PO:10:149 | Efficacy of anifrolumab in a case of systemic lupus erythematosus with pulmonary involvement

Fabio Brandolino¹, Giulia Pazzola¹, Luca Ronzoni³, Andreina Teresa Manfredi^1|2, Carlo Salvarani^1|2 | ¹SC Reumatologia, IRCCS Arcispedale Santa Maria Nuova, AUSL Reggio Emilia; ²Università di Modena e Reggio Emilia, Scuola di specializzazione in Reumatologia, Modena; ³SC Malattie dell'Apparato Respiratorio, IRCCS Arcispedale Santa Maria Nuova, AUSL Reggio Emilia, Italy

Background. Pulmonary involvement in systemic lupus erythematosus (SLE) is a heterogeneous and usually non-severe complication, affecting 50–70% of patients (and up to 4–5% at disease onset). Pleuro-parenchymal manifestations include pleuritis (30%), interstitial lung disease (ILD, 1–15%), and lupus pneumonitis (1–4%), the latter often requiring differential diagnosis from infectious forms. Vascular manifestations comprise pulmonary arterial hypertension (PAH, 0.5–17.5%), diffuse alveolar hemorrhage (DAH, 1–5.4%), and venous thromboembolism (VTE, 9%; 35–42% in the presence of antiphospholipid antibodies), as well as acute reversible hypoxemia (ARH). Rare forms include airway disease and shrinking lung syndrome. Anifrolumab, a monoclonal antibody that blocks the type I interferon receptor, has shown efficacy in moderate to severe SLE, particularly for mucocutaneous, articular, and serological manifestations. According to European guidelines, Anifrolumab is recommended as first-line therapy for extra-renal (non–major organ) manifestations, especially cutaneous, and as add-on therapy for severe disease. We report a case of SLE with lupus pneumonitis and antiphospholipid syndrome (APS) successfully treated with Anifrolumab.

Materials and Methods. A 45-year-old Caucasian man, with no significant past medical history, experienced three episodes of bilateral pneumonia (bibasilar consolidations) since mid-2022, all requiring hospitalization. One episode was complicated by pulmonary embolism and another by a generalized rash, both treated with antibiotics and corticosteroids. Laboratory findings revealed positivity for ANA (1:2560, homogeneous nuclear pattern), anti-dsDNA, anti-Ro52, anti-nucleosome, anti-histone antibodies, rheumatoid factor, and lupus anticoagulant. Rheumatologic assessment revealed Jaccoud arthropathy and widespread erythematous maculopapular lesions on the trunk, back, and limbs. Given the absence of infectious findings (including negative BAL), the pulmonary abnormalities were attributed to autoimmune disease. A diagnosis of SLE with cutaneous involvement (histologically confirmed subacute cutaneous lupus erythematosus), pulmonary and articular manifestations, and associated APS was established. The patient started oral anticoagulation, high-dose corticosteroids, hydroxychloroquine (discontinued due to cutaneous reaction), and rituximab (1 g × 2), achieving initial improvement. Rituximab was discontinued after the second course due to an adverse reaction, followed by recurrence of bilateral pneumonia and cutaneous flare. Consequently, Anifrolumab 300 mg every 4 weeks was initiated.

Results. After six months of Anifrolumab therapy, HRCT showed marked improvement, inflammatory markers normalized, and the patient reported substantial improvement in respiratory, cutaneous, and articular symptoms, allowing progressive corticosteroid tapering.

Conclusions. Anifrolumab represents a promising therapeutic option for patients with severe SLE manifestations who are refractory to or intolerant of conventional treatments. Modulation of the type I interferon signaling pathway may play a key role in achieving systemic disease control. In patients with pulmonary involvement, a multidisciplinary approach and careful differentiation from infectious causes are essential.