Fibrosing Alveolitis in Patients with Neurofibromatosis

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Abstract
Fibrosing alveolitis, or intestitial pulmonary fibrosis, is a common manifestation of neurofibromatosis and was observed in 7 of 70 patients with the disease. Though neurofibromatosis is congenital, fibrosing alveolitis does not appear until adulthood and occurs in 20% of patients with the disease who are over 30 yr old. Characteristic radiographic findings include linear, interstitial density and large upper lobe bullae. This combination limits the differential diagnosis. Pathological examination demonstrates alveolar wall thickening progressing to fibrosis and lung destruction. Pulmonary function tests can show obstructive or restrictive lung disease.

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