AB1153 Idiopatic lobular panniculitis (DISEASE WEBER-CHRISTIAN): current aspects
Authors: O.N. Egorova, B.S. Belov, S.I. Glukhova, S.G. Radenska-Lopovok
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Idiopatic lobular panniculitis (ILP) (syn. Weber-Christian disease) is the least studied condition from the group of systemic connective tissue diseases, which is characterised by predominant involvement of subcutaneous fat (SCF) tissue. <h3>Objectives</h3> To evaluate the interrelation between clinical signs and lab parameters in ILP patients. <h3>Methods</h3> The study evaluated 67 patients (9 males and 58 females) aged 20–76 years with verified ILP diagnosis and mean disease duration of 78.91<sup>48;540</sup> months who were on the record at V. A. Nasonova Research Institute of Rheumatology during 2007–2017 yy. Alfa-1 antitrypsin, liver fractions, amylase, lipase, trypsin, ferritine, creatine phosphokinase (CPK), leptin and TNF- α levels were measured, chest CT and histopathological study of skin and SCF lesions biopsy specimens were made in addition to conventional clinical examination. <h3>Results</h3> ILP was found to affect all age groups, with 57% of cases falling on able-bodied adults aged 45–60 yy. Based on clinical manifestations including location, distribution, spatial extent of lesions, and clinical course of the disease, the following 4 clinical forms of ILP were identified: nodular (30 patients), plaque-like,<sup>10</sup> infiltrative<sup>15</sup> and mesenteric.<sup>12</sup> ILP population demonstrated significant ESR (<i>p</i>=0,01) and CRP (<i>p</i>&lt;0,0001) elevation. ESR elevation correlated with palpatory nodular pain intensity, assessed by visual analogue scale (VAS (<i>p</i>&lt;0,05, r=0,29), with the amount of affected body surface area (BSA) measured using the hand area surface (HAS) to equal 1% BSA (<i>p</i>&lt;0,05, r=0,50), with elevation of body temperature (<i>p</i>&lt;0,05, r=0,68) and CRP (<i>p</i>&lt;0,05, r=0,68). CRP elevation correlated with pain intensity measured by VAS (<i>p</i>&lt;0,05, r=0,46), affected BSA (<i>p</i>&lt;0,05, r=0,61) and presence of stage II nodules (<i>p</i>&lt;0,05, r=0,41). Histopathologic features of skin and SCF biopsy specimens were studied in 65 patients (97,01%), including antero – and retroperitoneal fat tissue biopsy specimens from 3 patients out of 5 (5,97%) with mesenteric panniculitis without skin and SCF involvement; the remaining 2 were not biopsied in hard to access areas. Histopathological study ruled out the probability of neoplasms and confirmed the diagnosis of lobular panniculitis in all specimens. Therapy included such common in rheumatology practice agents as glucocorticosteroids, NSAIDs, cytotoxic drugs, hydroxychloroquine, and oths). Therapeutic success was documented in 62,68% cases, therapeutic failure and disease progression – in 17,91% (12 patients), requiring dose escalation and modification of therapeutic regimen. <h3>Conclusions</h3> Identified correlation between clinical features and lab parameters measuring disease activity confirms ILP as a systemic inflammatory disease of the connective tissue. There's a flagrant necessity to improve physicians awareness of ILP, as well as need in future studies to enable earlier ILP diagnosis and identify more effective treatment of the disease. <h3>Disclosure of Interest</h3> None declared