Tension Pneumothorax as Initial Manifestation of Granulomatosis with Polyangiitis (GPA)

Granulomatosis with polyangiitis (Wegener's granulomatosis) is a systemic vasculitis that primarily affects small and medium vessels. Its manifestations are usually confined to the upper airway, lower airway and kidney. It can also affect other organs and systems, although this is unusual. We describe the case of a 67-year-old woman who presented with a tension pneumothorax due to rupture of a pulmonary cavity. This pulmonary cavity proved to be secondary to systemic disease which also caused a tumour in her kidney. Biopsy showed non-necrotizing granulomatosis, and even though antineutrophil cytoplasmic antibodies (ANCA) were negative, the diagnosis of granulomatosis with polyangiitis was made. Granulomatosis with polyangiitis (GPA) can be a challenging diagnosis when the initial manifestation is atypical, so a careful history and physical examination are needed to make the diagnosis.It is not uncommon for patients with multisystemic inflammatory disease to attend several different specialty clinics before the diagnosis is reached.GPA with negative ANCA is rare, and occurs more frequently in non-severe forms of the disease.