Unusual causes of pneumothorax

Spontaneous pneumothorax may occur in association with various diseases of the lung. Several uncommon pulmonary conditions are frequently associated with pneumothorax, such as lymphangioleiomyomatosis (LAM) and pulmonary Langerhans’ cell histiocytosis (PLCH). On the other hand, there are common systemic diseases that are infrequently or rarely associated with pneumothorax. Examples of the latter group include: anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, systemic lupus erythematosis (SLE), rheumatoid arthritis and related conditions, scleroderma, dermatomyositis, and inflammatory bowel disease. Finally, extremely rare conditions such as Birt-Hogg-Dube syndrome have an association with pneumothorax. This chapter will provide a review of the evaluation and management of patients with these unusual causes of pneumothorax