Table 3.

Clinical Characteristics of Pleuroparenchymal Fibroelastosis

No gender preponderance Age at onset Wide-ranging, younger than in idiopathic pulmonary fibrosis (IPF) Smoking history Unrelated to the incidence of PPFE Clinical symptoms Exertional dyspnea and dry cough with insidious onset Chest pain due to pneumothorax Loss of body weight Physical findings Slender stature and flattened thoracic cage Crackles sometimes audible Serum biomarkers KL-6 within the normal or around the upper normal limit Elevated Surfactant protein D (SP-D) Autoantibodies such as rheumatoid factor and antinuclear antibody sometimes elevated Prognosis Wide-ranging in each case studies from slowly progressive with 10 - 20 years of presentation to rapidly progressive course Poorer prognosis of secondary PPFE such as transplantation- associated PPFE