title: Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases.
abstract: Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma.
authors.affiliation: Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Matsushita
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Yukiyo
authors.initials: Y
authors.lastname: Matsushita
authors.forename: Takeshi
authors.initials: T
authors.lastname: Echigo
authors.forename: Takamasa
authors.initials: T
authors.lastname: Wayaku
authors.forename: Mayuka
authors.initials: M
authors.lastname: Horikawa
authors.forename: Fumihide
authors.initials: F
authors.lastname: Ogawa
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D044466:Asian People; D001327:Autoimmune Diseases / Q000097:blood*; D053264:B-Cell Activating Factor / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008180:Lupus Erythematosus, Systemic / Q000097:blood; D008297:Male; D008875:Middle Aged; D012594:Scleroderma, Localized / Q000097:blood*; D012595:Scleroderma, Systemic / Q000097:blood; D012872:Skin Diseases, Vesiculobullous / Q000097:blood*
chemical_list: D053264:B-Cell Activating Factor