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POS0422 (2021)
PANNICULITIS AS A MANIFESTATION OF AN AUTOIMMUNE/AUTOINFLAMMATORY DISEASE
O. Egorova1, A. Datsina2, A. Potapova3
1V.A. Nasonova Research Institute of Rheumatology, Laboratory for the Study of Comorbid Infections and Monitoring the Safety of Drug Therapy, Moscow, Russian Federation
2Pirogov Russian National Research Medical University, Ministry of Health of Russia, Pirogov Russian National Research Medical University, Moscow, Russian Federation
3V.A. Nasonova Research Institute of Rheumatology, V.A. Nasonova Research Institute of Rheumatology, Moscow, Russian Federation

Background: ASIA syndrome or Schonfeld syndrome is an autoimmune/inflammatory condition induced by adjuvants in genetically susceptible individuals with the development of rheumatic diseases (RD), multiple sclerosis, sarcoidosis, and others.


Objectives: to characterize the symptoms of panniculitis (Pn) associated with ASIA-syndrome


Methods: Within 7 years, ASIA syndrome was diagnosed in 12 women, average age 37.2±7.4, with a referral diagnosis of “Erythema Nodosum” or “Panniculitis”. The duration of the disease was 16.7±3.9 months. In addition to general clinical study, the serum concentration of α-1 antitrypsin, amylase, lipase, ferritin, creatine phosphokinase (CPK), immunological parameters (ANP-Hep2, dsDNA, C3 and C4, CRP, ANCA, Scl-70, antibodies to cardiolipins G and M), computed tomography of the chest organs, pathomorphological and immunohistochemical examination of a biopsy specimen of skin with subcutaneous fat tissue (SFA) from the affected areas.


Results: The development of ASIA syndrome was preceded by gluteoplasty using propantriol-1,2,3 (in 4 patients), liposuction (2), biorevitalization with hyaluronic acid (2) and endoprosthetics with breast implants (2), bone metal construction (1) and mesh hernioplasty (1). The clinical picture of Pn was characterized by generalized red-purple painful (VAS pain 55.8±17.3 mm) subcutaneous indurations on the upper limbs and trunk (in 100% of cases), face (16.6%) and lower limbs (33.3%), with ulceration and oily fluid leakage (58.6%). The saucer symptom was recorded in 83.3% of the observed patients. In 66.6% of cases, fever and articular syndrome were observed, in 41.6% – myasthenic syndrome and lymphadenopathy, in 8.3% – xerophthalmia, keratoconjunctivitis dry, xerostomia and recurrent parotitis. In blood tests, leukopenia (up to 2.0x109/l) was identified in 33.3% of patients, a significant increase in the level of creatinine phosphakinase – in 16,6%, a two or more times increase in ESR and CRP – in 100%. All patients demonstrated immunological changes. The morphological picture of the skin and SFA in 75% of cases resembled lobular Pn, in 8.3% – tumor lymphocytes with the immunophenotype of cytotoxic T-lymphocytes: CD3+, CD8+ were detected. The results obtained allowed confirming RD in 8 patients: systemic lupus erythematosus and idiopathic lobular panniculitis in 2 patients (respectively) and 1 in dermatomyositis, systemic scleroderma of Sjogren’s disease, mixed connective tissue disease. One case was diagnosed with sarcoidosis and T-cell lymphoma. In 2 patients after the liposuction procedure the course of Pn was reversible.


Conclusion: Diagnosis of Pn associated with ASIA syndrome is a complex task that requires a multidisciplinary approach to verify the diagnosis and treatment tactics.


Disclosure of Interests: None declared


Citation: Ann Rheum Dis, volume 80, supplement 1, year 2021, page 440
Session: Systemic sclerosis, myositis and related syndromes - aetiology, pathogenesis and animal models (POSTERS only)