Wegener's granulomatosis, chest

(Friedrich Wegener, born 1907, German pathologist), a disease of unknown cause. It is generally considered a hypersensitivity disorder with granulomatous inflammation and necrotizing small vessel vasculitis. It typically involves the upper and lower respiratory tracts, paranasal sinuses and kidneys, resulting in glomerulonephritis. A limited form of the disease, without renal involvement, also exists.

A high percentage of patients have antineutrophil cytoplasmic antibodies (ANCA), described as cytoplasmic or c-ANCA on immunofluorescence; the presence of c-ANCA has a sensitivity of 88% and a specificity of 95% for diagnosing Wegener's granulomatosis.

Pulmonary involvement is present in 95% of cases, with cough, haemoptysis and dyspnoea being most common. However, pulmonary involvement may be asymptomatic. The most common pulmonary abnormality consists of small or large cavitary nodules that may be transient and recurrent (Fig.1). Pulmonary vasculitis with haemorrhage can also be seen. Involvement of the trachea can result in airway narrowing and obstruction in as many as 15% of patients; this is associated with thickening and irregularity of the airway wall as shown on CT. Clinically, morbidity and mortality is more often related to glomerulonephritis and renal insufficiency than to bronchopulmonary disease. For a general description of the disease, see Wegeners granulomatosis.

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