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.
History: Described by Wegner in 1939.
Aetiology: Unknown. Clinical features: (1) Blood stained nasal secretions.
(2) Systemic focal vasculitis.
Signs:
Anterior Rhinoscopy.
- Nasal mucosa thickened.
- 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.
(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
Systemic:
Spread: To eyes, kidneys, joints, heart and CNS.
Complications:
(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.
Systemic:
- Steroids: 40-60mg/kg/day.
- Cytotoxic drugs:(a) Azathioprine 3mg/kg/day. (b) Cyclophosphamide 2mg/kg/day.
Spread: To eyes, kidneys, joints, heart and CNS.
Complications:
- Opportunistic fungal infections.
- Cytotoxic drug.
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