title: Interleukin 1 components in cicatricial pemphigoid. Role in intravenous immunoglobulin therapy.
abstract: Interleukin (IL-)1 is an important mediator of inflammatory responses and plays an important role in the pathogenesis of various autoimmune diseases. Cicatricial pemphigoid (CP) is a multisystem autoimmune inflammatory disease. We have studied the role of IL-1 in its pathogenesis. We have investigated the serum levels of IL-1 components (IL-1alpha, IL-1beta, and IL-1Ra), and determined the role of intravenous immunoglobulin (IVIg) therapy in patients with CP. Serum levels of IL-1alpha and beta were significantly higher in untreated patients with active disease compared to levels in patients in prolonged clinical remission and normal human controls (P<0.0001). The serum levels of IL-1Ra were higher in patients in prolonged clinical remission compared to patients with active disease (P=0.002). Hence elevated levels of IL-1alpha and beta and low levels of IL-1Ra correlate with disease activity. The levels of IL-1alpha and beta were statistically significantly higher in sera of CP patients with active disease pre-IVIg therapy compared to post-IVIg therapy (P<0.0001). Statistically significantly higher levels of IL-1Ra were present in post-IVIg treatment serum samples when compared to levels in pre-IVIg treatment (P<0.0001). In the in vitro experiments, the levels of IL-1alpha and beta produced by the peripheral blood mononuclear cells (PBMC) isolated from patients before IVIg therapy were significantly higher when compared to the PBMC isolated from post-IVIg patients (P<0.0001). Significantly higher levels of IL-1Ra were observed in the supernatants of PBMC collected from pre-IVIg patients and cultured with exogenously added IVIg, when compared to the levels of PBMC to which IVIg was not added (P<0.0001). IL-1 may be an important cytokine involved in the pathogenesis of CP. The regulation of IL-1 could be one of the mechanisms, amongst others, by which IVIg may exert its beneficial effect in the treatment of CP.
authors.affiliation: Department of Oral Medicine and Diagnostic Sciences, Harvard School of Dental Medicine, New England Baptist Hospital, Boston, MA 02115, USA.
authors.forename: S
authors.initials: S
authors.lastname: Kumari
authors.forename: K C
authors.initials: KC
authors.lastname: Bhol
authors.forename: F
authors.initials: F
authors.lastname: Rehman
authors.forename: C S
authors.initials: CS
authors.lastname: Foster
authors.forename: A R
authors.initials: AR
authors.lastname: Ahmed
mesh_terms: D002478:Cells, Cultured; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1 / Q000096:biosynthesis / Q000097:blood / Q000502:physiology*; D007963:Leukocytes, Mononuclear / Q000276:immunology / Q000378:metabolism; D010390:Pemphigoid, Benign Mucous Membrane / Q000097:blood / Q000276:immunology* / Q000628:therapy*; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors; D012795:Sialoglycoproteins / Q000096:biosynthesis / Q000097:blood
chemical_list: C507763:IL1RN protein, human; D016756:Immunoglobulins, Intravenous; D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1; D017472:Receptors, Interleukin-1; D012795:Sialoglycoproteins