-> Specially effect Respiratory system, Vascular system, Renal System.
-> Necrotizing granulomas every where, typically involved renal tubules of respiratory system.
Wegener’s granulomatosis is another form of necrotising vasculitis characterised by a clinicopathologic triad consisting of the following:
i) Acute necrotising granulomas of the upper and lower respiratory tracts involving nose, sinuses and lungs.
ii) focal necrotising vasculitis, particularly of the lungs and upper airways.
iii) focal or diffuse necrotising glomerulonephritis.
A limited form of Wegener’s granulomatosis is the same condition without renal involvement. As with PAN, the condition is more common in adult males and involves multiple organs and tissues. Most commonly involved organs are the lungs, paranasal sinuses, nasopharynx and
kidneys. Other involved organs are joints, skin, eyes, ears, heart and nervous system. Accordingly, clinical features are variable. Typical features include pneumonitis with bilateral infiltrates in the lungs, chronic sinusitis, nasopharyngeal ulcerations and renal disease.The etiology is not known but possibly the lesions occur due to the presence of circulating immune complexes. This is supported by the observation of subepithelial immunoglobulin deposits on the glomerular basement membrane and induction of remission by immunosuppressive therapy. The serum of these patients shows c-ANCA positivity. Disseminated form of Wegener’s
granulomatosis differs from a related entity, idiopathic lethal midline granuloma, in the sense that the latter condition is highly destructive and progressively necrotic disease of the upper airways.
(* Goodposture syndrome- it is also result due to self antibody attack our own respiratory & renal system).
Clinical features -::
•) Congestion of nasal mucosa.
•) Gesting of nasal mucosa.
•) Ulceration of nasal mucosa.
•) Perforation of nasal mucosa.
•) Saddle nose deformity.
•) Paranasal sinitis - paranasal sinus become inflammed.
-> Pharyngitis occur.
-> Ostitis media, mastitis.
-> Laryngitis.
-> Epiglottis.
Due to that - Respiratory tract become narrowed , patients have Stridor( obstruct in upper air way).
•) Nodules are seen in lungs chest X-ray.
•) Cavity formation in lungs.
•) CNCA or Proteinase 3 ANCA positive (90-95%).
Clinically -:Cough, Dsypnea, Hemoptysis.
•) Glomerular capillaries inflammed - focal segmented Necrotic Granulonephritis.
•) In severe Cresentic Granulonephritis.
Clinically - proteinuria, hematouria
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