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Wegener Granulomatosis

Huzaefah Syed, MPH, MD, Christopher M. Wise, MD and Uyen Nguyen, MD Reviewed 04/2024
 


BASICS

DESCRIPTION

  • A disease characterized by granulomatous vasculitis affecting small arteries with multiple organs' involvement.

  • The characteristic triad of involvement includes the upper airway (e.g....

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

  • BP, glucose, and potassium for steroid effects

  • CBC with differential every 1 to 2 weeks to monitor for bone marrow toxicity from cyclophosphamide...

REFERENCES

1
Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382(7):622–631.
2
Terrier B, Charles P, Aumaitre O, et al. ANC...

SEE ALSO

Polyarteritis Nodosa; Vasculitis 

CODES

ICD10

  • M31.30 Wegener's granulomatosis without renal involvement

  • M31.31 Wegener's granulomatosis with renal involvement

SNOMED

  • 195353004 Wegener's granulomatosis (disorder)

  • 232460001 Wegener's granulo...

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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