Antineutrophil cytoplasmic antibody (ANCA)-positive interstitial pneumonia (IP) is reported as IP that is ANCA-positive and does not involve organ damage associated with vasculitis other than the lungs. While the combination of glucocorticoid and rituximab is effective in ANCA-associated vasculitis, the treatment strategy for ANCA-positive IP has not been established. Here, we report the first case of successful treatment of proteinase 3 (PR3)-ANCA-positive IP with a moderate dose of glucocorticoid and rituximab. The patient was an 80-year-old male who presented with subacute dry cough and dyspnoea. Blood tests revealed elevated levels of C-reactive protein, Krebs von den Lungen 6 (KL-6), and PR3-ANCA. Chest computed tomography (CT) showed interstitial shadows and infiltrates around honeycomb cysts. 18F-fluorodeoxyglucose (FDG) positron emission tomography CT revealed an uptake of FDG in the IP area. After starting treatment with a moderate dose of prednisolone and rituximab, the patient's clinical symptoms disappeared, C-reactive protein and KL-6 turned to be normal, and infiltrates around the cysts of honeycomb lungs disappeared. Prednisolone was gradually decreased to 2 mg, and no relapse or adverse events were observed during the course of treatment. Our case suggests that early treatment with a moderate dose of glucocorticoid and rituximab is effective for PR3-ANCA-positive IP.
Keywords: Antineutrophil cytoplasmic antibody; PR3-ANCA; interstitial pneumonia; rituximab; vasculitis.
© Japan College of Rheumatology 2023. Published by Oxford University Press.