The disorder is characterized by inflammation of the bronchioles and small pulmonary vessels that leads to fibrosis and destruction of alveolar cell walls.
Cigarette smoking is considered a risk factor.
Individuals with this disorder are also at increased risk for spontaneous pneumothorax . Symptoms include cough, chest pains, shortness of breath, fever , weight loss, and malaise . Bronchoscopy with biopsy confirms the diagnosis . Childhood forms exist and cause more systemic symptoms and findings such as failure to thrive , weight loss, fevers, irritability, rash , and bone pains. Treatment is with corticosteroids .