Background/Purpose: While lymphadenopathy and/or lymphadenitis (LAD) is considered a relatively common clinical finding in SLE patients, its clinical significance is poorly understood. Previous studies described the prevalence of lymphadenopathy, but not the prognostic significance and/or characteristic serologic associations. We report the prevalence, type of LAD, and the phenotypical profile of SLE patients who develop this manifestation over the course of their disease.

Methods: We conducted a retrospective case series analysis on 220 SLE patients enrolled at Beth Israel Deaconess Medical Center Lupus Cohort in 2008-2019 comparing patients with and without LAD. Patients were recruited from the Rheumatology clinic and eligible to participate if they met 1994 ACR SLE criteria and provided informed consent. Data on LAD was obtained from physical exam documentation and/or imaging reports.  When available, pathology samples were reviewed from tissue obtained during routine clinical care.  The study was IRB approved. Statistical analysis: Chi-square Test and Fisher’s exact test were used for categorical variables using VassarStats.

Results: In our cohort, 13% (28/220) of patients developed LAD, a significantly lower proportion than previously reported (Dubois et al, JAMA 1964). LAD typically was a presenting and/or early manifestation occurring within the 12 months of diagnosis for most patients (82%) and typically predicted more active clinical course. The mean duration of time since SLE diagnosis for all patients was 15.2 years.    Within the LAD group, 19 patients underwent lymph node biopsy as part of their workup.  The predominant pathology patterns were necrotizing (26%), reactive (47%), and proliferative (16%) lymphadenitis. Of note, one biopsy revealed Non-Hodgkin lymphoma, one patient had sequential biopsies with initial reactive hyperplasia followed by diffuse large B cell lymphoma on a deeper pelvic node, and one biopsy was non diagnostic.   We then asked whether the disease phenotype was different between the LAD group and the non-LAD group. Patients with LAD were more likely than non-LAD patients to have fever (OR 5.4, p=< 0.0001) nephritis (OR 1.7, p=0.1897), splenomegaly (OR 22.9, p=0.0076), and leukopenia (OR 2.0, p=0.0969). LAD patients also had more serologically active disease than non-LAD patients; specifically LAD patients were more likely to have positive Smith (OR 4.18, p=0.005), RNP (OR 4.05, p=0.0087), and SSB (OR 2.41, p=0.0424) as well as hypocomplementemia (C3 OR 4.80, p=0.0051; C4 OR 4.03, p=0.0039).

Conclusion: In our cohort, lymphadenopathy was an uncommon SLE manifestation. Interestingly, necrotizing LAD, which is often associated with infection, was relatively prevalent in our cohort.  Our data suggest that patients with lymphadenopathy are more likely to have constitutional symptoms, cytopenias, and splenomegaly as well as increased auto-antibodies and hypocomplementemia. These symptoms can be seen with lymphomas and two patients were ultimately found to have lymphoma (DLBCL and NHL). These patients developed LAD either within 12 months or after more than 10 years of SLE diagnosis. This suggests that biopsy is warranted in SLE patients with LAD at any point in their disease course.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-of-lymphadenopathy-in-systemic-lupus-erythematous-a-case-control-study-from-a-tertiary-care-center/