Churg Strauss Syndrome

Question 1

What are the clinical features of Churg-Strauss Syndrome?

Answer 1

1. Prodrome Phase
   - Allergic rhinitis; asthma; allergic rash
   - Lasts several years
   - Hence patients are often misdiagnosed with an allergy
2. Eosinophilia & Tissue Infiltration
   - Infiltration of various organs – heart, lung, GI etc.
   - Multi-organ involvement -> similar to many other vasculitis
3. Vasculitis

Question 2

What is the criterior for diagnosis of Churg strauss syndrome?

Answer 2

Criteria (presence of 4 or more of the criteria)

• Asthma
• Paranasal sinus abnormality
• Pulmonary infiltrates, non-fixed
• Polyneuropathy or mononeuritis multiplex
• Eosinophilia more than 10% in peripheral blood
• Extravascular eosinophils accompanying vasculitis on histology

Question 3

What is the investigations for Churg strauss syndrome?

Answer 3

• Eosinophilia – up to 60% of WCC (Total Whites)
• ESR, CRP raised
• MPO ANCA usually positive – p-ANCA pattern

Question 4

What is the management of Churg Strauss syndrome?

Answer 4

• Typically responds well to corticosteroids alone

- In neuropathy or glomerulonephritis – cyclophosphamide to induce remission

Question 5

Essentially, just remember:

• Initially, patient presents with lots of allergic symptoms: rhinitis, asthma, rash -> hence often mis-diagnosed with _______
• CSS has multi-organ involvement, like polyarteritis nodosa
• Bloods will show elevated Eosinophils, and Histology will show _________
• ________ +ve
• Treatment is “Cs” – Corticosteroids and Cyclophosphamide (only if _____________)

Answer 5

ATOPY;

granulomatous infiltrates;

P-ANCA;

neuropathy / GN