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Wegener Granulomatosis

Huzaefah Syed, MD and Christopher M. Wise, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • A disease characterized by granulomatous vasculitis involving multiple organs

  • The characteristic triad of involvement includes the upper airway (e.g., otitis, sinusitis, nasal mucosa)...

DIAGNOSIS

HISTORY

  • Fever: 34%

  • Weight loss: 16%

  • Cough: 34%

  • Arthralgia/arthritis: 44%

  • Epistaxis: 11%

  • Hemoptysis: 18%

  • Rash: 13%

  • Chest pain, anorexia, proptosis, dyspnea, oral ulcers, hearing loss, headache: all ...

TREATMENT

  • Patients with significant renal involvement or serious pulmonary disease require cyclophosphamide or rituximab for optimal initial control in most cases but may be converted to methotrexate,...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Early, careful monitoring of upper airway, pulmonary, and renal manifestations

  • BP, glucose, and potassium for steroid effects

  • CBC with differentia...

REFERENCES

1
Lyons PA, Rayner TF, Trivedi S, et al. Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med.  2012;367(3):214–223. [View Abstract on OvidMedline]
2
Stone JH, Mer...

SEE ALSO

Polyarteritis Nodosa; Vasculitis 

CODES

ICD10

  • M31.30 Wegener's granulomatosis without renal involvement

  • M31.31 Wegener's granulomatosis with renal involvement

ICD9

446.4 Wegener's granulomatosis 

SNOMED

  • 195353004 Wegener's granulomatosis (d...

CLINICAL PEARLS

  • Biopsies of mucosal lesions, skin lesions, lung, or kidney typically are needed to confirm a diagnosis of Wegner granulomatosis, but tissue diagnosis is often difficult unless an open ...

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