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Competing Interests: Competing interests: None declared.

In three patients with immunoglobulin G4-related dacryoadenitis and sialoadenitis (IgG4-DS), we assessed the changes in the hardness of affected submandibular glands by the real-time tissue elastography at baseline and 2 weeks after the initiation of treatment with oral glucocorticoids. Elastography revealed "blue" meaning to be a hard tissue matched the hypoechoic areas inside the enlarged submandibular glands before treatment. The stiff elastographic changes rapidly improved after 2 weeks. This may relate with the reversibility of salivary function by glucocorticoid treatment in patients with IgG4-DS.

Sporothrix brasiliensis is the main species of the S. schenckii complex implicated in the zoonotic epidemics of sporotrichosis in Rio de Janeiro, Brazil. Epidemiological features have been already described, such as zoonotic transmission by cats and increased frequency of atypical clinical aspects. The involvement of the face by contact with cats is common in childhood; as a result, ophthalmic manifestations have increased. We report a case of acute dacryocystitis in a 9-year-old girl. A calmodulin-based molecular phylogeny was used to identify the agent as S. brasiliensis. This is a rare type of presentation, usually complicated with nasolacrimal duct occlusion. The patient was cured without sequelae after treatment with a low dose of saturated solution of potassium iodide and decompressive oculoplastic surgery. Therapeutic options and considerations of aetiological agents and serology are discussed., (© 2014 British Association of Dermatologists.)

Abstract Objective. Immunoglobulin (Ig)G4-related disease (IgG4-RD) is a new disease entity that has only been identified this century. Clinical information is thus lacking. We established the Sapporo Medical University and Related Institutes Database for Investigation and Best Treatments of IgG4-related Disease (SMART) to clarify the clinical features of IgG4-RD and provide useful information for clinicians. Methods. Participants comprised 122 patients with IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), representing lacrimal and/or salivary lesions of IgG4-RD, followed-up in December 2013. We analyzed the sex ratio, mean age at onset, organ dysfunction, history or complications of malignancy, treatments, rate of clinical remission, and relapse. Results. The sex ratio was roughly equal. Mean age at diagnosis was 59.0 years. Positron emission tomography revealed that the ratio of other organ involvements was 61.4%. Complications of malignancy were observed in 7.4% of cases. Glucocorticoid was used to treat 92.1% of cases, and the mean maintenance dose of prednisolone was 4.8 mg/day. Rituximab was added in three cases, and showed good steroid-sparing effect. The clinical remission rate was 73.8%, and the annual relapse rate was 11.5%. Half of the cases experienced relapses within 7 years of initial treatment. Conclusion. We analyzed the clinical features and treatments of IgG4-DS using SMART, providing useful information for everyday clinical practice.

Regulation of the adhesion of mononuclear cells to endothelial cells is considered to be a critical step for the treatment of inflammatory diseases, including autoimmune diseases. K-13182 was identified as a novel inhibitor for these adhesions. K-13182 inhibited the expression of vascular cell adhesion molecule-1 (VCAM-1, CD106) on human umbilical vein endothelial cells (HUVECs) and on mouse vascular endothelial cell line (MAECs) induced by tumour necrosis factor (TNF)-alpha. K-13182 also inhibited the adhesion of mononuclear cells to these HUVECs and MAECs, indicating that K-13182 suppressed these adhesions mediated by cellular adhesion molecules including VCAM-1. To evaluate the therapeutic effect in autoimmune disease model mice, K-13182 was orally administered to non-obese diabetic (NOD) mice as Sjögren's syndrome (SS) model mice. Severe destructive inflammatory lesions were observed in the lacrimal glands of vehicle-treated control mice; however, 8-week administration of K-13182 inhibited the mononuclear cell infiltration into the inflammatory lesions of the lacrimal glands. In K-13182-treated mice, the decrease in tear secretion was also prevented compared to the control mice. In addition, the apoptosis and the expression of FasL (CD178), perforin, and granzyme A was suppressed in the lacrimal glands of K-13182-treated mice. Therefore, K-13182 demonstrated the possibility of therapeutic efficacy for the inflammatory region of autoimmune disease model mice. These data reveal that VCAM-1 is a promising target molecule for the treatment of autoimmune diseases as a therapeutic strategy and that K-13182 has the potential as a new anti-inflammatory drug for SS.