Piero Ruscitti1,2, Daniela Iacono3,4, Francesco Ciccia3,4, Giacomo Emmi3,4, Paola Cipriani3,4, Rosa Daniela Grembiale3,4, Federico Perosa3,4, Lorenzo Emmi3,4, Giovanni Triolo3,4, Roberto Giacomelli3,4, Gabriele Valentini3,4. 1. From the Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila; Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples; Rheumatology Section, Department of Internal Medicine, University of Palermo, Palermo; Department of Experimental and Clinical Medicine, University of Florence, Florence; Department of Health Sciences, University of Catanzaro "Magna Graecia," Catanzaro; Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy. pieroruscitti@live.com piero.ruscitti@graduate.univaq.it. 2. P. Ruscitti, MD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; D. Iacono, MD, Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples; F. Ciccia, MD, PhD, Rheumatology Section, Department of Internal Medicine, University of Palermo; G. Emmi, MD, PhD, Department of Experimental and Clinical Medicine, University of Florence; P. Cipriani, MD, PhD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; R.D. Grembiale, MD, Department of Health Sciences, University of Catanzaro "Magna Graecia"; F. Perosa, MD, PhD, Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School; L. Emmi, MD, Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School; G. Triolo, MD, Rheumatology Section, Department of Internal Medicine, University of Palermo; R. Giacomelli, MD, PhD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; G. Valentini, MD, Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples. pieroruscitti@live.com piero.ruscitti@graduate.univaq.it. 3. From the Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila; Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples; Rheumatology Section, Department of Internal Medicine, University of Palermo, Palermo; Department of Experimental and Clinical Medicine, University of Florence, Florence; Department of Health Sciences, University of Catanzaro "Magna Graecia," Catanzaro; Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy. 4. P. Ruscitti, MD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; D. Iacono, MD, Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples; F. Ciccia, MD, PhD, Rheumatology Section, Department of Internal Medicine, University of Palermo; G. Emmi, MD, PhD, Department of Experimental and Clinical Medicine, University of Florence; P. Cipriani, MD, PhD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; R.D. Grembiale, MD, Department of Health Sciences, University of Catanzaro "Magna Graecia"; F. Perosa, MD, PhD, Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School; L. Emmi, MD, Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School; G. Triolo, MD, Rheumatology Section, Department of Internal Medicine, University of Palermo; R. Giacomelli, MD, PhD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; G. Valentini, MD, Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples.
Abstract
OBJECTIVE: Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, which can complicate adult-onset Still disease (AOSD). We investigated AOSD clinical features at the time of diagnosis, to assess predictors of MAS occurrence. Further, we analyzed the outcomes of patients with AOSD who experience MAS. METHODS: Patients with AOSD admitted to any Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale center were retrospectively analyzed for features typical of AOSD, MAS occurrence, and their survival rate. RESULTS: Of 119 patients with AOSD, 17 experienced MAS (12 at admission and 5 during followup). Twelve patients with MAS at first admission differed from the remaining 107 in prevalence of lymphadenopathy and liver involvement at the time of diagnosis. In addition, serum ferritin levels and systemic score values were significantly higher in the patients presenting with MAS. At the time of diagnosis, the 5 patients who developed MAS differed from the remaining 102 in the prevalence of abdominal pain, and they showed increased systemic score values. In the multivariate analysis, lymphadenopathy (OR 7.22, 95% CI 1.49-34.97, p = 0.014) and abdominal pain (OR 4.36, 95% CI 1.24-15.39, p = 0.022) were predictive of MAS occurrence. Finally, MAS occurrence significantly reduced the survival rate of patients with AOSD (p < 0.0001). CONCLUSION: MAS occurrence significantly reduced the survival rate in patients with AOSD. Patients with MAS at baseline presented an increased prevalence of lymphadenopathy and liver involvement, as well as high serum ferritin levels and systemic score values. The presence of lymphadenopathy and abdominal pain was associated with MAS occurrence.
OBJECTIVE:Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, which can complicate adult-onset Still disease (AOSD). We investigated AOSD clinical features at the time of diagnosis, to assess predictors of MAS occurrence. Further, we analyzed the outcomes of patients with AOSD who experience MAS. METHODS:Patients with AOSD admitted to any Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale center were retrospectively analyzed for features typical of AOSD, MAS occurrence, and their survival rate. RESULTS: Of 119 patients with AOSD, 17 experienced MAS (12 at admission and 5 during followup). Twelve patients with MAS at first admission differed from the remaining 107 in prevalence of lymphadenopathy and liver involvement at the time of diagnosis. In addition, serum ferritin levels and systemic score values were significantly higher in the patients presenting with MAS. At the time of diagnosis, the 5 patients who developed MAS differed from the remaining 102 in the prevalence of abdominal pain, and they showed increased systemic score values. In the multivariate analysis, lymphadenopathy (OR 7.22, 95% CI 1.49-34.97, p = 0.014) and abdominal pain (OR 4.36, 95% CI 1.24-15.39, p = 0.022) were predictive of MAS occurrence. Finally, MAS occurrence significantly reduced the survival rate of patients with AOSD (p < 0.0001). CONCLUSION: MAS occurrence significantly reduced the survival rate in patients with AOSD. Patients with MAS at baseline presented an increased prevalence of lymphadenopathy and liver involvement, as well as high serum ferritin levels and systemic score values. The presence of lymphadenopathy and abdominal pain was associated with MAS occurrence.