NOSE - Wegner’s granulomatosis

What is Wegner's Granulomatosis?
 It is a systemic disease of unknown Aetiology, causing necrotizing granulomata in the upper and lower respiratory tract and kidneys.
History: Described by Wegner in 1939.
What is the Aetiology of Wegner's granulomatosis?
 Unknown

What are the Clinical features of Wegner's granulomatosis?

(1) Blood stained nasal secretions
(2) Systemic focal vasculitis
SIGNS: Anterior Rhinoscopy

  1. Nasal mucosa thickened
  2. Crusting may be present

Clinical and pathological classification 2 types :

Type I                                                     Type II
(1) Moderate nasal obstruction               (1) Mild anaemia
(2) Bloody discharge                                  (2) Raised ESR
(3) Patient is anaemic and weak             (3) Few systemic features
(4) Occasional erosion of vomer           (4) Saddling deformity of nose
(5) Raised creatinine levels                      (5) Pulmonary involvement common
(6) Early renal symptoms                   (6) Less crusting
(7) Large blood stained crusts From nose

 

What Investigations are required for the diagnosis of wegner's granulomatosis?
(1) Blood investigations for (a) Anaemia (b) 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:
Reveals:
a) Epithelioid granulomata
b) Fibrinoid necrosis

What is the treatment of Wegner's granulomatosis?

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)
Spread : To eyes, kidneys, joints, heart and CNS
What is the Complications of Wegner's granulomatosis?

1. Opportunistic fungal infections
2. Toxic effects of Cytotoxic drugs and steroids



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