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Objectives Acute lupus pneumonitis (ALP) is a rare manifestation of Systemic Lupus Erythematous (SLE). We report a case of recurrent ALP treated with anifrolumab.Methods A 47-year-old man presented in August 2022 with progressive dyspnea. Tests showed normal blood count, C-reactive protein (CRP) of 20.22 mg/dL, negative procalcitonin. Chest CT revealed bilateral lung consolidations. No improvement was seen on antibiotics. Corticosteroid therapy led to rapid improvement. He was discharged on prednisone 50 mg/daily.In November 2022, he developed a new episode of acute respiratory failure. CT angiography showed new consolidations and a pulmonary embolism. He was initiated on apixaban and steroids, with resolution of symptoms.In July 2023, after suspension of steroids, a relapse occurred, confirmed by CT, with no relapse of pulmonary embolism. Bronchoalveolar lavage (BAL) for infections was negative. Autoimmune panel revealed ANA 1:2560 homogeneous, anti-Ro52, anti-DNA (IF and EIA) positivity. Lupus anticoagulant was positive. A rheumatologic evaluation revealed knee synovitis, Jaccoud’s arthropathy, and acute cutaneous lupus (ACLE), leading to a diagnosis of SLE. Prednisone and rituximab were initiated; it was suggested to switch to warfarin, not initiated because of the patient’s poor compliance.In March 2024 he relapsed with new pulmonary embolism and consolidations, with a concomitant cutaneous flare. Rituximab was reattempted but discontinued due to adverse reactions; apixaban was switched to warfarin.Results In July 2024, there was a new pulmonary relapse. There were complement consumption, increased levels of anti-dsDNA and elevated inflammatory markers. Diffuse ACLE flare was noted. Due to the patient’s condition, BAL was deferred. He was successfully treated with antibiotics and methylprednisolone. Anifrolumab was then started in August 2024.By November 2024, there was significant radiological improvement (figure 1). At last follow-up in October 2025, no recurrence was seen on prednisone 2.5 mg/daily (cSLEDAI=0).Abstract PO:04:101 Figure 1Conclusions This is, to our knowledge, the first report of the use of anifrolumab in acute pulmonary involvement. The main limitation is that BAL was not performed during the last relapse. Nevertheless, BAL had already been performed, and the concomitant presence of ACLE flare, increased anti-dsDNA levels, complement consumption, negative infectious tests, and steroid responsiveness strongly support the diagnosis of ALP recurrence.