Wegener's granulomatosus mainly involves small vessels with
early, major disease of respiratory tract excluding asthma. Granulomata
are characteristic but not essential feature for diagnosis of W.G.Clinical Features:
1- Renal manifestations are variable from asymptomatic urinary findings
to RPGN.
2- Constitutional symptoms as fever, weight loss, fatigue.
3- Extrarenal manifestations including mononeuritis, myositis, arthritis,
cutaneous vasculitis, angina and in classic PAN mesentric ischaemia
or cerebral strokes.
4- Upper respiratory symptoms including sinusitis, epistaxis, pharyngel
lesions and otitis media or externa. These are more common in W.G.
than PAN.Treatment:
Patients with active urine sediment (proteinuria, haematuria, casts),
renal impairment and documented lesions in renal biopsy should be treated
by immunosuppressive drugs to achieve remission. The standard treatment
is prednisolone and cyclophosphamide. The dose and whether
prednisolone alone or combined drug regimen, depend on disease activity
and initial reponse to treatment. Cyclosporin A 5mg/kg/d can be used
when these drugs are toxic or have no satisfactory response.Plasma exchange has been reported for treatment of severe cases. Use of
azathioprine alone has been disappointing.
Henoch-Schönlein Purpura (HSP)