Article Text

PDF
THU0493 Approaches to Treatment of Weber-Christian Disease
  1. B. S. Belov1,
  2. O. N. Egorova1,
  3. S. G. Radenska-Lopovok1
  1. 1Institute of Rheumatology of RAMS, Moscow, Russian Federation

Abstract

Background Weber-Christian disease (WCD) is a rare understudied disease, which manifests itself in recurrent subcutaneous fatnecrosis often involving damage of the internals. Treatment of the disease is not standardized, and as a matter of fact it is provided empirically.

Objectives to assess the efficiency of therapy in case of various forms of WCD

Methods We studied 31 patients (29 women, 2 men) with WBD, age 30-74, duration of disease 8.1±2.4 years. Besides general clinical examination we determined the level of α1-antitrypsin, amylase, lipase, ferritin, creatine phosphokinase, made chest CT scan and carried out the pathomorphology study of skin biopsy specimen taken from the area of a node. The following forms of the disease were identified: nodular form (NF) (14 patients), plaque form (PF) (12 patients) and infiltrative form (IF) (5 patients). In all cases the lobular panniculitis was verified by means of histopathological examination. All patients took glucocorticoids (GC), average daily dose of 12.8 ± 1.6 mg, including 5.7± 1.4 mg (for NF), 16.6± 1.2 mg (for PF), 17.6 ± 1.7 mg (for IF). In case of NF we additionally prescribed Hydroxychloroquine, the dose of 600 mg per day (8 patients), and Azathioprine, the dose of 100 mg per day (4 patients); in case of PF – Cyclophosphamide (Cph), the dose of 200 mg per week (10 patients) or Azathioprine (150 mg per day, 2 patients). Patients with IF took Cph (200 mg per week). In case there was no effect after 3 months, we prescribed Mycophenolate Mofetil (1.5-2 g per day). On average, the observation period was 6 months.

Results The treatment made it possible to achieve a significant positive dynamics in patients with NF and PF: the body temperature got back to normal and there was regression of indurations (26 patients), joint pain intensity was reduced (14 patients), laboratory data on inflammatory intensity revealed decrease (18 patients). Average daily dose of GC was reduced to 4.5±0.7 mg and 11.4±1.1 mg, respectively. In general, a significant improvement was evident in all patients with NF and in 75 % of patients with PF. In case of IF an average daily dose was increased to 25.4±1.7 mg per day. The treatment effect was satisfactory for 2 patients of this group, although it was unsatisfactory for 3 patients. Thus, the need to consider prescribing genetically engineered biological therapy was urged. Treatment tolerance was found good, no adverse events were registered.

Conclusions To work out dosages and treatment schedules for patients with WCD further studies are required.

Disclosure of Interest None Declared

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.