Purpose of Review <p>Lymphoproliferative disorders encompass a variety of hematologic diseases that are increasingly recognized. Patients with these conditions may present to rheumatologists or other specialists prior to a hematologist-oncologist. This review is intended to highlight key distinguishing features between rheumatologic conditions and lymphoproliferative disorders, and suggest diagnostic approaches.</p> Recent Findings <p>Recent studies have shown that lymphoproliferative diseases can mimic rheumatologic disease, and differentiation is complex. Advances in imaging techniques such as FDG PET/CT, and improved understanding of lymph node histopathology have improved diagnostic accuracy. Attention to specific details such as the pattern of organ involvement, the presence or absence of key clinical features, and the consistency of treatment response can assist in accurate diagnosis.</p> Summary <p>Our review highlights the importance of considering lymphoproliferative disorders in the differential diagnosis of rheumatologic disease. A thorough clinical history, targeted biopsies, and collaboration with hematopathologists are essential for timely diagnosis and care. Future research should concentrate on identifying biomarkers and formulating diagnostic algorithms to aid in differentiating lymphoproliferative disorders from autoimmune diseases, which will ultimately improve patient outcomes.</p>