The literature data base has been updated with one paper:

Bhardwaj & Bhardwaj comment on a recent case study of a woman with lymphangioleiomyomatosis (LAM) (Singh and Singh, 2013). The authors discuss two other causes of cystic lung disease – pulmonary Langerhans cell histiocytosis (PLCH) and Birt-Hogg-Dubé symdrome (BHD) – as alternative diagnoses. The authors describe the radiological differences between PLCH, LAM and BHD, to aid distinguishing between the three diseases.

PLCH is caused by overproliferation of Langerhans-type histiocytic cells, causing complex shaped cysts to form in the upper lobes of the lung. PLCH is highly associated with smoking, with more than 95% of patients being current or ex-smokers. LAM lung cysts are usually found in the upper lobes of the lung, but are generally simple, round cysts, so can be differentiated from PLCH cysts by their shape. LAM is almost predominantly seen in women of child bearing age, meaning that they can be differentiated from BHD lung cysts by their location in the lungs and epidemiology. BHD lung cysts are predominantly located in the basal areas of the lungs, and are autosomal dominantly inherited, meaning that male or female FLCN mutation carriers of any age can be affected.

This paper is available to download from the BHD Articles Library: Clinical Research.

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