Background. Overlap syndromes involving systemic lupus erythematosus (SLE) and ANCA-associated vasculitis (AAV) are increasingly recognized but remain diagnostically and therapeutically challenging – especially when complicated by progressive interstitial lung disease (ILD) of the usual interstitial pneumonia (UIP) pattern. The presence of dual seropositivity, extrapulmonary manifestations such as scleritis, and atypical ILD patterns complicates clinical decision-making. Objective. To provide a comprehensive narrative review of SLE–ANCA overlap syndromes with a focus on interstitial lung involvement, supported by a clinically illustrative case of a patient with SLE, ANCA positivity, anterior scleritis, and UIP-pattern ILD. Methods. A selective, non-systematic review of the literature (2000–2025) was performed across PubMed, Scopus, and Google Scholar. Seven original studies were included and analyzed to identify clinical, serological, radiological, and histopathological patterns in SLE–ANCA overlap syndromes with ILD. Data were summarized narratively and presented in comparative tables. Case summary. A 46-year-old female with a prior SLE diagnosis presented with exertional dyspnea, anterior scleritis, and radiological features consistent with UIP. She exhibited ANA and dual ANCA positivity, along with elevated inflammatory markers. Due to cyclophosphamide intolerance, she was treated with corticosteroids and rituximab. This regimen led to improved pulmonary function, radiological stability, and resolution of ocular inflammation. Conclusion. SLE–ANCA overlap with UIP-ILD represents a rare but clinically aggressive autoimmune intersection. Early diagnosis through extended serological testing, high-resolution imaging, and multidisciplinary evaluation is essential. B-cell– targeted therapy, such as rituximab, offers a promising steroid-sparing option in complex presentations, particularly for patients intolerant to cyclophosphamide. © 2025 Elsevier B.V., All rights reserved.