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Granulomatosis with Polyangiitis
Aka: Granulomatosis with Polyangiitis, Wegener's Granulomatosis
- See Also
- Interstitial Lung Disease
- Small Vessel Vasculitis
- Glomerulonephritis
- Epidemiology
- Rare Incidence
- Peak Incidence during fourth decade of life
- Pathophysiology
- Renamed in 2012 as Granulomatosis with Polyangiitis
- Previously known as Wegener's Granulomatosis
- Classic Triad of Involvement
- Interstitial Lung Disease
- Glomerulonephritis
- Upper respiratory tract (Sinus and nasal disease)
- Granulomatous ANCA-Associated Small Vessel Vasculitis
- Necrotizing Granulomas
- Symptoms
- Paranasal Sinus congestion
- Sinus pain
- Rhinorrhea
- Purulent Nasal Discharge
- Epistaxis
- Respiratory
- Cough
- Hemoptysis
- Dyspnea
- Signs
- Head and neck changes
- Nasal mucosa ulceration
- Septal perforation
- Cartilaginous destruction (Saddle nose deformity)
- Gingival ulceration
- Recurrent Sinusitis
- Otitis Media
- Hearing Loss
- Lung Changes
- See Interstitial Lung Disease
- Pneumonia
- Renal
- See Glomerulonephritis
- Eye Involvement may also occur
- Conjunctivitis
- Uveitis
- Retinitis
- Chemosis
- Exophthalmos
- Rheumatologic
- Polyarthritis
- Neurologic
- Neuropathy
- Differential Diagnosis
- See Interstitial Lung Disease
- See ANCA-Associated Small Vessel Vasculitis
- Polyarteritis Nodosa
- Labs
- Antineutrophil Cytoplasmic Antibodies (ANCA)
- cANCA positive in 75-90% of patients
- pANCA positive in 20% of cases
- ANCA Test Specificity: 98%
- Despite Specificity, high False Positive Rate due to rare Incidence of condition
- Indications for ANCA testing (do not obtain solely due to recurrent Sinusitis)
- Pulmonary-Renal Syndrome
- Rapidly progressive Renal Failure
- Mononeuritis multiplex
- Pulmonary Hemorrhage
- Complete Blood Count
- Anemia
- Leukocytosis
- Eosinophilia
- Urinalysis
- Consistent with Glomerulonephritis
- Hyperglobulinemia
- Imaging: Chest XRay
- See Interstitial Lung Disease
- Bronchopneumonic patches
- Multiple nodular densities (may cavitate)
- Diagnostics
- Open lung biopsy (most definitive)
- Renal and sinus biopsy are often non-diagnostic
- Management
- Induction Therapy
- Cyclophosphamide (Cytoxan)
- Corticosteroids
- Consider high-dose IV Methylprednisolone for 3 days
- Maintenance Therapy
- Taper Prednisone
- Maintain cyclophosphamide for 12 to 18 months
- Coarse
- Mortality often associated with Renal Failure
- References
- Allen in Goldman (2000) Cecil Medicine, p. 1529-32
- Calabrese in Ruddy (2001) Kelley's Rheum, p. 1167-76
- Ali (2018) Am Fam Physician 98(3): 164-70 [PubMed]