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Wegener’s Granulomatosis

Wegener’s Granulomatosis. Kristine Scruggs AM Report 14 September 2009. Presentation. Upper Airway (95%) Persistent rhinorhea Purulent/bloody nasal discharge Oral and/or nasal ulcers Sinus pain Other: Hoarseness, stridor, earache, conductive and/or sensorineural hearing loss, otorrhea

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Wegener’s Granulomatosis

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  1. Wegener’s Granulomatosis Kristine Scruggs AM Report 14 September 2009

  2. Presentation • Upper Airway (95%) • Persistent rhinorhea • Purulent/bloody nasal discharge • Oral and/or nasal ulcers • Sinus pain • Other: Hoarseness, stridor, earache, conductive and/or sensorineural hearing loss, otorrhea • Lower Airway (85-90%) • Cough • Dyspnea • Hemoptysis • Pleuritic pain • Pulmonary consolidation and/or Pleural effusion • Renal (77%) • Glomerulonephritis • Proteinuria • Hematuria, RBC casts • Eyes (52%) • Conjunctivitis • Dacryocystitis • Episcleritis/scleritis • Granulomatous sclerouveitis • Ciliary vessel vasculitis • Retroorbital mass lesions -> Proptosis • Skin (46%) • Papules, vesicles, palpable purpura, ulcers, or subcutaneous nodules • CNS (23%) • Cranial neuritis, mononeuritis multiplex, cerebral vasculitis and/or granuloma • Cardiac (8%) • Pericarditis, coronary vasculitis, cardiomyopathy • Other (common) • Malaise, weakness, arthralgias, anorexia, weight loss, fever.

  3. Epidemiology • 3/100,000 patients • Much more common in Caucasians • M:F = 1:1 • Mean age of onset ~40 yrs • Occurs at any age • 15% at <19 yrs, but rare before adolescence

  4. Diagnosis • Clinical Criteria: • Nasal or oral inflammation • Abnormal CXR showing nodules, fixed infiltrates, or cavities • Abnormal urinary sediment (microscopic hematuria +/- RBC casts) • Granulomatous inflammation on biopsy of an artery or perivascular area • 2+ yields sens. of 88%, spec. of 92% • Laboratory Evaluation: • Leukocytosis • Thrombocytosis (>400,000/mm3) • Elevated ESR, CRP • Normochromic, normocytic anemia

  5. Diagnosis, cont. • Biopsy • Taken from site of active disease • Leukocytoclastic, necrotizing vasculitis with little or no complement and IgG on immunoflorescence • Granulomatous inflammation differentiates from MPA • Kidney: segmental necrotizing GN, pauci-immune on immunofluorescence or EM • ANCA • Indirect immunofluorescence assay: • c-ANCA or p-ANCA • 90-95% sensitive • ELISA: • PR3 or MPO

  6. Medium Vessel Polyarteritis nodosa Renal infarcts, renal vasculitis, visceral microaneurysms ANCA - Kawasaki’s disease Coronary artery involvement Mucocutaneous lesions, LAD Large Vessel Giant cell (temporal) arteritis Takayasu’s arteritis Small Vessel Churg-Strauss syndrome Asthma, eosinophilia ANCA + Microscopic polyangiitis ANCA + Pulm-Renal syndrome No granulomatous disease Henoch-Schonlein purpura Dermal-Renal syndrome Cryoglobulinemic vasculitis Cutaneous leukocytoclastic angiitis Behcet’s disease Goodpasture’s syndrome Pulm-Renal syndrome Anti-GBM Differential Diagnosis

  7. Jennette, et al. NEJM. November 1997.

  8. + + + Jennette, et al. NEJM. November 1997.

  9. 80 90 90 Jennette, et al. NEJM. November 1997.

  10. Pulmonary Hemorrhagic Alveolar Capillaritis Neutrophils in the alveolar septa and hemorrhage into the alveolar spaces Jennette, et al. NEJM. November 1997. www.merck.com

  11. Treatment Recommendations • Induction of Remission • Daily oral cyclophosphamide + glucocorticoids (most aggressive Rx) • Monthly cyclophosphamide + glucocorticoids • Low-dose weekly oral MTX + glucocorticoids (mild disease) • No role for PLEX unless DAH present • Maintenance of Remission (12-18 mos) • Weekly oral MTX • Daily oral Azathioprine • Cyclophosphamide not advised 2/2 toxicity • Glucocorticoids should be quickly tapered off • Treatment of Relapse - Reinduction

  12. Morbidities of disease: Renal insufficiency Hearing loss Tracheal stenosis Saddle nose deformity Chronic sinus dysfunction Morbidities of treatment: Glucocorticoids – DM, infection, cataracts, osteoporosis, cushingoid features Cyclophosphamide – Cystitis (30%), bladder cancer (6%), myelodysplasia (2%), permanent infertility Disease was universally fatal prior to established treatment regimens With above therapies, improvement seen in >90%, CR in 75% Relapse seen in 50% of CR Reinduction almost always successful Prognosis

  13. References • Jennette JC, Falk RJ. Small-Vessel Vasculitis. N Engl J Med. 1997 Nov 20; 337 (21): 1512-23. • Kasper, et al. Harrison’s Principles of Internal Medicine, 16th Ed. • King, et al. Up To Date. Clinical manifestations and diagnosis of Wegener's granulomatosis and microscopic polyangiitis. 2009 Apr 21. • Stone JH, et al. Initial and maintenance therapy of Wegener's granulomatosis and microscopic polyangiitis. Up To Date. 2009 Jun 19.

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