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THINK TWICE: ANGIOEDEMA IN A PEDIATRIC PATIENT WITH MEDIASTINAL MASS AND LYMPHADENOPATHY
Abstract   Peer reviewed

THINK TWICE: ANGIOEDEMA IN A PEDIATRIC PATIENT WITH MEDIASTINAL MASS AND LYMPHADENOPATHY

C. Debnath, A. Doss and C. Horner
Annals of allergy, asthma, & immunology, Vol.135(5 Supplement 1), pp.S163-S163
11/2025
DOI: 10.1016/j.anai.2025.08.492

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Abstract

Introduction Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations. Lymphadenopathy can occur in up to 33% of SLE cases at presentation, while angioedema is rare. Diagnostic anchoring on more common etiologies can delay recognition of SLE, underscoring the need for a broad differential and reassessment with new clinical data, as demonstrated in our case. Case Description A 12-year-old male presented with acute dyspnea. Imaging revealed a 4.3 cm left upper mediastinal mass, multistation thoracic lymphadenopathy and left pleural effusion. A PET scan indicated extensive tracer-avid lymphadenopathy above and below the diaphragm, and a small pericardial effusion, initially concerning for lymphoma. The patient developed new lip and tongue angioedema without urticaria, partially responsive to antihistamines. Complement studies revealed low C3, C4, C1q, and CH50, but normal C1 esterase inhibitor levels and function, shifting the differential from histaminergic or hereditary angioedema to a consumptive process. Further history revealed intermittent arthritis involving the right finger and elbow. Extensive workup (see Figure 1) suggested SLE with renal involvement. However, as initial hospitalization focused on ruling out lymphoproliferative disease, the family opted for a second opinion at another hospital. Eventually, the patient was started on immunosuppressive therapy for SLE with improvement. Discussion Acquired angioedema with SLE can present with hypocomplementemia and transient low C1-INH level; however, our case did not, adding diagnostic complexity. Avoiding anchoring bias and recognizing atypical SLE presentations early are crucial for optimizing patient outcomes and preventing diagnostic delays.

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