Allergic angiitis and granulomatosis

Allergic angiitis and granulomatosis, also known as Churg-Strauss syndrome, is characterized by asthma, eosinophilia, non-necrotizing angiitis, and extravascular granulomata, usually with eosinophilic infiltrates. Allergic angiitis and granulomatosis is an uncommon disease. The angiitis is disseminated and involves pulmonary and systemic arteries and veins. Peripheral blood hypereosinophilia and elevation of serum IgE are common.

Pulmonary features in allergic angiitis and granulomatosis are related to underlying asthma which is present in all patients. A phasic pattern of allergic angiitis and granulomatosis syndrome has been described: initial allergic rhinitis, evolving into asthma and followed by peripheral blood eosinophilia, eosinophilic tissue infiltrates, and ultimately vasculitis. A study of 154 patients with allergic angiitis and granulomatosis revealed the following: 84 males, average age of 28 years at onset of allergic rhinitis, average age of 35 years at onset of asthma, and average age of 38 years when vasculitis was diagnosed. Other observations included a mean peak eosinophil count of 12.9 * 109/l, anemia in 83 per cent, granulomas in 40 per cent, tissue eosinophilia in 50 per cent, and vasculitis in more than 70 per cent. Chest radiograph abnormalities are present in over 60 per cent of patients and include patchy and occasionally diffuse alveolar-interstitial infiltrates in the perihilar area. Massive pulmonary alveolar hemorrhage is rare. Respiratory failure is the cause of death in a small percentage, although death from status asthmaticus has been reported in up to 8 per cent. The main causes of death are heart failure, renal failure, cerebral hemorrhage, and gastrointestinal hemorrhage.

Upper airway disease may manifest as allergic rhinitis, nasal polyps, nasal mucosal crusting, and septal perforation in up to 70 per cent of patients. Chronic paranasal sinus pain or tenderness and seasonal allergy are also present.

Treatment of allergic angiitis and granulomatosis consists of high-dose systemic corticosteroids, which can produce a dramatic response. Therapy should be instituted as soon as the diagnosis is established.

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