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).
•
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.
· 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.
•
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.
Related Topics
Privacy Policy, Terms and Conditions, DMCA Policy and Compliant
Copyright © 2018-2023 BrainKart.com; All Rights Reserved. Developed by Therithal info, Chennai.