SUMMARY The frequency of either CD4–8– (double negative; DN) or CD4+ Vα24+Vβ11+ NKT cells, the expression of CD1d and the binding of CD1d‐tetramer loaded with α‐galactosylceramide (α‐GalCer) to NKT cells were analysed in peripheral blood mononuclear cells (PBMCs) of patients with Wegener's granulomatosis (WG), relapsing polychondritis (RP) and healthy subjects (HS). DN and CD4+ Vα24+Vβ11+ NKT cells as well as CD1d‐α‐GalCer tetramer‐positive NKT cells, were significantly decreased in number in both WG and RP patients compared to those from HS. When cytokine profiles were analysed in these PBMCs upon stimulation with phorbol ester and calcium ionophore, CD4+ T cells from patients with WG and RP exhibited a Th1 bias, whereas CD4+ NKT cells from WG patients in remission showed a Th2 bias. These findings suggest that NKT cells (especially CD4+ NKT cells) play a regulatory role in Th1 autoimmunity in patients with WG and RP. The reduction in NKT cell counts appears to be associated with the low responsiveness to α‐GalCer. The dysfunction of NKT cells to recognize ligands such as α‐GalCer may also contribute to the defects observed in NKT cells from WG and RP patients.

Novel biological activities of macrophage migration inhibitory factor (MIF) have been rediscovered. In addition, elevation of the serum MIF level has been reported in different types of disorders, including various inflammatory and autoimmune diseases. In the present study, serum MIF levels were analyzed in patients with Wegener's granulomatosis (WG) and relapsing polychondritis. It was shown that the serum MIF levels in these patients were significantly higher than those of normal healthy controls. In a WG patient, the MIF level showed a good correlation with clinical symptoms and C-ANCA titers. Thus, serum MIF levels will be a useful laboratory parameter for following the clinical course of WG patients and determining medical treatment. The immunopathologic roles of MIF in these diseases are discussed.

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Angiofibroma arising outside the nasopharynx is unusual. The nasal septum is an extremely rare site for an angiofibroma to originate, and, to date, only 3 cases have been reported in the literature. We reported here the fourth case of nasal septal angiofibroma. A 27-year-old male patient with nasal deformity applied to the Erciyes University ENT Department. He also complained of nasal obstruction on the right for 6 years. Two years ago, he had experienced an epistaxis, which oozed for 2 weeks after being packed. Neither the patient nor his family had a history of any hematologic disorder. On examination, an external nasal deviation to left was remarkable. A dark grayish mass was seen through the right nostril, between the nasal septum and the inferior turbinate on anterior rhinoscopy. There was no mass in the nasopharynx on posterior rhinoscopy via endoscope.