{
  "abstract": "Objectives Systemic lupus erythematosus (SLE) and hypocomplementemic urticarial vasculitis syndrome (HUVS) are both characterized by immune complex deposition and systemic inflammation. It has been hypothesized that impaired DNASE1 activity may represent a common pathogenic mechanism underlying both conditions, through defective clearance of extracellular DNA, leading to its accumulation, enhanced autoantibody production, and heightened activation of the type I interferon (IFN-I) pathway. Blocking the IFN-I receptor with anifrolumab may represent a potential therapeutic approach for these patients. We describe the clinical course and treatment response in three patients with SLE-associated HUVS treated with anifrolumab.Methods We present three patients with SLE-associated HUVS, all of whom were refractory to previous therapies and were followed over one year. Disease activity (SLEDAI-2K and SLE-DAS), joint involvement (28-joint count), flare frequency, lung function parameters, and daily glucocorticoid dose were assessed at baseline, and after 6 and 12 months of anifrolumab treatment.Results Detailed baseline characteristics of our patients are shown in table 1. All patients had moderate to severe disease activity at baseline, with SLEDAI-2 score ranging from 16 to 22, and SLE-DAS between 13.14-40.17. All patients had significantly reduced diffusion capacity for carbon monoxide (DLCO). Pulmonary function testing revealed an obstructive pattern consistent with small-airway involvement characteristic of HUVS-related lung disease. After 12 months of anifrolumab therapy, there was a marked reduction in SLEDAI-2K (ranging from 2-12) and SLE-DAS (ranging from 1.12-8.67) in all patients. Complete resolution of cutaneous manifestations and improvement in lung function were also recorded. There were no flares during the treatment period. Additionally, the prednisone dose was reduced in all patients. There were no adverse events associated with the anifrolumab treatment.Abstract PO:11:288 Table 1Baseline characteristics of patientsConclusions Anifrolumab was well tolerated and associated with a marked reduction in disease activity, improvement in pulmonary and cutaneous manifestations, and glucocorticoid tapering in patients with refractory SLE-associated HUVS.",
  "authors": [
    {
      "affiliations": [
        "University Clinical Centre of Serbia, Clinic of Allergy and Immunology, Belgrade, Serbia",
        "University of Belgrade, Faculty of Medicine, Belgrade, Serbia"
      ],
      "name": "Maja Stojanovic"
    },
    {
      "affiliations": [
        "University Clinical Centre of Serbia, Clinic of Allergy and Immunology, Belgrade, Serbia"
      ],
      "name": "Sara Radovic"
    },
    {
      "affiliations": [
        "University Clinical Centre of Serbia, Clinic of Allergy and Immunology, Belgrade, Serbia"
      ],
      "name": "Ana Drazic"
    },
    {
      "affiliations": [
        "University Clinical Centre of Serbia, Clinic of Allergy and Immunology, Belgrade, Serbia",
        "University of Belgrade, Faculty of Medicine, Belgrade, Serbia"
      ],
      "name": "Aleksandra Plavsic"
    },
    {
      "affiliations": [
        "University Clinical Centre of Serbia, Clinic of Allergy and Immunology, Belgrade, Serbia",
        "University of Belgrade, Faculty of Medicine, Belgrade, Serbia"
      ],
      "name": "Rada Miskovic"
    }
  ],
  "title": "PO:11:288 Anifrolumab for the treatment of refractory systemic lupus erythematosus-associated hypocomplementemic urticarial vasculitis: report of three cases",
  "uid": "9e1702a0-97ca-51a1-85eb-73e72aa60714"
}