A 28-year-old woman developed spontaneously a right- sided pneumothora
x, the leading clinical symptom of an as yet undiagnosed systemic scle
rosis. The diagnosis was confirmed by Raynaud's phenomenon, microstomi
a, arthralgia, distal oesophageal dysfunction and antinuclear antibodi
es. Initial treatment with pleural suction was followed by thoracoscop
y and segmental pulmonary resection, Spontaneous pneumothorax is a rar
e complication in patients with systemic sclerosis, most likely caused
by the rupture of subpleural cysts.