Eosinophilic Granulomatosis with Polyangiitis (EGPA) – At A Glance

Eosinophilic Granulomatosis with Polyangiitis (EGPA)  was previously called as Churg Strauss Syndrome 

EGPA is a systemic inflammatory disease of small and medium sized vessels.

Commonly affect lungs and skin, but can affect other systems as well.


Causes Of EGPA

  • Cause is unknown
  • Believed to be multifactorial
  • Strong association with Anti Neutophil Cytoplasmic Antibodies (ANAC), which is responsible for autoimmune diseases
  • Associated with severe asthma
  • HLA-DRB4 increases the risk 

Presentation Of EGPA
 
* Major presentation features have been marked in bold
 
Has three phases
  • Prodromal phase
    • Seen in teenagers and early twenties 
    • Allergic rhinitis
    • Asthma
  • Eosinophilic phase
    • Eosinophilic pneumonia
    • Gastroenteris
    • High levels of circulating eosinophils
  • Vasculitic phase
    • Progress to this phase around thirties and early forties 
    • Vasculitis of small and medium sized vessels with granulomatous inflammation
    • Constitutional symptoms
      • Fever
      • Malaise
      • Fatigue
      • Weight loss
      • Athralgia
      • Myalgia


Even though vasculitis of EGPA mainly affects the lungs and skin, it can affect the heart, kidneys, intestine and nerves as well producing various symptoms and signs.

  • Pulmonary
    • Asthma 
    • Cough
    • Haemoptysis
    • Allergic rhinitis
    • Sinusitis
    • Pneumonitis
  • Skin 
    • Leukocytoclastic angiitis
    • Purpura
    • Livedo reticularis
    • Skin necrosis
  • Cardiovascular
    • Myocarditis
    • Pericarditis
    • Constrictive pericarditis
  • Gastrointestinal
    • Abdominal pain 
    • Diarrhoea
    • GI bleeding (Rare)
  • Nephrological
    • Renal failure – HTN
  • Neurological
    • Mononeuritis multiplex 
    • Peripheral neuropathy 



Diagnostic Criteria Of EGPA By ERS

  • Asthma 
  • High levels of eosinophils in the blood (More than 10% of all WBC) 
  • Damage from vasculitis to the skin, nerves, kidney, lung, heart
    or
    Blood tests suggesting vasculitis


Investigations Of EGPA
  • Haematological tests
    • FBC
      • More than 10% eosinophils of all WBC
      • ESR, CRP elevated
    • Renal profile – BUN, S. Cr, SE
    • Urine analysis
    • p-ANCA 
      • Positive in 40% of patients
    • Serum IgE 
      • Elevated
  • Chest X-Ray
    • Pulmonary opacities
    • Pulmonary infiltrates 
  • Lung function tests
    • Spirometry
  • Biopsy of skin, lungs, nerves, kidneys, muscle
    • Elevated eosinophils
    • Granulomatous inflammation
  • Bronchscopic lavage
    • Washout has high eosinophilic count
  • ECG, Echo


Treatment Of EGPA
  • Steroid is the mainstay of treatment 
  • Should be given for long durations
  • Control of asthma 
    • Inhaled corticosteroids
  • For severe disease, not responding for steroids
    • Cyclophosphamide 
  • Add on therapy
    • Given along with steroids
    • Azathioprine
    • Methotrexate
  • Newer drugs
    • Rituximab 
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