THU0558 ADULT-ONSET STILL’S DISEASE PROGNOSIS SCORE. CLINICAL PATTERNS, COMPLICATIONS AND BIOLOGIC TREATMENT
Authors: Ivette Casafont-Solé, Susana Holgado, J. Narváez, Maribel Mora, Josep Roca, Anahy Brandy-Garcia, Lourdes Mateo, Melania Martínez-Morillo, Laia Gifre, Maria Aparicio Espinar, Águeda Prior-Español, Anne Riveros, Clara Sanguesa, Jordi Camins-Fàbregas, Annika Nack, Joan Miquel Nolla, Alejandro Olive
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Adult-onset Still's disease (AOSD) is an uncommon disease with an unpredictable clinical course and variable prognosis. Sometimes, it requires biologic treatment in early phases. A prognosis score has been described, which has never been applied in a Spanish case series. <h3>Objectives:</h3> To apply the prognosis score described by <i>Pouchot et al</i> (Systemic Score System (SSS)) on a 64 cases series diagnosed with AOSD in Spanish population and to determine if SSS high values registered at the onset of the pathology are related to AOSD clinical patterns (monocyclic, polycyclic and chronic course), requirement of biologic treatment along the disease's course and development of AOSD clinical complications. To establish the relationship between its value and the AOSD-related mortality. <h3>Methods:</h3> Retrospective study realized in two University Hospitals. Clinical, laboratory, AOSD-related complications data, administered biologic treatments and number of deaths (AOSD related or not) were recorded. Each patient was characterized for the presence of AOSD-related complications such as macrophage activation syndrome (MAS), myocarditis, lung involvement (pulmonary hypertension, interstitial infiltrate), renal involvement (tubulointerstitial nephritis, acute renal failure), secondary amyloidosis and AOSD-related death. SSS was applied at the onset of the disease development, assigning a point to each of the next 12 variables: fever, exanthema, pleuritis, pneumonitis, pericarditis, alteration of liver tests or hepatomegaly, splenomegaly, lymphadenopathy, odynophagia, leukocytosis &gt;15,000/mm<sup>3</sup>, myalgia and abdominal pain. A ≥7 score has been validated on other populations as the one which identifies the patients with high risk of complications. The relationship between SSS value and the next parameters was determined: clinical course, complications, biologic treatments administered and AOSD-related mortality. <h3>Results:</h3> Data from 64 patients was analyzed (40,6% men, mean age 37 years). SSS values of &lt;7 were obtained in 50 patients (78,3%) and of ≥7 in 14 patients (21,7%). MAS and renal involvement were significantly related with a score of ≥7 (Table 1). Lung involvement, myocarditis and secondary amyloidosis were not significantly associated with a high SSS value. Even so, analyzing each case, it was found that individually they had a score of ≥7 (except for lung involvement). Biologic treatment requirement along the disease course was related to SSS ≥7. Moreover, SSS value was not related to the different clinical patterns or the not-related-AOSD deaths. It was not possible to determine the relationship between the score and the AOSD complications-related deaths due to having only two registered cases. Despite this, it was found that individually both had a SSS of ≥7. <h3>Conclusion:</h3> The prognosis score described by <i>Pouchot et al</i> could be useful to identify those patients with high risk of developing clinical complications and those who will need biologic treatment along the course of their disease. It is necessary a higher number of patients to determine if the score could be useful to estimate the death risk related to AOSD complications. <h3>References:</h3> [1] Ruscitti, et al. Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Medicine (2016)14:194 <h3>Disclosure of Interests:</h3> Ivette Casafont-Solé: None declared, Susana Holgado: None declared, J. Narváez Consultant for: Bristol-Myers Squibb, Maribel Mora: None declared, Josep Roca: None declared, Anahy Brandy-Garcia: None declared, Lourdes Mateo: None declared, Melania Martínez-Morillo: None declared, Laia Gifre: None declared, Maria Aparicio Espinar: None declared, Águeda Prior-Español: None declared, Anne Riveros: None declared, Clara Sanguesa: None declared, Jordi Camins-Fàbregas: None declared, Annika Nack: None declared, Joan Miquel Nolla: None declared, Alejandro Olive: None declared