Approach to the Diagnosis and Management of Diffuse Cystic Lung Diseases

Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of diseases that need a detailed clinical, radiological, and sometimes pathological evaluation to reach at the underlying diagnosis. Disease-modifying therapies are available for a few diseases such as lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH), and should be considered in patients with progressive or advanced disease. In addition, the management of patients with DCLDs includes prevention and treatment of complications such as spontaneous pneumothorax. This chapter reviews the key aspects of clinical, radiological, and other diagnostic modalities as well as the currently available treatment options that can help physicians in managing DCLD patients.