Spontaneous improvement of interstitial pneumonia with autoimmune features: A case report

Interstitial pneumonia with autoimmune features (IPAF) was proposed to describe patients with interstitial lung disease who do not meet the classification criteria for a defined connective tissue disease. Here, we report a spontaneous improvement case of IPAF. A 58-year-old man developed dry cough and dyspnoea. Positive result was obtained for the anti-centromere antibody. High-resolution computed tomography (HRCT) showed reticular abnormalities and ground-glass opacities. Cryobiopsy specimens revealed cellular non-specific interstitial pneumonia. The patient displayed periungual erythema and nail fold bleeding, but no sclerosis. He did not meet the criteria for systemic scleroderma, but did meet those for IPAF. Because symptoms slightly improved, the patient declined immunosuppressive treatment. After 6 months, repeated HRCT showed an apparent reduction in the area of ground-glass opacities. The forced vital capacity improved from 2.72 to 3.47 L and serum Krebs von den Lungen (KL)-6 decreased from 1977 to 531 U/ml, and symptoms disappeared.