Relapsing Polychondritis (RP) is an uncommon, chronic, and potentially life-threatening multisystem disorder characterized by recurrent inflammatory episodes affecting the cartilaginous tissues of the external ears, nose, peripheral joints, larynx and tracheobronchial tree, sometimes leading to their destruction. RP can also inflame other proteoglycan-rich structures, such as the eye, heart, blood vessels and inner ears. Systemic symptoms are common, and vasculitis affecting skin or internal organs may occur. The etiology of RP is still unknown, but the pathogenetic role of the autoimmunity is suggested by frequent overlaps with various autoimmune diseases, and by the presence of autoantibody against cartilage in the serum of patients with RP. Although several reports have demonstrated the clinicopathologic manifestations and radiologic findings of RP, there are no specific features of RP. Therefore, it is difficult to show the diagnosis of RP. Airway involvements are major causes of morbidity and mortality, and they have accounted for most of the deaths due to RP. To suppress the inflammation of airway mucosa and cartilage is extremely important in the successful treatment for RP. Above all, earlier diagnosis would lead to better outcomes.

View full abstract

  IL-22 is an IL-10 family cytokine that acts mainly on epithelial cells. It is produced by immune cell subsets, including CD4⁺ T cells, natural killer cells, and natural killer T cells. In the skin, IL-22 mediates keratinocyte proliferation and epidermal hyperplasia, inhibits terminal differentiation of keratinocytes, and induces the production of antimicrobial proteins. Although IL-22 production was initially linked with IL-17 expression in Th17 cells, IL-22 production can also occur in an apparently unique subset of cells that lacks the production of IL-17 and IFN-γ (Th22). Interestingly, Th22 cells express skin homing chemokine receptors CCR4 and CCR10. Indeed, Th22 cells reside in the normal skin and are shown to be enriched in the lesional skin of inflammatory skin diseases, indicating the importance of IL-22 in skin homeostasis and pathogenesis of skin diseases. Although psoriasis is the first example of an organ-specific immune disorder for which the role of IL-22 has been comprehensively studied, a growing body of evidence indicates that this cytokine also plays a critical role in the pathogenesis of atopic dermatitis. In this review, we discuss the role of IL-22 in the pathogenesis of skin diseases, particularly focusing on psoriasis and atopic dermatitis. Targeting IL-22 may have promise as a potential therapeutic for various skin diseases.

View full abstract

  In the past 40 years, prognosis for patients with systemic lupus erythematosusu(SLE) has improved, and 10-year survival now becomes approximately over 95%. This may be due probably to a combination of earlier disease diagnosis and diagnosis of milder disease, due in part to availability of multiple serological tests for SLE, use of steroids and other immunosuppressive agents, and availability of renal dialysis and transplantation. Although the prognosis of SLE itself improved a lot, still the potential for significant morbidity and mortality remains in the group of patients with partially responsive or treatment resistant disease such as type IV lupus nephritis and CNS involvement, etc. To overcome these problems, various therapeutic approaches have been newly developed based on the understanding of molecular mechanisms involved in the pathogenesis of SLE. In this paper, usefulness of treatments targeting molecules that are strongly associated with immune disorders in lupus patients will be discussed.

View full abstract

  Epidemic studies have shown that rheumatoid arthritis (RA) patients have shorter life expectancy than healthy persons, primarily due to cardiovascular events. The aim of our study was to explore inhibitory effects on atherosclerosis of RA patients by TNF-inhibitor etanercept (ETN). We studied six RA patients with moderate or high disease activity as defined by the European League Against Rheumatism (EULAR) disease activity score (DAS28-ESR) in spite of treatment with methotrexate (MTX) 8 mg/week. We measured their pulse wave velocity (PWV) before and after treatment with ETN 25 mg/week for one year. There were no additional medications other than ETN that might influence on atherosclerosis. Their PWV decreased in five of six patients and the average decreased from 1474.8 cm/sec to 1432.5 cm/sec. The average of DAS28-ESR score was significantly decreased from 5.56 to 2.87 and they achieved good response by the EULAR criteria. There were no significant changes in the blood pressure, serum lipid (total cholesterol and triglyceride) and HbA1c ; all values were within normal limits before and after ETN treatment. It is suggested that the improvement of PWV is due to anti-inflammatory effects, leading inhibition of atherosclerosis, of ETN in RA patients.

View full abstract

  A 59-year old woman had been suffering from myalgia, eruption and dyspnea on effort for a month. She was referred to our hospital because her symptoms were not improved by antibiotic therapy at a clinic. At first presentation she showed cutaneous manifestations including heliotrope eyelids and mechanic's hands, slightly elevated serum creatine kinase (CK), elevated serum C-reactive protein level, and interstitial pneumonia (IP), which led to a diagnosis as dermatomyositis. After admission to our hospital, her hypoxia due to IP progressed despite disappeared myalgia and normalized serum CK level. Intravenous steroid pulse therapy followed by oral cyclosporine and intravenous cyclophosphamide pulse therapy was not effective for the IP for more than a month. Gastroscopy revealed superficial depressed (0-IIc) type early gastric cancer. However, it was out of indication for endoscopic mucosal resection because of the histopathologic type (signet ring cell carcinoma) and possible submucosal invasion. In addition to those immunosuppressive therapies, proximal gastrectomy with total intravenous anesthesia was performed. One month after the operation, improvement of IP as well as cutaneous manifestations was confirmed by respiratory function test, pulmonary computed tomography, and discontinuance of oxygen inhalation. We conclude that this was a case of clinically amyopathic dermatomyositis with rapidly progressive IP and gastric cancer, which was ameliorated by a combination of medication (steroid, cyclosporine, and cyclophosphamide) and surgery (gastrectomy for early gastric cancer).

View full abstract

  A 62-year-old woman presented to a primary care doctor on January 2006 due to a sore throat and high fever, and had received medication for a common cold. She was referred to our hospital in February 2006 because of additional manifestations such as painful rashes on the lower limb similar to erythema nodosum and polyarthralgia on her feet, shoulder and finger joints. She was initially treated with an anti-inflammatory drug (NSAID) for polyarthritis but the symptoms did not improved. In addition, the serum level of anti-streptolysin O antibody (ASO) was elevated at the second visit more than that at the first visit. She was diagnosed to have rheumatic fever (RF) based on the polyarthritis, inflammatory data and an increase of the ASO level. She was treated with 10 mg a day of prednisolone (PSL) and sultamicillin tosilate. However, a systolic murmur that had been never noticed by previous auscultation appeared after the third hospital day and the mitral regurgitation was also detected on echocardiogram. She was then treated with 40 mg a day of PSL because of an appearance of the carditis due to RF. The increased PSL dose promptly improved the systolic murmur as well as the arthritis. This report presented an RF case with carditis detected by an development of the systolic murmur in an adult female.

View full abstract

  We herein report the case of a female with Sjogren's syndrome (SS) complicated with trigeminal nerve palsy. Although her sicca symptoms had been unnoticed, head magnetic resonance imaging (MRI) for detecting brain abnormalities revealed parotid gland changes associated with SS. SS should be considerd as a possible cause of trigeminal nerve disturbances. In addition, parotid gland changes related to SS should be aware in examination of cranial nerve disturbances with MRI.

View full abstract