The Japan Society for Clinical Immunology Vol. 33, No. 1, p.31
Wrong: Daisuke WAKAURA
Right: Daisuke WAKURA

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  In this review, I summarized recent studies about nonatopic dermatitis, IgE associated atopic dermatitis and autoallergic atopic dermatitis in the natural history of atopic dermatitis with gene -gene and gene -environment interaction.
  Also, new hypothesis of the mechanism of atopic dermatitis which was called OUTSIDE-INSIDE-OUTSIDE story wes introduced.

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  Psoriasis is a chronic inflammatory skin disease, which is characterized by red, scaly, raised plaques. Its symptoms may range in severity and there are severe clinical subtypes of psoriasis including pustular psoriasis. Pre-biological systemic therapies were targeting mainly keratinocyte proliferation and T cell activation. However, there were cases, in which these classic therapies were not fully effective or could not be used because of the accompanied severe organ failure of the psoriasis patient. Recent progress in psoriasis research has clarified which of the cells, cell surface molecules and cytokines play a pivotal role in the pathogenesis of psoriasis. Biological systemic therapies targeting these molecules have turned out to be extremely effective in treating psoriasis and are now used to treat moderate to severe psoriasis worldwide. These biological therapies are not only more effective than pre-biological systemic therapies, but also show more rapid response, and long-term efficacy is expected. The TNF-α inhibitors are now approved as the first biological therapy for psoriasis in Japan. These TNF-α inhibitors are expected to be a new standard therapy for severe psoriasis, however, since the long-term safety is an unsolved issue, careful clinical monitoring is required. (193 words)

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  To predict the response of patients with rheumatoid arthritis (RA) to infliximab, patient profile and laboratory findings were compared to determine whether there was any association with the clinical course of the disease, and the clinical significance of serum rheumatoid factor (RF) in the response to this treatment was considered. Sixty-two RA patients were treated with infliximab, 87.9% of whom were positive for RF. At baseline and 12 months after the start of treatment, RF titers were significantly lower in the low-CRP group (CRP at 12 months<0.3 mg/dl) compared with that in the high-CRP group (CRP at 12 months >1.5 mg/dl). Furthermore, at baseline and 12 months, RF titers were significantly lower in the good-CRP-response group (ΔCRP≥1.5 mg/d) compared with the poor-CRP-response group (ΔCRP≤0.3 mg/d). The percent changes in RF and CRP from baseline were correlated throughout the clinical course. The duration of disease in good-CRP-response group was shorter than that in poor-CRP-response group significantly. The rate of positivity for HLA-DR4 in good response group tends to be higher than that in poor-CRP-response group. We concluded that several factors, including disease duration, positivity for HLA-DR4, and the serum RF titer at baseline, might predict the patient response to infliximab.

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  A 30-year-old pregnant woman experienced mild dyspnea in April 2009. She complained of mild myalgia and was subsequently admitted to our hospital in June 2009 because of worsening dyspnea. Physical examination revealed fine crackles in the lower lung field, but no eruptions externally. Laboratory findings revealed elevated serum levels of myogenic enzymes (aldolase, 17.6 IU/l and myoglobin, 247.2 ng/ml) and positive titers for the anti-Jo-1 antibody and hypoxia (PaO₂, 79.4 Torr). The chest radiograph revealed a ground-glass opacity. The patient was diagnosed as interstitial pneumonia (IP) associated with polymyositis (PM) at 20 weeks of gestation. On July 9, we commenced the initial treatment-steroid pulse therapy with 60 mg/day of prednisolone and 3 mg/day of tacrolimus. We also induced abortion. The treatment of corticosteroids and tacrolimus was, however, ineffective even after increasing the tacrolimus dose to 6 mg/day. On July 30, she suddenly experienced chest pain along with severe dyspnea. Computed tomography revealed the presence of pneumomediastinum and deterioration of the IP. We added cyclophosphamide pulse therapy to the existing regimen ; this improved the disease course, reduced hypoxia, and improved radiographic findings. We believe that this is a rare case of IP with PM during pregnancy.

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  A 25-year-old-man was referred with an approximately 1-year history of recurrent fever, tonsillitis, testicular pain and testicular swelling in June 2009. He also complained of visual disturbance, some erythema nodosum (EN) like lesions on his lower extremities and oral aphthous ulcerations for 5 months. Opthalmological consultation confirmed retinochoroiditis, and ultrasonography revealed epididymitis. A biopsy of the EN like lesion showed a necrotizing vasculitis of the small and medium-sized vessels with septal panniculitis in the subcutis. The laboratory findings revealed an elevation of CRP and positive HLA-B51. He was diagnosed with incomplete Behcet's disease according to the Japanese criteria (1987). After the initiation of the therapy with 10 mg/day of prednisolone, his symptoms promptly ameliorated. In our case, although epididymitis as well as the histopathological findings suggested polyarteritis nodosa (PN), a diagnosis of BD was established on the basis of other contemporaneous findings. Necrotizing vasculitis as a cutaneous manifestation in patients with BD has been rarely reported. However, BD and PN, including cutaneous PN, have rarely described in conjunction. Therefore, we propose that PN-like necrotizing vasculitis might be the subtype of BD.

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  We report an asymptomatic mother of a cutaneous neonatal lupus child was diagnosed with Sjögren's syndrome suspected after parturition. A 30-year old woman was visited our hospital to evaluated autoimmune disease because her baby was diagnosed as cutaneous neonatal lupus. Both gum test and Schirmer's test were positive. Antinuclear antibody, anti-SS-A and anti-SS-B antibody were positive. Serum IgG was elevated (2918 mg/dl). Finally, this case was suspected as Sjögren's syndrome. Continuoues follow-up of asymptomatic mothers of a neonatal lupus child is warranted.

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  Behçet's disease (BD) is a multisystem immune-mediated inflammatory disorder. The choice of treatments depends on organ involvements and its severity. The treatments for pediatric patients usually consist of corticosteroids and immunosuppressants, but some of them are refractory to these treatments. Recently, tumor necrosis factor alpha (TNF-α) appears to play an important role in the immunopathogenesis in BD. We describe two patients with childhood-onset BD, successfully treated by anti-TNF agents. One patient, a 18-year-old male, was diagnosed as having BD at 8 years of age. He developed abdominal pain and bloody diarrhea at 17 years of age. Gastrointestinal endoscopic examination revealed esophageal and ileocolic ulceration consistent with intestinal BD. Methylpredonisolone pulse therapy and immunosuppressants were administrated, but did not respond. Infliximab infusions (4 mg/kg) were given at weeks of 0, 2, 6, and then every 8 weeks. A dramatic improvement in gastrointestinal symptoms and extraintestinal manifestations were observed. Another patient, a 9-year-old girl, developed recurrent oral ulcers and folliculitis at 4 years of age. She was diagnosed as having BD at 6 years of age, and treated with colchicines, corticosteroids and immunosuppressive agents. She was suffering from fever, malaise, and arthralgia in addition to mucocutaneous manifestation. At 8 years of age, etanercept was started at a dosage of 25 mg subcutaneously twice a week. A resolution of the systemic and mucocutaneous symptoms was achieved. Furthermore, she was free from corticosteroids in three months. Anti-TNF therapy would be a good choice for juvenile BD refractory to conventional treatments.

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  Pneumothorax is a rare pleuropulmonary manifestation of systemic lupus erythematosus. We encountered a 37-year-old Japanese woman who had systemic lupus erythematosus complicated by recurrent pneumothorax during treatment for recurrent serositis with glucocorticoid therapy. She was admitted for the third episode of lupus peritonitis in December 2005. Intravenous cyclophoshamide and increased dose of oral prednisolone were administered. In early January 2006, hemoptysis was observed and bronchofiberscopy revealed hemorrhage from the left lower lobe. After intravenous methylprednisolone pulse therapy and oral cyclosporine therapy were added, pleurisy and pulmonary hemorrhage improved. On February 22nd, she suddenly developed pneumothorax on the right side, followed by pneumothorax on the left side after 2 days. This pneumothorax on the left side did not improve despite chest tube drainage for over one month. She underwent thoracoscopic partial lobectomy of lower lobe of the left lung, and her symptoms improved.
  Review of the literature identified 10 case reports of systemic lupus erythematosus complicated by pneumothorax. All of the patients including our case had underlying pulmonary lesions, and 9/11 patients had pleurisy. Besides 10/11 patients received glucocorticoid therapy before the occurrence of pneumothorax. Tissue fragility caused by these factors might contribute to the complication of pneumothorax in patients with systemic lupus erythematosus.

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