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Thursday, October 15, 2009

MRCP Part 1 MCQ question #20

A 28 patient with a 10 year history of bronchial asthma presents with footdrop, he is noted to have purpura and painful skin nodules, the chest radiograph shows several peripheral nodules, these features are characteristic of:

a) Leoffler's syndrome
b) Idiopathic Hypereosinophilic Syndrome
c) chronic eosinophilic pneumonia
d) Churg-Strauss syndrome
e) Allergic bronchopulmonary aspergillosis

Answer:
d) Churg-Strauss syndrome

Explanation
These features are suggestive of Churg-Strauss syndrome, also known as allergic granulomatosis. Almost all patients with allergic granulomatosis and angiitis have a strong history of atopic allergy with preexisting bronchial asthma for an average of 5 to 10 years before diagnosis of the vasculitis. Patients present with fever, shortness of breath, and a variety of complaints related to skin and nerve involvement (e.g., purpura, painful skin nodules, skin infarction, footdrop, wristdrop, or painful neuropathy). Myocardial involvement occurs in a minority of patients but on occasion can dominate the clinical presentation. Wheezing is common. Clinically significant renal involvement is uncommon. In 25% of cases, the chest radiograph shows peripheral infiltrates or nodules that seldom cavitate. Pleural effusion, pericardial effusion, or both may occur with or without infiltrates. Churg-Strauss syndrome, though rare, most often occurs in patients with bronchial asthma. There is an association between Churg-Stauss syndrome and treatment of asthma with leukotriene inhibitors