Development of the index of idiopathic lobular panniculitis activity on the basis of the clinical and laboratory characteristics of a series of clinical observations (the authors' own data)

Idiopathic lobular panniculitis (ILP) is regarded as a clinical and pathological variant of lobular panniculitis with typical clinical symptoms. To assess the nature of the disease and to solve therapy problems, there is a need to clarify and grade disease activity, which is the basis for this investigation. Objective: to create a rating scale for the activity of the inflammatory process in ILP on the basis of clinical and laboratory parameters. Patients and methods. 67 patients (9 men and 58 women) with a verified diagno sis of ILP, who have been followed up at the V.A. Nasonova Research Institute of Rheumatology in 2007 to 2017, were examined. The patients' age was 20 to 76 years; the mean disease duration was 78.91 [48; 540] months. Physical examination, chest computed tomography, immunological and TB tests, and pathomorphological examination of the skin biopsy of the node were carried out; the serum concentrations of α1-antitrypsin, amylase, lipase, ferritin, creatine phosphokinase, and body mass index were determined. Results. Analysis of clinical manifestations could identify four ILP forms: nodular (n=30), plague (n=10), infiltrative (n=15), and mesenteric (n=12) ones that had clinical and laboratory features. Along with lesions of the skin and subcutaneous adipose tissue, 51 (76.11%) patients complained of weakness (p<0.001); 48 (71.64%) had rising body temperatures to febrile values (p=0.0001). 31 (46.26%) patients were noted to have articular syndrome that was characterized by polyarthralgia in 90.32% of cases (p<0.0001) and by knee and/or ankle arthritis in only 3 (9.67%) patients. The examinees showed a significant increase in erythrocyte sedimentation rate (p=0.01) and C-reactive protein levels (p<0.0001). Based on these findings, the authors developed an ILP activity index (AI) that included the characteristics of the status of 7 organ systems. The maximum score for individual systems is 1 to 3 in relation to the number of estimated parameters. Conclusion. The proposed ILP AI is of practical value. There is a need for further investigations and maybe a search for new parameters of ILP activity.

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