ENT Specialist WEGNERS GRANULOMATOSIS

WEGNER’S GRANULOMATOSIS

Definition: It is a systemic disease of unknown aetiology, causing necrotizing granuloma in the upper and lower respiratory tract, skin and kidneys.
History: Described by Wegner in 1939.
Aetiology: Unknown. Clinical features: (1) Blood stained nasal secretions.
(2) Systemic focal vasculitis.
ENT Specialist Screenshot 2020 11 28 Ganpati nose
Signs: Anterior Rhinoscopy.
  1. Nasal mucosa thickened.
  2. Crusting may be present.
Clinical and pathological classification 2 types: Type I (1) Moderate nasal obstruction.
(2) Bloody discharge.
(3) Patient is anaemic and weak.
(4) Occasional erosion of vomer.
(5) Raised creatinine levels.
(6) Early renal symptoms.
(7) Large blood-stained crusts from nose.
Type II (1) Mild anaemia.
(2) Raised ESR.
(3) Few systemic features.
(4) Saddling deformity of nose.
(5) Pulmonary involvement common.
(6) Less crusting.
Investigations: (1) Blood investigations for Anaemia and raised ESR.
(2) X-ray: Chest, PNS, CT Scan paranasal sinuses if severe sinusitis.
(3) Renal function tests: Reduced sinuses creatinine clearance.
(4) Nasal Biopsy: It shows
    • a) Necrosis and ulceration of mucosa.
    • b) Epithelioid granuloma.
    c) Necrotizing vasculitis involving small arteries or veins.
Medical treatment:
Systemic: 
  1. Steroids: 40-60mg/kg/day.
  2.  Cytotoxic drugs:(a) Azathioprine 3mg/kg/day. (b) Cyclophosphamide 2mg/kg/day.
Local: Glucose in Glycerin nasal drops. Surgical: Treatment of sinusitis by Functional Endoscopic Sinus Surgery (FESS). If perforation of the nasal septum is there, then surgical repair is recommended by septal perforation repair by nasal flap or septal button. If saddle nose deformity is there, then surgical repair is done by augmentation rhinoplasty.
Spread: To eyes, kidneys, joints, heart and CNS.
Complications:
  1. Opportunistic fungal infections.
  2. Cytotoxic drug.

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