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Steroid-resistant Weber-Christian Disease.

Weber-Christian Disease (WCD), relapsing febrile nodular nonsuppurative panniculitis, is characterized by recurrent subcutaneous inflammatory nodules in the adipose tissue in addition to fever, malaise and other systemic manisfestations due to widespread involvement of visceral fat. Panniculitis is the term used to describe disorders in which inflammation occurs in the subcutaneous tissue. It may be a sign of underlying systemic disease, such as systemic lupus erythematosus, scleroderma, lymphomas and leukemia, sarcoidosis, and fungal and bacterial infections. The pathogenesis of WCDremains to be clarified. It commonlyoccurs in females between the ages of twenty and forty. Subcutaneous nodules, which begin insidiously, usually appear on the thighs and legs, but frequently also on the arms and trunk. They are usually tender, and vary from 0.5 to more than 10 cm in diameter coalescing to form large indurated areas. Someof them subside spontaneously forming dimpled areas, and these atrophic deppressions are characteristic of the disease. The histologic changes can be divided into three stages; 1) focal degeneration of fat cells accompanied by inflammatory infiltrate, 2) ingestion of degenerated fat by macrophages, 3) fibrosis. In severe cases, the inflammation can involve the lungs, heart, gastrointestinal tract, spleen, kidneys, and adrenal glands. Sytemic involvement of the adipose tissue of viscera occasionally results in a poor prognosis. Major causes of death are sepsis, hepatic failure, hemorrhage, and thrombosis. No specific treatment is available for WCD.Corticosteroids have been used without satisfactory results, although they are often effective to treat acute attacks of the disease. Cyclosporin A (CyA), a cyclical hydrophobic polypeptide consisting of ll amino acids with a molecular weight of 1,202, was originally isolated from cultures of the fungus Tolypocladium inflatum grown from a soil sample collected in Southern Norway (1). CyAhas potent immunosuppressive properties that result from selective inhibition of T lymphocyte activation (2). CyA interacts with the 17 kDa intracytoplasmic receptor, termed cyclophilin, that possesses peptidylprolyl cis-trans isomerase activity. Cyclophilin-CyA complex binds with high affinity to calcineurin, the Ca2+and calmodulindependent protein phosphatase, and inhibits its phosphatase activity. Inactivated calcineurin thus cannot dephosphorylate the cytoplasmic subunit of NF-AT(NF-ATc), which in turn inhibits its translocation from the cytoplasm into the nucleus to form a competent transcriptional activator (NF-ATn). NFATninteracts with functional sequences within the enhancers of early activation genes including interleukin-2 (IL-2) and this is the primary reason why CyA inhibits T cell activation (3). Furthermore, CyAhas been shown to inhibit the function of various cell types including B cells (4) and macrophages. CyA has been successfully used to prevent graft rejection after organ transplantation (5). It has been shownto be effective to treat autoimmune diseases in animal models (6) and humanrheumatoid arthritis. CyAalso demonstrates a prominent clinical efficacy in the treatment of progressive interstitial pneumonitis associated with dermatomyositis/polymyositis (7). Usuki et al (8) has successfully treated WCDby CyA and additional reports followed since then. In this aspect, Iwasaki et al (9) reported the successful treatment of WCD with CyAwith normalization of increased serum interferon y (IFN-y) and soluble IL-2 receptor suggesting that T cell-mediated immuneresponse is involved in the pathogenesis of WCD as shownin this issue.