POS0750 THE CHARACTERISTICS OF SYSTEMIC LUPUS ERYTHEMATOSUS WHO HAVE CONSTITUTIONAL SYMPTOMS AND LYMPHADENOPATHY
Authors: Y. Erez, S. Gulle, A. Karakas, R. Bekis, A. Balci, M. Birlik, G. Can, F. Onen, İ. Sari
Journal: Annals of the Rheumatic Diseases (2021)

Background: Systemic lupus erythematosus (SLE) is the prototype of systemic autoimmune diseases. It may affect any organ system and may present with a variety of clinical symptoms. Lymphadenopathy (LAP) is a nonspecific finding of SLE and often associated to the active disease state. LAP associated SLE pose a significant diagnostic challenge especially in the presence of constitutional symptoms. Objectives: In this retrospective cohort study, we evaluated the clinical and imaging characteristics of SLE patients who presented with LAP and constitutional symptoms. Methods: SLE Patients with lymphadenopathy who had constitutional symptoms were reviewed retrospectively. The characteristics of the patients such as age, sex, disease duration, clinical features, and laboratory tests including autoantibodies against ANA, dsDNA and ENA were recorded. Imaging findings such as positron emission tomography–computed tomography (PET/CT), thoracal or abdominal CT or diagnostic lymph node biopsy were identified. Results: There were 28 patients in this study. The mean age at the time of LAP was 38.9±15.2 years, 22 (78.6%) were female. All patients were fulfilling SLICC 2012 classification criteria for SLE. LAP was the presenting feature of SLE in 15 (53.6%) patients. The most common clinical features associated with LAP were arthritis (42.8%), fever (35.7%), acute lupus rash (32.1%) and serositis (28.5%). There were 11 (39.2%) who had significant proteinuria and 4 (14.3%) had class III or IV renal histology. Anti-phospholipid antibodies were present in nearly one-third of the patients and 12 of them had venous or arterial thrombosis or pregnancy co-morbidity. Cervical LAP was present in 57.1%, axillary LAP in 82.1% and inguinal in 36%. On CT, the prevalence of thoracal and abdominal LAP and hepatomegaly and splenomegaly were 61.5%, 37,5%, 40% and 16% respectively. The pericardial and pleural effusion and intra-abdominal free fluid were 19.2%, 30.8% and 10.2% respectively. There were 19 (67.8%) patients who underwent PET/CT imaging and all were reported as reactive LAP. In terms of SUV max 7 (36.8%) of them had a threshold ≥2.5. 17 (60.7%) patients required lymph node biopsy; 2 did not include lymphoid tissue, 4 (30.7%) with atypical lymphoid hyperplasia suggestive of viral infection and remaining were reactive lymphoid hyperplasia. Autoantibody testing showed that anti-dsDNA was positive in 66.7% and the most common anti-ENAs were anti-nucleosome (35.7%) and anti-Sm-RNP (35.7%) followed by anti-SSA (25%), anti-ribosomal P (17.9%) and anti-histone (14%). The clinical and laboratory features of the clinical and laboratory features of the group is summarized in Table 1. Table 1. Data at the time of LAP Age, years 38.9±15.2 Disease duration, months 3 (2- 300) SLE-DAI, median (min-max) 9 (2-30) Sex, female n(%) 22 (78.6) Clinical features n (% ) Acute cutaneous 9 (32.1) Arthritis 12 (42.8) Fever 10 (35.7) Other constitutional symptoms 18 (64.2) Serositis 8 (28.5) Renal involvement 11 (39.2) Neurologic involvement 1 (3.6) Leucopenia 7 (25.0) Trombositopenia 5 (17.8) Hemolitic anemia 7 (25.0) ANA (+)&gt;1/320 28 (100) Anti-dsDNA (+) 18 (66.7) Anti-Sm(+) 5 (17.9) Anti-phospholipid antibody (+) 9 (32.1) Low complement 16 (88.5) Conclusion: We showed that SLE patients who had constitutional findings and LAP could be the presenting feature and may confer a significant diagnostic challenge in which the patients might require invasive procedures and advanced imaging modalities. PET/CT revealed reactive changes in lymph nodes in all patients but lymph node biopsy revealed atypical changes in some patients suggestive of viral infections. On the other hand, LAP patients with constitutional symptoms represents a severe phonotype of the SLE as there were significant renal and thrombotic disease. Disclosure of Interests: None declared