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Key Features

Essentials of Diagnosis

  • Triad of

    • Upper respiratory tract disease

    • Lower respiratory disease

    • Glomerulonephritis

  • Suspect if upper respiratory tract symptoms (eg, nasal congestion, sinusitis) are refractory to usual treatment

  • Tissue biopsy usually necessary for diagnosis

  • ANCAs, usually directed against proteinase-3 (less commonly against myeloperoxidase present in severe, active disease) (90% of patients)

  • Kidney disease often rapidly progressive

General Considerations

  • The disease presents as vasculitis of small arteries, arterioles, and capillaries, necrotizing granulomatous lesions of both upper and lower respiratory tract, and glomerulonephritis

Demographics

  • Annual incidence of 12 per million

  • Occurs most commonly in the fourth and fifth decades of life

  • Affects men and women with equal frequency

Clinical Findings

Symptoms and Signs

  • Usually develops over 4–12 months

  • Fever, malaise, and weight loss

  • Upper respiratory tract symptoms develop in 90% of patients

  • Lower respiratory tract symptoms develop in 60% of patients

  • Some patients develop both upper and lower respiratory tract symptoms

  • Upper respiratory tract symptoms can include

    • Nasal congestion

    • Sinusitis

    • Otitis media, mastoiditis

    • Inflammation of the gums

    • Stridor due to subglottic stenosis

  • Physical examination can be remarkable for congestion, crusting, ulceration, bleeding, and even perforation of the nasal septum

  • The lung is affected initially in 40% and eventually in 80%, with symptoms including cough, dyspnea, and hemoptysis

  • Renal involvement (75%) may be subclinical until renal insufficiency is advanced

  • Other early symptoms can include

    • Unilateral proptosis (from orbital pseudotumor)

    • Red eye from scleritis

    • Arthritis

    • Purpura

    • Dysesthesia due to neuropathy

  • Destruction of the nasal cartilage with "saddle nose" deformity occurs late

  • Patients are at high risk for venous thrombotic events (deep venous thrombosis, pulmonary embolism)

Differential Diagnosis

  • Polyarteritis nodosa

  • Microscopic polyangiitis

  • Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome)

  • Chronic sinusitis

  • Goodpasture disease

  • Systemic lupus erythematosus

  • Sarcoidosis

  • Cocaine use

Diagnosis

Laboratory Tests

  • c-ANCA

    • Caused by antibodies to proteinase-3, a constituent of neutrophil granules

    • Has a high specificity (> 90%)

    • In the setting of active disease, the sensitivity of c-ANCA is also reasonably high (= 70%)

  • p-ANCA

    • Caused by antibodies to myeloperoxidase and is much less specific than c-ANCA

    • Approximately 10–25% of patients with classic granulomatosis with polyangiitis have p-ANCA

  • If kidney involvement is present

    • There is proteinuria

    • The urinary sediment contains red cells (with or without white cells) and red cell casts

Imaging Studies

  • Chest CT is more sensitive than chest radiograph for infiltrates, nodules, masses, and cavities

  • Pleural effusions are uncommon

Procedures

  • ANCA testing does not eliminate the need in most cases for confirmation of the diagnosis by tissue biopsy

  • The full range of pathologic changes is usually evident only on thoracoscopic lung biopsy

  • Histologic features include

    • Vasculitis

    • Granulomatous inflammation

    • Geographic necrosis

    • Acute and chronic ...

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