Erdheim-Chester Disease Key Facts Terminology Rare histiocytic infiltrative disorder that most commonly affects bones but may affect other organs, including lung Clinical Issues Bone involvement is most common site 50%…

Langerhans Cell Histiocytosis Key Facts Terminology Proliferation of Langerhans cells that form multiple interstitial, bilateral, peribronchiolar pulmonary nodules Clinical Issues Highest incidence is in cigarette smokers (95% of patients) 18-40…

Sarcoidosis Key Facts Etiology/Pathogenesis Etiology unknown, but evidence suggests it may represent exaggerated cellular immune response to an unidentified antigen Clinical Issues Most commonly affected sites include lungs (95% of…

Blastomycosis Key Facts Terminology Pulmonary infection caused by inhalation of the fungus Blastomyces dermatidis Clinical Issues Geographic distribution in the USA: Mississippi, Missouri, and Ohio River valleys Acute infection is…

Respiratory Bronchiolitis Key Facts Terminology Respiratory bronchiolitis-interstitial lung disease (RB-ILD) Synonym: Smoker’s bronchiolitis Mild form of interstitial lung disease Etiology/Pathogenesis Closely associated with smoking history Occupational exposure to fumes Clinical…

Bronchiolitis Obliterans with Organizing Pneumonia Key Facts Terminology Synonyms: Cryptogenic organizing pneumonia (COP), bronchiolitis obliterans Definition Idiopathic interstitial and airway disease process with features of pneumonia Etiology/Pathogenesis No definitive etiology…

Bronchocentric Granulomatosis Key Facts Terminology Reaction pattern of lung parenchyma characterized by broncho- and bronchiolocentric necrotizing granulomatous inflammation Clinical Issues Most cases of BCG are allergic and associated with allergic…

Protocol for Lung Cancer Specimen Examination Lung: Resection Surgical Pathology Cancer Case Summary (Checklist) Specimen ____ Lung ____ Lobe(s) of lung (specify): ______________________________ ____ Bronchus (specify): ______________________________ ____ Other (specify):…

Desquamative Interstitial Pneumonia Key Facts Terminology A distinctive histologic reaction pattern to lung injury related to cigarette smoking Clinical Issues Dyspnea Cough Cessation of smoking generally leads to regression of…

Lymphangioleiomyomatosis Key Facts Terminology Lymphangioleiomyomatosis (LAM) Etiology/Pathogenesis Unknown etiology May share similar genetic relationship with tuberous sclerosis complex Clinical Issues Incidence More common in premenopausal women Rarely described in children…