SESSION TITLE: Critical Care Student/Resident Case Report Posters III
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Autoimmune/Inflammatory Syndrome Induced by Adjuvants (ASIA) is a rare disorder recently introduced in the literature. Autoimmune phenomena associated with adjuvant exposure has long been suggested, though this has only recently been recognized as an inflammatory syndrome, rarely leading to multi-organ failure.
CASE PRESENTATION: A 50 year old male to female transgender patient who participated in silicone “parties” where liquid silicone was injected into her cheeks and hips experienced myalgias, arthralgias and insomnia for months prior to developing acute dyspnea and a petechial rash. The clinical course was complicated by respiratory failure, myocardial infarction, acute encephalopathy and acute renal failure requiring supportive care. Laboratory investigations revealed low complement levels, cryoglobulinemia and an elevated rheumatoid factor as well as the presence of HLA-DRB1/DQB1. Skin biopsy demonstrated leukocytoclastic vasculitis; renal biopsy showed acute vasculitic glomerulonephritis. Brain imaging revealed small vessel vasculitis. Pulse-dose steroids were administered without improvement. The patient subsequently underwent plasma exchange followed by Rituximab, resulting in rapid improvement of renal, pulmonary and neurologic symptoms.
DISCUSSION: Silicone and silicone containing compounds have been recognized to be toxic to humans. Silicone in nature, commonly found as silicone dioxide (silica), is the pathogenic agent implicated in several occupational lung diseases such as silicosis and asbestosis. In vitro, silica and its derivatives are potent macrophage activators resulting in a massive production of IL-17 leading to an influx of activated neutrophils. This cascade results in the transportation of silica through the lymphatic system. Within regional lymph nodes, silica's immune adjuvant effect stimulates the production of IgE and IgG, ultimately leading to chronic T-cell activation. Despite these documented effects of silica, silicone remains largely inert. However, there is convincing evidence implicating the injection of liquid silicone in the development of severe autoimmune disease. In 2010, Shoenfeld et. al. proposed the criteria for the diagnosis of ASIA (table 1) in effect consolidating multiple clinical symptoms associated with various adjuvants. The patient met 3 major and 2 minor criteria.
CONCLUSIONS: ASIA represents a rare cause of inflammatory disease. In patients with chronic adjuvant exposure, clinical suspicion should be maintained to diagnose this potentially life threatening condition.
Reference #1: Shoenfeld, Yehuda, & N Agmon-Levin. "ASIA-autoimmune/inflammatory syndrome induced by adjuvants." J of Autoimm 36.1 (2011): 4-8.
Reference #2: Tervaert, et. al. "Silicone implant incompatibility syndrome (SIIS): A frequent cause of ASIA (Shoenfeld’s syndrome)." Imm res 56.2-3 (2013): 293-298.
Reference #3: Vera-Lastra, O., et al. "Human adjuvant disease induced by foreign substances: a new model of ASIA (Shoenfeld's syndrome)." Lupus 21.2 (2012): 128-135.
DISCLOSURE: The following authors have nothing to disclose: Tomer Pelleg, Faisal Siddiqui, Susanti Ie, Edmundo Rubio, Mahtab Foroozesh
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