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Updated: Oct 15, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Spontaneous improvement of interstitial pneumonia with autoimmune features: A case report
Hirotsugu Ohkubo1, Akiko Nakano1, Kohei Fujita1
1Department of Respiratory Medicine, Allergy and Clinical Immunology Nagoya City University Graduate School of Medical Sciences Nagoya Japan.
Abstract:
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.
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