interstitial lung disease 1

An interstitial lung disease characterized by a progressive remodeling of the alveolar interstitium that has_material_basis_in heterozygous mutation in the SFTPA1 gene on chromosome 10q22.

Signs and symptoms

• Dyspnea
• Crackles
• Ground-glass opacification
• Interlobular septal thickening
• Cough
• Usual interstitial pneumonia
• Restrictive ventilatory defect
• Elevated bronchoalveolar lavage fluid eosinophil proportion
• Elevated bronchoalveolar lavage fluid neutrophil proportion
• Decreased DLCO

Also known as: Idiopathic pulmonary fibrosis