AB0647 CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME TRIGGERED BY CONCURRENT VZV AND HSV INFECTION. A CASE REPORT
Authors: L. Kakoullis, K. Parperis, S. Psarelis, E. Papachristodoulou, G. Panos
Journal: Annals of the Rheumatic Diseases (2021)

Background: Catastrophic antiphospholipid syndrome (CAPS) is a severe autoimmune condition, characterized by multiorgan failure due to thromboses and/or hemorrhage. Concurrent HSV/VZV infection is an exceedingly rare event, occurring mostly in immunocompromised patients. Herein, we report the first case of CAPS triggered by concurrent HSV/VZV infection, manifesting with zosteriform rash and haemorrhages at multiple sites. Objectives: To describe a case of CAPS provoked by concurrent HSV/VZV infection, manifesting with zosteriform rash and haemorrhages at multiple sites. Methods: A 36-year-old male, with a history of antiphospholipid syndrome (APS) presented with a 5-day history of epigastric pain, associated with fever, hematuria, rash and swelling in bilateral ears. Past medical history was remarkable for APS, based on 4 episodes of DVT/PE and triple positive antiphospholipid antibodies, on chronic well-maintained warfarin anticoagulation. On examination, a purpuric rash was evident on the right side of the neck as well as on both ears, consistent with a C3 dermatomal distribution (Figure 1). Laboratory studies revealed anemia, thrombocytopenia and acute kidney injury. Figure 1. Results: A full-body CT scan demonstrated the presence of multifocal lymphadenopathy, alveolar infiltrates suggestive of diffuse alveolar haemorrhage, and haemorrhage in both kidneys and the left adrenal gland; anticoagulation was held, despite the INR being within the patient’s baseline therapeutic levels. He was admitted with a working diagnosis of CAPS and possible zoster infection. Appropriate immunologic workup was requested. He was prescribed intravenous acyclovir, antibiotics, pulse dose of glucocorticoids, and IVIG. Bronchoscopy with bronchoalveolar lavage revealed the presence of haemosiderin-laden macrophages. The rash regressed, and the patients’ condition improved. He was discharged with glucocorticoid tapering regimen, hydroxychloroquine, aspirin and warfarin. Antibody titers were taken prior to IVIG administration at presentation, and at 4 weeks. High VZV IgG titers found at presentation regressed over four times on follow-up. Furthermore, at 4 weeks the patient had developed IgM antibodies against both VZV and HSV. These findings confirmed a concurrent infection. Conclusion: This is the first report of coexisting HSV/VZV infection associated with CAPS. A literature review identified a total of 28 patients with coexisting HSV/VZV infection, whereas only one case of CAPS triggered by HSV was identified 1 . This case illustrates that concurrent infection can occur in the absence of immunosuppressive therapy in patients with APS, serving as a trigger for hemorrhagic CAPS. Simultaneous treatment with antiviral against herpesviruses, glucocorticoids and IVIG may mitigate the inflammatory cascade associated with CAPS. References: [1]Catoggio C, Alvarez-Uría A, Fernandez PL, Cervera R, Espinosa G. Catastrophic antiphospholipid syndrome triggered by fulminant disseminated herpes simplex infection in a patient with systemic lupus erythematosus. Lupus. 2012 Oct;21(12):1359-61. Disclosure of Interests: None declared.