Wegener's Granulomatosis diagnosis and treatment

• The peak incidence occurs in 30s and 40s.
• This form of systemic vasculitis presents with upper and lower respiratory tract lesions in association with a focal G.N.
• It may be preceded by months or years with recurrent rhinitis, epistaxis (nasal crusting), sinusitis, otitis media (serous) or history of pulmonary
symptoms such as cough, haemoptysis, chest pain or dyspnea.
The most common ocular abnormality is proptosis due to inflammation of the retro-orbital tissue, diplopia may occur due to entrapment of the extraocular muscles.
• Limited wegener's granulomatosis, there is minimal systemic necrotising vasculitis and local granuloma predominates with chronic sinusitis, nasal and orbital destruction with or cavitating lung lesions .
Wegener's Granulomatosis

Investigations

1- ESR High ,,,  normochromic normocytic anaemia of chronic disease + high PNL  + thrombocytosis.
2- Eosinophilia is a characteristic in patients with churg - strauss vasculitis and Wegner's granulomatosis with pulmonary lesions.
3- Anti-neutophil cytoplasmic antibodies (ANCA), two patterns of immunofluorescence are distinguished :

a- Cytoplasmic granular staining of neutophil (C-ANCA) -ve in 80 % of Wegner's granuloma.
b- Perinuclear staining (P-ANCA) in microscopic polyangiitis.

PositiveANCA occur in many other diseases ..including malignancy, infection, inflarrmatory bowel disease, rheumatoid arthritis, systemic .Iupus and pulmonary fibrosis so, the diagnosis of these conditions cannot be made on the ANCA test alone .

How to manage Wegener's Granulomatosis ?

Treatment 
• Steroid and cyclophosphamide
• Plasmapharesis
• Mycophenolate recently can be used.

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