WEGENER'S GRANULOMATOSIS

WEGENER'S GRANULOMATOSIS:

Definition: 

• Wegener's granulomatosis is a rare idiopathic, multisystem disease characterized by generalized small vessel vasculitis affecting respiratory tract and kidneys
• The disease affects males more commonly than females.

Clinical features:

It commonly presents during the fourth and fifth decades.
Initially presents with mild upper respiratory tract symptoms accompanied by night sweats or arthralgia.

Upper respiratory tract: Necrotizing vasculitis leads to

• Perforation of the nasal septum.
• Destruction of the bones of the paranasal sinuses.
• Collapse of the nasal arch resulting in a 'saddle nose' deformity.
• Nasal-paranasal sinus fistulae. 

Lower respiratory tract: Blateral pulmonary granuloma causing productive cough and haemoptysis.

Miscellaneous: 

• Glomerulonephritis: may lead to renal failure.
• Arthralgia 
• Haemorrhagic dermatitis 
• Polyneuritis.

Ocular manifestations:

Anterior segment:

• Peripheral ulcerative keratitis 
• Necrotizing scleritis 
• Conjunctivitis and episcleritis 
• Acute anterior uveitis.
• Nasolacrimal obstruction, dacryocystitis and rarely tarsal-conjunctival disease.

Orbit:

• Orbital involvement is usually due to direct spread from diseased paranasal sinuses. 
• Presents as bilateral 'pseudotumour'  with pain, proptosis,  chemosis and  ophthalmoplegia, 

Posterior segment:

• Occlusive  retinal vasculitis leading to either focal retinal infarcts or major vascular occlusions 
• Optic neuropathy .

Investigations:

• Detail clinical history and complete ophthalmoscopic examination
• The possibility of Wegener granulomatosis should be consider  when bilateral orbital inflammation associated with paranasal sinus pathology.
• Anti-neutrophil cytoplasic antibodies (c-ANCA) are found in over 90% of patients with active disease.

Treatment:

• Treatment is with systemic steroids and cyclophosphamide. 
• In resistant cases ciclosporin, azathioprine, antithymocyte globulin or plasmapheresis may be useful.
• Surgical orbital decompression may be required for severe orbital involvement.
• The prognosis for life is variable.