@entity1222 ( @entity1222 ) is a relatively uncommon @entity358 that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of @entity1222 is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of @entity1 are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. @entity32 may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and @entity6980 . Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and @entity66 . Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with @entity1222 . All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most @entity1 is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and @entity120 may shorten life expectancy. @entity1 with @entity74 may require lung transplantation.

XXXX cell histiocytosis.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity32 :: ('MESH:D007249', 'Disease') :: ['Bronchiolar inflammation'] @entity66 :: ('MESH:D004194', 'Disease') :: ['cystic abnormalities'] @entity1222 :: ('MESH:D006646', 'Disease') :: ['Pulmonary Langerhans Cell Histiocytosis', 'PLCH'] @entity120 :: ('MESH:D006976', 'Disease') :: ['secondary pulmonary hypertension'] @entity6980 :: ('MESH:D066253', 'Disease') :: ['pulmonary vascular remodeling'] @entity358 :: ('MESH:D008171', 'Disease') :: ['lung disease'] @entity74 :: ('MESH:D030342', 'Disease') :: ['progressive disease']
@entity358 :: (MESH:D008171,Disease) :: ['Pulmonary langerhans']
