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Wegener’s Granulomatosis
 

 General

  • Male:female ratio of 2:1

  • Peak in 40s

  • Autoimmune disease characterized by necrotizing granulomas and angiitis

  • Diagnosis is made by lung or kidney biopsy

  • Death comes from renal failure or respiratory failure

  • Treated with steroids and cytotoxic drugs

Respiratory Tract (100% involved)

Upper respiratory tract

  • Mucosal thickening in paranasal sinuses

  • Bone and cartilage destruction

Lungs

  • Multiple nodules of varying sizes, especially at bases

  • Cavitate frequently (50%)

  • Masses wax and wane

  • Pleural effusion (25%)

  • Alveolar infiltrate occasionally

Other Organs

  • Urinary tract:focal glomerulonephritis (50%)

  • Joints:migratory polyarthropathy (56%)

  • Skin:inflammatory skin lesions (44%)

  • Eyes and ears:proptosis and otitis media (29%)

  • Heart and pericardium: myocardial infarction (28%)

  • CNS: neuritis (22%)

Symptoms

  • Rhinorrhea

  • Sinusitis

  • Epistaxis

  • Cough with hemoptysis

Midline Lethal Granuloma

  • Variant of Wegener’s consisting of mutilating granulomatosis and neoplastic lesions limited to nose and paranasal sinuses

 

WH/ ‘93

 

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