Granulomatosis with Polyangiitis (formerly known as Wegener’s Granulomatosis)

9 Oct

Name:

-No longer referred to as Wegener’s Granulomatosis- Wegener was found to have been a Nazi who performed medical experiments on prisoners

 

Symptoms at presentation:

-Upper respiratory:  recurrent epistaxis, mucosal ulcerations, nasal septal deformities

-Other ENT symptoms: sinusitis, otitis media, hearing loss, sublgottic stenosis, oral lesions.

-Pulmonary presentations:  Infiltrates, nodules, hemoptysis

-Eye presentations: conjunctivitis, dacrocystitis, scleritis, proptosis

Most patients present with nasal/sinus/pulmonary presentations; few present with glomerulonephritis.  However, most develop renal insufficiency later in disease.

 

Diagnosis:

Clinical features of pulmonary-renal vasculitis, biopsy demonstrating glomerulonephritis/vasculitis, and positive cANCA.

-Renal biopsy would show necrotizing glomerulonephritis with crescent formation

-About 80% of patients have no renal involvement at presentation

 

Testing:

cANCA has a specificity of 95% but its level is dependent on the severity of the disease

ESR is a useful test which often can relate to disease activity

Anemia with thrombocytosis is common

 

Management:

-Oral cyclophosphamide with oral prednisolone

 

 

Morbidity and Future Risks

Increased risk of cancer (bladder cancer is a significant risk for patients on cyclophosphamide)

PCP pneumonia is actually a frequent complication– currently no prophylaxis is recommended but this could change in the future

-About 42% of these patients will develop chronic renal insufficiency

Hearing loss and subglottic stenosis are also common

 

Submitted by J. Grover.

 

References: Shahrir M, and Said M.  “Upper Respiratory Tract Symptoms, Renal Involvement and Vasculitis:  A Case Report and Review of Wegener Granulomatosis.”  J Clin Med Res 2010; 2 (4): 189-193.;Tintinalli’s Emergency Medicine: A Comprehensive Study Guide. 7th Edition.; picture

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