg–Strauss syndrome (allergic granulomatosis)

Definition

Multisystem disorder consisting of asthma and rhinitis, eosinophilia, eosinophilic infiltration, small vessel vasculitis and extravascular granulomas.

Age

Peak incidence 30–40 years.

Sex

M = F

Aetiology/pathophysiology

It is thought to be an autoimmune disorder.

Clinical features

Prodromal disorder of allergic rhinitis and asthma.

Peripheral blood eosinophilia and eosinophilic infiltration of the lung and gastrointestinal tract.

Systemic vasculitis of the medium and small vessels with vascular and extravascular granulomatosis. Affected organs include the skin with subcutaneous nodules, purpura and haemorrhages; heart with pericarditis, cardiac failure and myocardial infarction; neurological system with a mononeuritis multiplex; kidney with a focal segmental glomerulonephritis; gastrointestinal system with an eosinophilic gastroenteritis and musculoskeletal system causing myalgia and migratory polyarthritis.

Investigations

Chest X-ray may show patchy pneumonia-like shadow-ing, which can be fleeting. The ESR is raised and there are raised levels of immunoglobulins. Anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies (pANCA) is pos-itive in 70%. The diagnosis can be confirmed on biopsy of affected organs.

Management

Treatment involves steroids and azathiporine.