Granulomatous Polyangiitis

Exam Cram

• GPA: granulomatous polyangiits is a systemic vasculopathy of small arteries

• Presentation: sinusitis, haemoptysis & haematoproteinuria

• Antibodies: c-ANCA

• Management: prednisolone & cyclophosphamide

Outline

Background 

• Systemic small vessel arteritis

Assessment

History:

• Sinusitis

• Short of breath, haemoptysis

Examination:

• Saddle nose deformity

Investigations

• ANCA: 80% sensitivity

• Anaemia

• Urinanalysis: haemoproteinuria

• CXR- pulmonary haemorrhage

• c-ANCA +ve

• Biopsy: arterial granulomas

Diagnosis

Diagnostic criteria: 1990 American College of Rheumatology- 2 or more criteria: 90% sensitivity & specificity

1. Nasal or oral inflammation- painful or painless ulcers, purulent or bloody nasal discharge

2. CXR- nodules, infiltrates, cavities

3. Urine- non visible haematuria, red cell casts, sediment

4. Biopsy- granulomatous inflammation within artery wall

Differentials: 

• Goodpasture's Disease has normal inflammatory markers

• EGPA (Eosinophilic Granulomatosis with Polyangiitis): PMH of asthma

Management

Acute:

1.  Prednisolone + Cyclophosphamide (sometimes pulsed methylpred)

2.  Rituxumab

Long term prophylaxis:

1. Azathioprine or methotrexate

Med Reg Notes

Plasma exchange not effective

Different names

• P-ANCA: MPO

• C-ANCA: PR3

References

Page written in 2024. 

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