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Paediatrics: Wegener’s granulomatosis

Triad of ANCA positive small vessel vasculitis, respiratory tract granu-lomata, renal disease.

Wegener’s granulomatosis

 

Triad of ANCA positive small vessel vasculitis, respiratory tract granu-lomata, renal disease. Rare. Usually diagnosed in adolescents (male = female). Staphylococcus aureus may have role in pathogenesis since 3 times greater carriage in Wegener’s granulomatosis (WG).

 

Clinical features

 

   Subacute disease can be present for years. Transformation into systemic disease (malaise, fever, weight loss, vasculitis) occurs.

   ENT (90%): nasal crusting, obstruction, and ulceration; serous otitis media; sinusitis. Nasal septum and sinus wall destruction (saddle nose deformity).

   Pulmonary (80%): subglottic stenosis (stridor); haemoptysis (25%); lower bronchial obstruction with atelectasis and pneumonia; pulmonary haemorrhage; asymptomatic nodules.

   Renal (90%): varies from mild asymptomatic (commoner microscopic haematuria; mild renal impairment) to fulminant diffuse necrotizing crescentic glomerulonephritis and renal failure.

   Arthritis (50%): non-erosive polyarthritis; muscle and joint pains common (60%).

   Skin (40%): palpable purpura of leucocytoclastic vasculitis; livido reticularis; pyoderma gangrenosum.

   CNS (30%): mononeuritis multiplex and sensorimotor peripheral neuropathy.

   Eye lesions: episcleritis; uveitis; orbital pseudotumour.

 

Investigations

 

·  Blood:

 

   FBC (normocytic, normochromic anaemia, leucocytosis, thrombocytosis). ESR and CRP raised (differential diagnosis: infection);

   renal screen with BP measurement and urinalysis at each visit. Renal biopsy if active sediment and declining renal function;

   CANCA (proteinase 3) positive in 90% patients with generalized WG. High specificity.

   Lungs:

   CXR, sputum culture, and cytology; CT lungs; bronchoscopy and biopsy if indicated.

   CT sinuses +/– nasendoscopy and biopsy.

   Histology: necrotizing, giant cell, granulomatous, medium vessel vasculitis in respiratory tract.

 

Course and treatment

 

   Systemic disease: treated with pulsed IV cyclophosphamide and steroids to induce remission. Remission maintenance with MTX or AZA. Minimize total steroid load. Only stop after min. 12mths disease-free.

Subacute and limited disease: have variable (milder) course; may respond to MTX alone or with low dose steroids. Long-term co-trimoxazole in remission reduces pulmonary infection and relapse rates. 10yr survival 75%; morbidity considerable.

 

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