Wegener's

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Wegener's

  1. 1. Wegener’s DEAPCIT
  2. 2. Definition • WG is an necrotising granulomatous diseases leading to – inflammation of the upper and lower respiratory tracts – Systemic vasculitis of small arteries and veins – Focal glomerulonephritis
  3. 3. Epidemiology • prevalence of 5 per 100,000 • males > females (except in laryngeal manifestations) • wide age range but increased in 4-5th decade • all racial groups affected but predominantly whites
  4. 4. Aetiology • cause of WG is unknown• ? viral related activation of ANCA • Increasing circumstantial evidence supports the concept that WG is an autoimmune
  5. 5. Pathogenesis • (ANCA) no direct evidence • Anti Neutrophil surface antibody → degranulation of the leukocytes and the release of toxic oxygen radicals and lysosomal enzymes • neutrophils activated by ANCA → directly damage endothelial cells → vasculitis • Titres for ANCA correlate with disease activity and predict relapses • granular cytoplasmic staining pattern ANCA (c-ANCA) has been strongly associated with WG • a smaller number of patients with WG have a perinuclear staining pattern ANCA (p-ANCA)
  6. 6. pathology • necrotising granulomatous vasculitis • vasculitis of small to medium-sized vessels and polymorphous inflammatory infiltrate composed of lymphocytes, histiocytes and less frequently eosinophils and neutrophils • “ischaemic” or “geographic” type necrosis with basophilic smudgy appearance • multinucleated giant cells or non-necrotising granulomas
  7. 7. Clinical • May be localised or systemic • H+N manifestations = initial symptoms in 75% • laryngx: – Upper airway findings include oedema, ulceration of larynx (25%) and significant subglottic stenosis (8.5%) – Hoarseness, cough, haemoptysis, dyspnoea, stridor and wheeze – Flattening of both insp and exp phase in flow-volume loop
  8. 8. Clinical (ear) • • External – Rarely involves external ear – OE 2ry to OM – Auricular chondritis similar to RP Middle – Serous otitis media – most common otologic manifestation • 2ry to ETD – CHL – Suppurative otitis media possibly with granulation tissue – CNVII palsy 2ry to otomastoiditis – TM perforation? • Inner – SNHL = 2nd most common L-T morbitidy (renal = 1st) • 35% affected • May develop rapidly • ? 2ry to vasculitis of cochlear vasculature • ? Immune complex deposition within cochlea – NB vertigo = rare – ? Tinnitus
  9. 9. Clinical (nose) • Nose: – Non-specific • Nasal congestion, rhinorrhoea, anosmia, epistaxis – Cobblestone mucosa – Crusting, septal perforation, saddle nose deformity, dorsal nasal pain (suggesting chondritis) and recurrent sinusitis – Anterior nasal septum in Kiesselbach’s plexus = commonly involved – Nasal airway cicatricial stenosis – Nasal cavity > maxillary > ethmoid > frontal > sphenoid – NB often mistaken for NK cell lymphoma
  10. 10. Clinical • Throat: – Oral cavity manifestations (rare) – hyperplasia of gingiva and gingivitis – Strawberry gingival hyperplasia – Upper airway findings include oedema, ulceration of larynx (25%) and significant subglottic stenosis (8.5%) • H+N: – Salivary involvement Sjögren syndrome – Can have massive enlargement of the SMG or parotid glands
  11. 11. Clinical • Pulmonary: – Classic chest radiographic findings include bilateral multiple parenchymal nodes (+/cavitation), or airway disease that simulates pneumonia • Renal: – Usual cause of death in WG and the most important prognostic feature
  12. 12. Investigation • Haematological – Elevated ESR – ANCA (C-ANCA & p-ANCA) – WG → c-ANCA is +ve in 70% and p-ANCA in 25% – Patients with polyarteritis nodosa and Kawasaki disease may also test positive • Radiological – CXR: for pulmonary infiltrates • Pathological – Biopsy vasculitis of small vessels, granulomatous changes, and focal necrosis • Urinalysis – Performed to evaluate urinary sediment (eg, RBC casts, haematuria, proteinuria)
  13. 13. Diagnosis • Criteria – Nasal or oral inflammation – Chest x-ray showing nodules, infiltrates (fixed), or cavities – Microscopic haematuria or red cell casts in urine – Granulomatous inflammation on biopsy (within vessel wall or perivascular) • New Criteria – all of: – Lack of eosinophilia – Biopsy verified necrotising vasculitis – Glomerulonephritis – Granulomatous inflammation – surrogate – PR3 c-ANCA +ve
  14. 14. Treatment • untreated mortality rate of 90% at two years • consists of meticulous dental and nasal care, removal of crusts from the nose and ET orifices and ME drainage • limited disease → prednisolone and methotrexate or prednisolone and cyclophosphamide for 3 months → methotrexate and azathioprine • systemic disease → prednisolone and cyclophosphamide for 3-12 months → methotrexate and azathioprine • other options include mycophenolate, anti-TNF and IV Ig • for isolated sinus disease, treatment includes low dose steroids, topical steroids, saline irrigations and antibiotics as needed • airway compromise is alleviated with systemic steroids and subglottic stenosis may warrant tracheotomy
  15. 15. Treatment • AIRWAY – Dilation and intralesional/inhaled steroid or mitomycin C – CO2 laser treatment of stenosis – Silicone stenting of stenosis – laryngotracheal reconstruction – endoscopic longitudinal incision of stenosis – Tracheostomy / Tracheal resection

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