Isolated Major Organ Involvement as the Initial Presentation of Systemic Lupus Erythematosus: A Diagnostic Challenge from a Tertiary Care Center

Background: Systemic lupus erythematosus (SLE) typically presents with multisystem involvement; however, some patients present with isolated major organ disease, leading to diagnostic delay. Objectives: The objective of this study was to evaluate the frequency, clinical characteristics, and immunological profile of SLE patients presenting with isolated major organ involvement at disease onset. Materials and Methods: Adult patients fulfilling the Systemic Lupus International Collaborating Clinics 2012 criteria were evaluated. Patients presenting with isolated renal, neuropsychiatric, or cardiopulmonary involvement without classical features were identified. Results: Out of 45 SLE patients, 18 (40%) presented with isolated major organ involvement. Renal involvement was the most common isolated presentation. All patients showed antinuclear antibody positivity and hypocomplementemia. Conclusion: Isolated major organ involvement is a common and diagnostically challenging initial presentation of SLE.