Churg-Strauss Disease

Pathology

Pathology affects small to medium sized vessels and is characterized by necrotising granuloma, which affects the vessels of the lungs, gastrointestinal (GI) tract, and peripheral nerves.

Aetiology

This pathology is often associated with perinuclear antineutrophil cytoplasmic antibody (pANCA).

Symptoms

• Chest tightness
• Wheeze
• Shortness of breath
• Sinusitis

Signs

The condition has 3 distinct stages:

• Sinusitis and onset of allergies
• Onset of acute asthma
• Systemic involvement including skin purpura, GI upset, and peripheral neuropathy

Investigations

• Full blood count (FBC) which shows eosinophilia, pANCA, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR)
• Imaging such as chest X-ray which shows eosinophilic lung deposits

Treatment

Medical treatment typically includes the use of corticosteroids and immunosuppression.

Complications

Complications include glomerulonephritis and vasculitis.

Prognosis

The prognosis of this condition is typically poor and can result in end organ damage.