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Wegener’s granulomatosis (WG): Treatment, symptoms, advice and help

About wegener’s granulomatosis

Wegener granulomatosis (WG) is a multisystem autoimmune disease in which inflammation and in small and medium-sized blood vessels causes tissue damage.

Wegener’s granulomatosis: Incidence, age and sex

The annual incidence of WG is 5-10 per million. Men are more likely to be affected with a male-to-female ratio of 1.5:1. Women are more likely to have limited disease. Onset of WG may occur at any age; patients typically present at age 35-55 years. WG is rare in childhood.

Signs and symptoms of wegener’s granulomatosis: Diagnosis

Nonspecific constitutional complaints: like fevers, night sweats ,fatigue, lethargy, loss of appetite & weight loss may occur. The other common symptoms are epistaxis, nasal crusting ,sinusitis, haemoptysis, musosal ulceration and deafness due to serous otitis media.

Wegener’s granulomatosis: Treatment

The diagnosis is usually confirmed by certain blood tests like serological assay for c-ANCA. Treatment should be started early, either with oral high-dose prednisole (1mg/kg daily) and continuous oral cyclophosphamide (2mg/kg daily) or with bolus i.v. methylprednisolone (10mg/kg) and cyclophosphamide (15mg/kg), initially fortnightly and subsequently monthly. Once remission has been induced (3-6 months) the dose or oral prednisolone is rapidly reduced and cyclophosphamide is usually given at a prophylactic dose (960mg thrice weekly). Mesna is used with bolus cyclophosphamide to reduce the risks of haemorrhagic cystitis. Occasionally, cyclophosphamide fails to induce a remission, in which case the diagnosis should be reconsidered.

Wegener’s granulomatosis: Complications

The most common ocular abnormality is proptosis which may cause diplopia loss of vision due to optic nerve compression .They may also develop lung disease,skin ulcers,renal disease & neurological involvement.Untreated nasal disease ultimately leads to destruction of bone and cartilage. Migratory pulmonary infiltrates and nodules occur in 50% of patients. A minority of patients present with glomerulonephritis.