In 1995 Yoshida et al proposed “autoimmune pancreatitis”. Recent studies suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 related with IgG4 as the pancreatic manifestation of IgG4-related disease (IgG4-RD), and type 2 related with a granulocytic epithelial lesion. There were several diagnostic criteria. This review provides a overview of autoimmune pancreatitis from diagnostic criteria and diagnostic strategie.

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  Lupus nephritis is major manifestation of systemic lupus erythematosus and could cause nephrotic syndrome or chronic kidney disease might lead to end-stage renal failure. The pathogenesis of macrophage as well as lymphocyte impairment had been described in lupus nephritis. The interstitial macrophage accumulation and interstitial change or fibrosis is more important than glomerular immunoglobulin deposition or glomerular macrophage accumulation in terms of renal outcome and survival proportion. The expressions of macrophage associated proteins such as CCL2/MCP-1, MIP-1 family and their receptors, CCR2, CCR1 and CCR5 are major target of therapeutic strategy for improving renal illness. The blockade of these chemokines or chemokine receptors ameliorates renal impairment without reducing glomerular immunoglobulin deposition. Deletion of CSF-1 signaling pathway represented more excellent effect in experimental lupus nephritis.
  The effect of specific antagonist for macrophage associated proteins, specific thyrosine kinase inhibitor for macrophage signaling pathway on glomerulonephritis in lupus prone mice had been reported with evaluation of renal leukocyte infiltration, anti-DNA antibody reduction, the amount of proteinuria, and their survival. The depletion of macrophage could be useful therapeutic tool including M2 macrophage and have synergistic effect with other immunomodulating agents.

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  Failure of the immunotolerance mechanisms causes multiple organ-specific autoimmune disorders. Mutations of autoimmune regulator (AIRE) gene result in central immunotolerance deficiency named autoimmune polyendocrinopathy, candidiasis, ectodermal dystrophy (APECED). Mutations of FOXP3 genes cause regulatory T cell (Treg) deficiency named immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome. Because T cell tolerance influences B cell tolerance, autoantibodies seem to reflect the presence of autoreactive T cells with the same antigen specificity. To date many differences in both clinical features and autoantibody profiles have been described between APECED and IPEX syndrome. In addition to the differences in target organs, we have found differences in the target antigens in the same organ, small intestine, between both disorders; anti-autoimmune enteropathy-related 75 kDa antigen (AIE-75) antibodies are specific to IPEX syndrome, whereas anti-tryptophan hydroxylase-1 (TPH-1) antibodies are specific to APECED. These facts suggest that immunotolerance to AIE-75 depends on the Treg, whereas the tolerance to TPH-1 depends on the central mechanisms. Furthermore, given the earlier onset and more serious clinical features of IPEX syndrome than APECED, physiological roles of Aire on the selection of Treg may be, if present, limited.

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  Juvenile dermatomyositis is a complex illness characterized by vascular/perivascular inflammation, primarily in the skin and muscles. In this review, we discuss how proteomic and genomic technologies have expanded our understanding of the immune pathogenesis of this disease. We will also discuss further directions that the field may take to use existing and developing technologies to further our understanding of this often-perplexing disease.

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  Antiphospholipid antibodies (aPLs) are a group of heterogenous antibodies with immunological and functional variations that are detected in the sera of patients with antiphospholipid syndrome (APS). Detection of these antibodies in an efficient and accurate manner remains a significant issue. It requires numerous immunological and functional tests, burdening the laboratory departments, and as a consequence, not sufficiently performed in many cases. We retrospectively studied a total of 212 subjects with or without collagen diseases including APS that visited the outpatients of multiple institutions (department of internal medicine at Health Science University of Hokkaido, department of medicine II and department of gastroenterology at Hokkaido University Hospital). All the subjects were measured aPL (anticardio anticardiolipin antibody IgG/IgM, anti-β2-glycoprotein I antibody IgG/IgM) using a fully automated chemiluminescence analyzer and compared measurement results with those obtained using the conventional ELISA method. These methods were found to have similar diagnostic accuracy, with κ values exceeding 0.6. Of 61 APS patients 41 (67%) were positive for two or more tests: significantly higher than other disease such as systemic lupus erythematosus (3/37, 9%) or non-SLE collagen disease (1/53, 2%). The fully automated chemiluminescence analyzer, which can simultaneously measure multiple aPLs, was thus determined to be useful for diagnosing APS.

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  A 60-year-old Japanese man developed a fever and dry cough. Although chest CT showed interstitial pneumonia, the primary disease was not revealed. The interstitial pneumonia was improved by treatment with oral prednisolone following methylprednisolone pulse therapy. After the discontinuation of prednisolone he developed swelling of the eyelids and polyarthralgia. Orbital magnetic resonance imaging showed enlargement of the bilateral lacrimal glands. ⁶⁷Gallium scintigraphy showed abnormal uptake of the bilateral lacrimal glands and pulmonary hilum. A lacrimal gland biopsy showed noncaseating granulomatous infiltration with occasional multinucleated giant cells consistent with sarcoidosis. His symptoms improved by treatment with oral prednisolone and triamcinolone injection into the lacrimal glands.

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