Background/Purpose: Acquired angioedema, defined as non-hereditary angioedema without associated urticaria, is an uncommon but potentially life-threatening feature among adults with systemic lupus erythematosus (SLE).  Although autoantibodies to C1 esterase inhibitor have been identified as a potential mechanism for non-histaminergic acquired angioedema in SLE, the exact pathophysiologic mechanisms remain unclear.  The incidence and prevalence of angioedema in pediatric SLE (pSLE) is unknown.  In this study, we sought to define and characterize acquired angioedema in pediatric systemic lupus erythematosus in a single pediatric rheumatology center.

Methods:  We conducted a chart review pSLE patients seen in a single center between 2000 and 2014. Data included family history and clinical presentation of angioedema.  

Results: A total of 160 pSLE patients were included.  Nine (6%) had history of acquired angioedema, 8 were females, 7 were African American, 2 were Hispanics.  Median age at diagnosis was 14 years, and with a mean follow up of 47 mos.  No patient had family history of angioedema.   Most common ACR SLE criteria met were hematologic (89%), arthritis (67%), renal (5 or 56%).   All 5 patients with renal disease had proliferative lupus nephritis, 2 of whom with Class IV lupus nephritis.  Two patients has neurologic involvement.  Immunoserologic features include antibodies to DAT (100%), dsDNA (89%), Sm (55%), RNP (6/9%), SSA (5/9%) and phospholipid (44%); significantly low C3 and C4 (100%) were noted in all patients.  C1 esterase function and nonfunction assays were normal or elevated.  Antibodies to C1 esterase were not noted in 4 patients in which the test was done.    Angioedema was present within 6 weeks of SLE diagnosis for 6 (67%) patients;  head and neck were the most common sites (8/9), with torso and extremity involvement in 4/9 patients.  No patient developed respiratory symptomatology.   Resolution of the angioedema was noted in all patients following initiation of corticosteroids treatment.  Angioedema was recurrent and feature of clinical disease flare in 2 patients (22%).

Conclusion: Acquired angioedema in a single center cohort of pSLE was described as disease manifestation.  Future research needs to address its underlying pathophysiologic mechanisms and its clinical significance in treatment and course of pSLE. 

Table 1 Clinical Presentation of Acquired Angioedema in 9 pSLE patients

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/characterization-of-pediatric-systemic-lupus-erythematosus-with-acquired-angioedema/