FRI0588 THE SCALE OF ACTIVITY IN IDIOPATHIC LOBULAR PANNICULITIS
Authors: Olga Egorova, Boris Belov, Svetlana Glukhova
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Idiopathic lobular panniculitis (ILP) refers to chronic systemic connective tissue lesions (M 35.6). The disease develops with typical clinical symptoms and is characterized by alternating periods of stable condition (remission) and active manifestations (exacerbations). Assessment of the activity and identification of the disadvantageous predictors is the main task in monitoring patients with ILP, however, the "gold standard" has not been developed yet. Objectives: to create a rating scale for the inflammatory process activity in ILP on the basis of clinical and laboratory parameters <h3>Methods</h3> We examined 67 patients (9 men and 58 women) with the ILP diagnosis verified in the V.A. Nasonova Research Institute of Rheumatology in 2007-2017. The age of patients ranged from 20 to 76 years, the average duration of the disease was 78.91[48;50] months. At the time of treatment at the institute only 16 patients (23,88%) were diagnosed with ILP. All the patients underwent comprehensive clinical examination and laboratory and instrumental examination of biochemical and immunological parameters, radiography or computed tomography (CT) of the chest organs, as well as pathomorphological examination of skin and subcutaneous fat biopsies from the node area. <h3>Results</h3> Analysis of clinical manifestations allowed to identify four forms of ILP: nodular (30 patients), plaque (10), infiltrative (15) and mesenteric (12), which had specific clinical and laboratory features. Based on the obtained data we developed a scale of the ILP activity (SA), which includes a description of the state of the 7 organ systems. The maximum score for individual systems is from 1 to 3 points depending on the number of estimated parameters. The total maximum possible score is 42 points. The score includes all types of damage since the onset of the disease (caused directly by ILP or developed as a result of therapy), while taking into account only signs that persist for 6 months. Score &lt;5 shows inactive disease, 5-10 - low disease activity; 11-20 - moderate activity and &gt;20 - high activity of the disease. Thus, low disease activity is characterized by the predominance in the clinical picture of limited moderately painful indurations in the absence of any significant changes in laboratory tests. At the moderate disease activity, changes in various localization with a predominance of proliferative disorders (common painful compaction of the 1st and/or 3rd stage, moderate area of damage to the trunk and limbs, subfebrile temperature, cognitive impairment, mesenteric disorders, etc.) are revealed against the background of less pronounced laboratory tests. ILP with high activity is characterized by the presence of fever and other common signs of the disease, the predominance of the 2<sup>nd</sup> stage with a pronounced VAS pain intensity of the induration, the common nature of the defeat of the pancreas, involvement of the lungs, heart and gastrointestinal tract in the pathological process. <h3>Conclusion</h3> In fact, SA is an analysis of the organ damage, assessment of the symptoms severity, and it takes into account the development of exacerbations with the involvement of new organs in the process of the disease. The proposed ILP SA is of practical importance. Further research and possibly search for new parameters of the ILP activity are needed. <h3>Disclosure of Interests</h3> None declared