Granulomatosis with Polyangiitis (GPA)
Formerly known as 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

Wegener's Granulomatosis. Four images span approximately two years and show typical waxing and waning of pulmonary masses (white and green arrows), some of which cavitate (blue arrow), some of which disappear over the course of time (yellow circle).
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Ravenel, JG, MD; Irshad, A, MD.  Wegener Granulomatosis, Thoracic. eMedicine
King, TE, MD.  Respiratory tract involvement in Wegener's granulomatosis.  UpToDate