Publications by authors named "Tamihiro Kawakami"

141 Publications

Current medico-psycho-social conditions of patients with West syndrome in Japan.

Epileptic Disord 2021 Aug;23(4):579-589

Department of Pediatrics, School of Medicine, Shinshu University, Matsumoto, Japan.

Objective: To unveil current medical and psychosocial conditions of patients with West syndrome in Japan.

Methods: A cross-sectional analysis was performed in patients with West syndrome registered in the Rare Epilepsy Syndrome Registry (RES-R) of Japan. Furthermore, new-onset patients registered in the RES-R were observed prospectively and their outcomes after one and two years of follow-up were compared with data at onset.

Results: For the cross-sectional study, 303 patients with West syndrome were included. Seizures (such as spasms, tonic seizures and focal seizures) occurred daily in 69.3% of the patients at registration. Seizure frequency of less than one per year was observed in cases of unknown etiology (22.6%), genetic etiology (23.8%) and malformation of cortical development (MCD; 19.1%). Neurological findings were absent in 37.0%, but a high rate of abnormality was seen in patients with Aicardi syndrome, hypoxic-ischemic encephalopathy (HIE), genetic etiology and MCD other than focal cortical dysplasia, accompanied by a >50% rate of bedridden patients. Abnormal EEG was found in 96.7%, and CT/MRI was abnormal in 62.7%. Treatments included antiepileptic drug therapy (94.3%), hormonal therapy (72.6%), diet therapy (8.3%) and surgery (15.8%). Intellectual/developmental delay was present in 88.4%, and was more severe in patients with Aicardi syndrome, genetic etiology and HIE. Autism spectrum disorder was found in 13.5%. For the longitudinal study, 27 new-onset West syndrome patients were included. The follow-up study revealed improved seizure status after two years in 66.7%, but worsened developmental status in 55.6%, with overall improvement in 51.9%.

Significance: The study reveals the challenging neurological, physical and developmental aspects, as well as intractable seizures, in patients with West syndrome. More than a half of the children showed developmental delay after onset, even though seizures were reduced during the course of the disease.
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http://dx.doi.org/10.1684/epd.2021.1301DOI Listing
August 2021

The relationship between anti-phosphatidylserine/prothrombin complex IgM antibodies and cutaneous ulcers in patients with cutaneous vasculitis.

J Dermatol 2021 Sep 25;48(9):1457-1458. Epub 2021 Jun 25.

Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

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http://dx.doi.org/10.1111/1346-8138.16014DOI Listing
September 2021

Anti-phosphatidylserine/prothrombin complex antibodies in patients with cutaneous vasculitis: Possible involvement in the pathogenesis.

J Dermatol 2021 May 18;48(5):703-706. Epub 2021 Feb 18.

Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Japan.

We assessed the IgG and IgM prevalence of anti-phosphatidylserine/prothrombin complex (aPS/PT) antibodies (Abs) in patients with vasculitis using a novel commercial ELISA kit. To examine whether aPS/PT Abs were involved in the pathogenesis of cutaneous vasculitis, inbred wild-type rats were intravenously administered with a rat IgM class aPS/PT monoclonal Ab established previously or with rat immunoglobulins as controls. To express PS on the surface of vascular endothelium, these rats were given a subcutaneous injection of cell-free histones in advance. Serum IgM aPS/PT Ab levels were elevated in patients with systemic vasculitis with skin involvement and cutaneous arteritis compared to those in patients with systemic vasculitis without skin involvement and healthy controls. There was no significant difference in the serum levels of IgG aPS/PT Abs between the patients and healthy controls. Correspondingly, inbred wild-type rats intravenously administered with the aPS/PT monoclonal IgM Ab after appropriate priming-subcutaneous histone injection developed cutaneous vasculitis. Some rats given rat IgM instead of the aPS/PT monoclonal Ab also developed cutaneous vasculitis, whereas vasculitis did not occur in rats given IgG or only priming by histones. We suggested that IgM aPS/PT Abs could be involved in the pathogenesis of cutaneous vasculitis based on these findings.
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http://dx.doi.org/10.1111/1346-8138.15810DOI Listing
May 2021

Pityriasis lichenoides et varioliformis acuta associated with anti-Ku-positive refractory interstitial lung disease and dermatomyositis.

J Dermatol 2020 Nov 17;47(11):e403-e404. Epub 2020 Aug 17.

Divisions of, Division of, Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

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http://dx.doi.org/10.1111/1346-8138.15556DOI Listing
November 2020

Early add-on administration of mepolizumab and intravenous immunoglobulin effective in treating eosinophilic granulomatosis with polyangiitis.

J Dermatol 2021 Apr 10;48(4):529-532. Epub 2020 Dec 10.

Division of Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

Treatment of eosinophilic granulomatosis with polyangiitis (EGPA) remains a challenge because currently available therapies, corticosteroids and immunomodulators, do not always control symptoms and are often associated with significant morbidity and relapse. Mepolizumab has been demonstrated to be an effective add-on therapy with steroid-sparing effect in cases of relapsing or refractory EGPA. Intravenous immunoglobulin (IVIG) therapy is effective against mononeuritis multiplex or heart failure in patients with EGPA who do not respond to corticosteroid-cyclophosphamide treatment. We present two cases of EGPA in which earlier add-on administration of adjunct mepolizumab and IVIG led to significant improvement in EGPA symptoms and prevention of flare-up of the disease. We suggested that earlier add-on combination administration of IVIG and mepolizumab might be a useful adjunct treatment to induce clinical remission of EGPA and improve the rate of remission, decrease relapse rate, and allow for reduced glucocorticoid use without any serious adverse drug effects.
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http://dx.doi.org/10.1111/1346-8138.15709DOI Listing
April 2021

Clinical practice guide for the treatment of perforating dermatosis.

J Dermatol 2020 Dec 23;47(12):1374-1382. Epub 2020 Oct 23.

Department of Dermatology, Juntendo University Urayasu Hospital, Urayasu, Japan.

Perforating dermatoses are a heterogeneous skin disease group defined by transepidermal elimination of various skin materials. Four classical forms of primary perforating dermatosis have been described, where the transepidermal elimination mechanism represents the hallmark of the disease: acquired reactive perforating collagenosis, elastosis perforans serpiginosa, Kyrle's disease and perforating folliculitis. Acquired reactive perforating collagenosis presents with transepidermal elimination of collagen fibers. Elastosis perforans serpiginosum presents with the elimination of elastic fibers. Kyrle's disease presents with transepidermal elimination of abnormal keratin. In perforating folliculitis, it is the content of the follicle. We established diagnostic criteria and severity classification. In addition, the Japanese guideline for treatment of perforating dermatoses was updated using the Medical Information Network Distribution Service (MINDS) methodology. The guideline is based on a systematic published work review completed from 1989 to 2019, and on a formal consensus and approval process. Most medical published work on the treatment is limited to individual case reports and small series of patients. The guideline covers treatment options considered relevant by the expert panel and approved in Japan at the time of the consensus conference.
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http://dx.doi.org/10.1111/1346-8138.15647DOI Listing
December 2020

Establishment of co-culture of human induced pluripotent stem cell-derived melanocytes and keratinocytes in vitro.

J Dermatol 2021 Jan 9;48(1):123-125. Epub 2020 Oct 9.

Division of Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

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http://dx.doi.org/10.1111/1346-8138.15623DOI Listing
January 2021

Clinical characteristics and social productivity levels of patients with malignant rheumatoid arthritis based on a nationwide clinical database in Japan: annual survey from 2003 to 2013.

Mod Rheumatol 2021 May 31;31(3):621-628. Epub 2020 Jul 31.

Department of Internal Medicine and Rheumatology, Juntendo University, Tokyo, Japan.

Objectives: Malignant rheumatoid arthritis (MRA) is defined as rheumatoid arthritis (RA) with systemic vasculitis or other severe extra-articular manifestations. Japan has a nationwide database for MRA. We analyzed the characteristics of Japanese patients with MRA based on data from the Ministry of Health, Labour and Welfare (MHLW).

Methods: We were permitted to use data on 43,108 patients who were registered in the MHLW database from 2003 to 2013.

Results: Median age was 65 (interquartile range, 57-72) years. Patients consisted of 71% females. Proportions of patients who had or had experienced interstitial pneumonia and pleuritis were increased, episcleritis was stable, and other MRA manifestations were decreased over time. The number of positive symptoms per patient also decreased over time. The median dose of glucocorticoid, percentage of patients undergoing surgery, and use of non-steroidal anti-inflammatory drugs and apheresis decreased year by year. Steinbrocker stage and class improved over time. Median C-reactive protein levels and erythrocyte sedimentation rate also decreased. Regarding social productivity levels of patients with MRA, the proportion of patients who were working or working from home increased and the proportion of patients recuperating or hospitalized decreased.

Conclusion: In patients with MRA, disease activity decreased and social productivity improved from 2003 to 2013.
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http://dx.doi.org/10.1080/14397595.2020.1795390DOI Listing
May 2021

Wound, pressure ulcer and burn guidelines - 1: Guidelines for wounds in general, second edition.

J Dermatol 2020 Aug 2;47(8):807-833. Epub 2020 Jul 2.

Department of Dermatology, JCHO Chukyo Hospital, Nagoya, Japan.

The Japanese Dermatological Association prepared the clinical guidelines for the "Wound, pressure ulcer and burn guidelines", second edition, focusing on treatments. Among them, "Guidelines for wounds in general" is intended to provide the knowledge necessary to heal wounds, without focusing on particular disorders. It informs the basic principles of wound treatment, before explanations are provided in individual chapters of the guidelines. We updated all sections by collecting references published since the publication of the first edition. In particular, we included new wound dressings and topical medications. Additionally, we added "Question 6: How should wound-related pain be considered, and what should be done to control it?" as a new section addressing wound pain, which was not included in the first edition.
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http://dx.doi.org/10.1111/1346-8138.15401DOI Listing
August 2020

Wound, pressure ulcer and burn guidelines - 6: Guidelines for the management of burns, second edition.

J Dermatol 2020 Nov 28;47(11):1207-1235. Epub 2020 Apr 28.

Department of Geriatric and Environmental Dermatology, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan.

"Wound, pressure ulcer and burn guidelines - 6: Guidelines for the management of burns, second edition" is revised from the first edition which was published in the Japanese Journal of Dermatology in 2016. The guidelines were drafted by the Wound, Pressure Ulcer and Burn Guidelines Drafting Committee delegated by the Japanese Dermatological Association, and intend to facilitate physicians' clinical decisions in preventing, diagnosing and treating burn injury. All sections are updated by collecting documents published since the publication of the first edition. Especially, the recommendation levels of dressing materials newly covered by the Japanese national health insurance are mentioned. In addition, the clinical questions (CQ) regarding the initial treatment of electrical (CQ15) and chemical burns (CQ16), and also the use of escharotomy (CQ22), are newly created.
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http://dx.doi.org/10.1111/1346-8138.15335DOI Listing
November 2020

Skin biopsies using dermoscopy for earlier diagnosis of intravascular large B-cell lymphoma.

J Dermatol 2020 Jul 10;47(7):e276-e278. Epub 2020 Apr 10.

Divisions of, Division of, Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

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http://dx.doi.org/10.1111/1346-8138.15350DOI Listing
July 2020

Survey of Japanese dermatological vasculitis specialists on cases of cutaneous arteritis (cutaneous polyarteritis nodosa).

J Dermatol 2020 May 24;47(5):534-537. Epub 2020 Feb 24.

Department of Dermatology, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.

We developed a questionnaire to examine the findings of cutaneous arteritis among dermatological specialists experienced in vasculitis as certified by the Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. We sent a questionnaire to 12 dermatological facilities identified through the revised Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. Retrospective data obtained from 84 patients at the 12 dermatological facilities between 2012 January 2016 December were evaluated. The 84 patients were categorized into two groups, a systemic steroid treatment group (group 1, n = 52) and a no systemic steroid treatment group (group 2, n = 32). C-reactive protein in group 1 patients was significantly higher than that in group 2 patients. Frequency of fever, arthritis, myalgia- and peripheral neuropathy in group 1 was significantly higher than that in group 2. We propose that these symptoms could serve as early markers for the transfer from cutaneous arteritis to systemic polyarteritis nodosa. We further suggest that patients who are subsequently associated with cerebral hemorrhage and infarction, who are originally diagnosed as having cutaneous arteritis, could progress to systemic polyarteritis nodosa. The study demonstrated that it is important for dermatologists to detect these findings early in order to establish an accurate diagnosis and a timely treatment.
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http://dx.doi.org/10.1111/1346-8138.15273DOI Listing
May 2020

Kojic acid alters pheomelanin content in human induced pluripotent stem cell-derived melanocytes.

J Dermatol 2020 Apr 17;47(4):435-436. Epub 2020 Feb 17.

Division of Dermatology, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

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http://dx.doi.org/10.1111/1346-8138.15260DOI Listing
April 2020

Relationship between lysosomal-associated membrane protein-2 and anti-phosphatidylserine/prothrombin complex antibody in the pathogenesis of cutaneous vasculitis.

Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):161-165. Epub 2020 Jan 27.

Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Japan.

Objectives: We investigated the relationship between lysosomal-associated membrane protein-2 (LAMP-2) and anti-phosphatidylserine/prothrombin (PS/PT) antibody in the pathogenesis of cutaneous vasculitis.

Methods: Cell surface LAMP-2 expression of human neutrophils was measured using flow cytometry. Twenty inbred wild-type Wistar-King-Aptekman-Hokudai (WKAH) rats were divided into four groups: Group 1, rabbit IgG injection only as negative control (n=5); Group 2, both histone and rabbit IgG injection (n=5); Group 3, anti-LAMP-2 antibody injection only (n=5); and Group 4, both histone and anti-LAMP-2 antibody injection (n=5). Ten WKAH rats were divided into two groups: Group A, histone, anti-PS/PT antibody, and anti-LAMP-2 antibody injection (n=5), and Group B, histone, anti-PS/PT antibody, and rabbit IgG injection as control (n=5).

Results: LAMP-2 expression on human neutrophils was induced by cell-free histone exposure in a dose- and time-dependent manner. Histopathological examination revealed the recruitment of neutrophils in cutaneous small vessels in all Group 4 rats. These observations were not evident in systemic organs other than the skin. LAMP-2 expression on the surface of vascular endothelial cells was evident in Group 2, exclusively in the skin, but not in Group 1. Thrombi were detected in various organs in all Groups A and B rats. However, no apparent thrombi were observed in the skin.

Conclusions: Anti-PS/PT and anti-LAMP-2 antibodies are responsible for independent effector mechanisms in the rats given intravenous injection of cell-free histones. We considered that undetermined factors other than cell-free histones could be required for the induction of cutaneous vasculitis by anti-PS/PT and anti-LAMP-2 antibodies.
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September 2020

Wound, pressure ulcer and burn guidelines - 4: Guidelines for the management of connective tissue disease/vasculitis-associated skin ulcers.

J Dermatol 2020 Oct 21;47(10):1071-1109. Epub 2020 Jan 21.

Tanioka Dermatology Clinic, Kyoto, Japan.

The Japanese Dermatological Association prepared guidelines focused on the treatment of skin ulcers associated with connective tissue disease/vasculitis practical in clinical settings of dermatological care. Skin ulcers associated with connective tissue diseases or vasculitis occur on the background of a wide variety of diseases including, typically, systemic sclerosis but also systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), various vasculitides and antiphospholipid antibody syndrome (APS). Therefore, in preparing the present guidelines, we considered diagnostic/therapeutic approaches appropriate for each of these disorders to be necessary and developed algorithms and clinical questions for systemic sclerosis, SLE, dermatomyositis, RA, vasculitis and APS.
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http://dx.doi.org/10.1111/1346-8138.15186DOI Listing
October 2020

Guidelines for the treatment of skin and mucosal lesions in Behçet's disease: A secondary publication.

J Dermatol 2020 Mar 6;47(3):223-235. Epub 2020 Jan 6.

Institute of Dermato-Immunology and Allergy, Southern Tohoku General Hospital, Koriyama, Japan.

In the current study, we present guidelines for the diagnosis and treatment of the mucocutaneous lesions of Behçet's disease, which is a chronic inflammatory disease characterized by the involvement of various organs, including mucocutaneous, ocular, vascular, intestinal and central nervous system lesions. It is often identified in the Middle East Mediterranean to East Asia region. Skin manifestations include erythema nodosum, papulopustular lesions and thrombophlebitis, and mucosal manifestations include oral and genital ulcers. These mucocutaneous lesions are characteristically the first signs of Behçet's disease and are important to be recognized for the early diagnosis of the disease. Moreover, these manifestations also recur and persist over the long-term course of the disease. The management of mucocutaneous lesions is important to prevent recurrence. We developed consensus guidelines that provide recommendations for general practitioners and dermatologists and physicians on the management of the mucocutaneous lesions of Behçet's disease.
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http://dx.doi.org/10.1111/1346-8138.15207DOI Listing
March 2020

Dectin-2-induced CCL2 production in tissue-resident macrophages ignites cardiac arteritis.

J Clin Invest 2019 06 6;129(9):3610-3624. Epub 2019 Jun 6.

Center for Immunology and Inflammatory Diseases, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Environmental triggers, including those from pathogens, are thought to play an important role in triggering autoimmune diseases, such as vasculitis, in genetically susceptible individuals. The mechanism by which activation of the innate immune system contributes to vessel-specific autoimmunity in vasculitis is not known. Systemic administration of Candida albicans water-soluble extract (CAWS) induces vasculitis in the aortic root and coronary arteries of mice that mimics human Kawasaki disease. We found that Dectin-2 signaling in macrophages resident in the aortic root of the heart induced early CCL2 production and the initial recruitment of CCR2+ inflammatory monocytes (iMo) into the aortic root and coronary arteries. iMo differentiated into monocyte-derived dendritic cells (Mo-DC) in the vessel wall and were induced to release IL-1β in a Dectin-2-Syk-NLRP3 inflammasome dependent pathway. IL-1β then activated cardiac endothelial cells to express CXCL1 and CCL2 and adhesion molecules that induced neutrophil and further iMo recruitment and accumulation in the aortic root and coronary arteries. Our findings demonstrate that Dectin-2-mediated induction of CCL2 production by macrophages resident in the aortic root and coronary arteries initiates vascular inflammation in a model of Kawasaki disease, suggesting an important role for the innate immune system in initiating vasculitis.
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http://dx.doi.org/10.1172/JCI123778DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6715376PMC
June 2019

[Cutaneous Manifestations of Sweet Disease].

Brain Nerve 2019 Apr;71(4):334-338

Division of Dermatology, Tohoku Medical and Pharmaceutical University.

Sweet disease, also known as acute febrile neutrophilic dermatosis, is a multisystem inflammatory disorder characterized by painful erythematous plaques and aseptic neutrophilic infiltration of various organs. It is also characterized by fever, polymorphonuclear leukocytosis, and painful erythematous cutaneous plaques. Cutaneous manifestations of Sweet disease are typically painful plaque-forming erythematous papules. Skin biopsies typically demonstrate dermal infiltration with neutrophils in the absence of vasculitis. Sweet disease is often associated with a hematological malignancy, especially acute myeloid leukemia or myeloid dysplasia syndrome. Sweet disease has clinical and laboratory features similar to those of Behçet's disease. Overlap exists between the clinical manifestations of Sweet disease and Behçet's disease. The neuro-Sweet disease is similar to the neuro-Behçet's disease. This article presents information on the pathogenesis, clinical approach, treatment, and expected evolution of these manifestations including the defference between neuro-Sweet disease and neuro-Behçet's disease. The possible role in this interesting association of Behçet's disease and Sweet disease is discussed and a review of the literature is presented.
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http://dx.doi.org/10.11477/mf.1416201271DOI Listing
April 2019

Sudden elevation of plasma D-dimer levels induced by the combination therapy of dabrafenib and trametinib: Report of two cases.

J Dermatol 2019 Apr 5;46(4):358-360. Epub 2019 Feb 5.

Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan.

The combination therapy of dabrafenib and trametinib revolutionized the treatment for BRAF V600-mutated melanoma. Various adverse events have been reported for this treatment, most notably fever. Herein, we report two cases of novel an adverse event, namely sudden and significant elevation of plasma D-dimer level induced by this therapy. In the first case, the remarkable elevation of plasma D-dimer level up to 87.4 mg/dL was noted on day 11, and in the second case, the plasma D-dimer level reached 125.5 mg/dL on day 25. In both cases, D-dimer levels gradually decreased after the cessation of this therapy. Although the exact cause is not clear, we assume two possible hypotheses: the first is that the combination therapy may induce disseminated intravascular coagulation, and the second is that the therapy induced pathological condition of secondary thrombotic microangiopathies. Our cases suggest that this thrombotic adverse event should not be overlooked, and coagulation parameters need to be monitored during the course of this treatment.
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http://dx.doi.org/10.1111/1346-8138.14798DOI Listing
April 2019

Formation and Disordered Degradation of Neutrophil Extracellular Traps in Necrotizing Lesions of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis.

Am J Pathol 2019 04 21;189(4):839-846. Epub 2019 Jan 21.

Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Japan. Electronic address:

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by the production of ANCAs and systemic necrotizing vasculitis in small vessels. Disordered regulation of neutrophil extracellular traps (NETs) is critically involved in the pathogenesis of AAV. NETs are web-like DNA decorated with antimicrobial proteins; they are extruded from activated neutrophils. The principal degradation factor of NETs in vivo is DNase I; however, NETs resistant to DNase I can persist in tissues and can lead to the production of ANCAs. Deposition of NETs has been demonstrated in glomerular crescents and necrotizing vasculitis in AAV. Here, the amount of NETs in formalin-fixed, paraffin-embedded tissue sections was examined, and the results for AAV were compared with the results for diseases that should be distinguished from AAV. NETs were more abundant in necrotizing vasculitis of AAV than in non-ANCA-associated vasculitis, or in granulomatous angiitis. Pulmonary granulomas in AAV and non-ANCA-associated diseases were further studied. The amount of NETs was significantly greater in necrotizing granulomas of AAV than in granulomas of sarcoidosis without necrosis. Although NETs were formed in necrotizing granulomas of tuberculosis equivalently to those formed in AAV, they were more susceptible to degradation by DNase I than were NETs in AAV. The formation and disordered degradation of NETs in necrotizing lesions are characteristics of AAV and are possibly related to its pathogenesis.
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http://dx.doi.org/10.1016/j.ajpath.2019.01.007DOI Listing
April 2019

2017 Clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis for the management of ANCA-associated vasculitis.

Mod Rheumatol 2019 Jan 10;29(1):20-30. Epub 2018 Sep 10.

p Division of Nephrology and Rheumatology, First Department of Internal Medicine , Kyorin University School of Medicine , Tokyo , Japan.

Objective: The Japan Research Committee for Intractable Vasculitis has fully revised the clinical practice guidelines (CPG) for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) to improve and standardize the medical treatment of the disease in Japan.

Methods: The previous CPG was published in a classical review style in Japanese in 2011 and 2014. We adopted the Grading of Recommendations Assessment, Development and Evaluation system for this revision, and various stakeholders, including patients, participated in it. The expected users of this CPG are AAV patients in Japan and their families and healthcare professionals, including both AAV specialists and non-specialists. We set clinical questions concerning the three important clinical topics of remission induction therapy, plasma exchange, remission maintenance therapy, and developed eight recommendation statements.

Results: For remission induction therapy for newly developed AAV, we weakly recommend glucocorticoid (GC) plus intravenous cyclophosphamide pulse (IVCY) or oral cyclophosphamide (POCY) rather than GC alone, and IVCY rather than POCY. We also weakly recommend CY rather than rituximab. In the case of AAV with severe renal impairment, we weakly recommend plasma exchange as a conjunction therapy. We weakly recommend azathioprine for remission maintenance therapy.

Conclusion: The revised CPG has demonstrated evidence-based treatment recommendations for AAV.
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http://dx.doi.org/10.1080/14397595.2018.1500437DOI Listing
January 2019

Clinical and Immunological Study of 30 Cases With Both IgG and IgA Anti-Keratinocyte Cell Surface Autoantibodies Toward the Definition of Intercellular IgG/IgA Dermatosis.

Front Immunol 2018 7;9:994. Epub 2018 May 7.

Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan.

Several sporadic cases, in which direct and indirect immunofluorescence studies simultaneously detected IgG and IgA autoantibodies to keratinocyte cell surfaces, have been reported mainly under the name of IgG/IgA pemphigus. However, there have been no systematic studies for this condition. In this study, we collected 30 cases of this condition from our cohort of more than 5,000 autoimmune bullous disease cases, which were consulted for our diagnostic methods from other institutes, and summarized their clinical and immunological findings. Clinically, there was no male-female prevalence, mean age of disease onset was 55.6 years, and mean duration before this condition was suspected was 18 months. The patients showed clinically bullous and pustular skin lesions preferentially on the trunk and extremities, and histopathologically intraepidermal pustules and blisters with infiltration of neutrophils and eosinophils. Immunologically, ELISAs frequently detected IgG and IgA autoantibodies to both desmogleins and desmocollins. From the characteristic clinical, histopathological, and immunological features, which are considerably different from those in classical IgG types of pemphigus, we propose this disease as a new disease entity with preferential name of intercellular IgG/IgA dermatosis (IGAD). This was the largest study of IGAD to date.
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http://dx.doi.org/10.3389/fimmu.2018.00994DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5950707PMC
July 2019

Risk Factors for Relapse of Antineutrophil Cytoplasmic Antibody-associated Vasculitis in Japan: A Nationwide, Prospective Cohort Study.

J Rheumatol 2018 04 1;45(4):521-528. Epub 2018 Feb 1.

From the Department of Environmental and Preventive Medicine, and Department of Nephrology and Laboratory Medicine, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa; Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama; Department of Rheumatology and Clinical Immunology, Saitama Medical Center, Saitama; Division of Endocrinology and Metabolism, Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa; Division of Rheumatology and Allergology, Departments of Internal Medicine and Dermatology, St. Marianna University School of Medicine, Kawasaki; Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University Graduate School of Medicine, Matsuyama; Department of Rheumatology, Faculty of Medicine, University of Tsukuba, Tsukuba; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Center for Nephrology and Urology, Division of Nephrology and Dialysis, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka; Third Department of Internal Medicine, Division of Immunology and Rheumatology, Hamamatsu University School of Medicine, Hamamatsu; Division of Rheumatology, Niigata Rheumatic Center, Shibata; Department of Pharmacovigilance, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University; Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine; Department of Respiratory Medicine, Toho University Omori Medical Center; Division of Nephrology, Tokyo Medical University Hachioji Medical Center; Department of Nephrology and Endocrinology, Graduate School of Medicine, The University of Tokyo; Nephrology and Rheumatology, First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan.

Objective: The aim was to elucidate the prognosis and risk factors associated with relapse during longterm remission maintenance therapy for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Methods: Patients with newly diagnosed AAV (n = 156) were registered in the Remission Induction Therapy in Japanese patients with ANCA-associated Vasculitides (RemIT-JAV) study, and among them, 83 patients who achieved remission were enrolled and followed up for 24 additional months in our nationwide, prospective cohort study (Co-RemIT-JAV; registration number UMIN 000006373). Patterns of maintenance therapy, effectiveness, and safety were evaluated from months 25 to 48 after the RemIT-JAV. The primary outcome measure was the rate of relapse. Secondary outcome measures included overall and renal survival, risk factors associated with relapse, and incidence rates of serious infections.

Results: The patients comprised 35 men and 48 women aged 65.3 ± 12.6 years. Between months 25 and 48, the survival rate was 95% (79/83). Causes of death included 1 thyroid cancer, 1 infection, and 2 unknown reasons. Four patients had developed endstage renal disease (ESRD) by Month 24; 1 developed ESRD beyond Month 25. The relapse rate was 24% (20/83) from months 25 to 48. Multivariable analysis revealed that oral prednisolone ≤ 2.5 mg/day at Month 24 was a significant risk factor for relapse between months 25 and 48 (HR = 3.1, 95% CI 1.1-8.5).

Conclusion: One-quarter of patients with AAV relapsed during maintenance therapy, and relapse was associated with the dose of oral prednisolone 24 months after the initiation of remission induction therapy in Japan.
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http://dx.doi.org/10.3899/jrheum.170508DOI Listing
April 2018

Disseminated erythema with intense and selective inflammation of sweat gland and lichenoid drug eruption during nivolumab therapy.

J Dermatol 2018 Feb 6;45(2):e33-e34. Epub 2017 Oct 6.

Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan.

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http://dx.doi.org/10.1111/1346-8138.14091DOI Listing
February 2018

Outline of guidelines for the management of vasculitis and vascular disorders in Japan, 2016 revised edition.

J Dermatol 2018 Feb 6;45(2):122-127. Epub 2017 Oct 6.

Department of Dermatology, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.

The proposal by the 1994 International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC1994) and by the CHCC2012 markedly influenced the classification and way of considering cutaneous vasculitis. In the proposal by the CHCC1994, hypersensitivity angiitis was defined as an equivalent pathological condition to microscopic polyangiitis or cutaneous leukocytoclastic angiitis (CLA), and it was not adopted as a disease name. However, CLA which was positioned as a type of small-vessel vasculitis is only a pathological name. In the proposal by the CHCC2012, a new category of single-organ vasculitis included CLA and cutaneous arteritis. Vasculitis allergica cutis (Ruiter) corresponded to CLA and cutaneous polyarteritis nodosa corresponded to cutaneous arteritis. The Japanese Dermatological Association (JDA) prepared guidelines for the management of vasculitis and vascular disorders in 2008 based on the proposal by the CHCC1994 and their original viewpoint of dermatology. The JDA subsequently revised the 2008 edition guidelines in 2016 following publication of the proposal of the CHCC2012 in Japanese. We presented the outline of the 2016 edition guidelines and propose a treatment algorithm for primary vasculitides based on the evaluation of the cutaneous symptoms for cases suspected as primary cutaneous vasculitides, which integrates the 2008 JDA guideline and CHCC2012 classification. This is the secondary English version of the original Japanese manuscript for the guideline for management of vasculitis and vascular disorders published in the Japanese Journal of Dermatology 127(3); 299-415, 2017.
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http://dx.doi.org/10.1111/1346-8138.14086DOI Listing
February 2018

Approach for the Derivation of Melanocytes from Induced Pluripotent Stem Cells.

J Invest Dermatol 2018 01 5;138(1):150-158. Epub 2017 Sep 5.

Institute of Dermatology & Cutaneous Sciences, Sapporo, Japan.

Induced pluripotent stem (iPS) cells have the ability to differentiate into multiple cell types in the body and have an unlimited growth potential. However, iPS cell-derived melanocytes produced by existing protocols have significant limitations in developing novel strategies for regenerative medicine and cell therapies of pigmentation disorders in humans because they involve culture in media containing fetal bovine serum and nonphysiological agents. In this study, we established an in vitro approach to generate iPS cell-derived human melanocytes that have higher proliferation rates and increased melanin production compared with melanocytes prepared by previously reported approaches. Importantly, our iPS cell-derived human melanocytes are prepared in fetal bovine serum-free culture conditions that do not contain any nonphysiological agents. We designed two original methods, transferring black colonies by pipette and recovering black cell pellets from centrifuged medium, and numerous human iPS cell-derived melanocytes proliferated in gelatinous dishes coated with Matrigel after 12 days. We also succeeded in inducing melanin pigmentation in the nude mouse skin in vivo using those human iPS cell-derived melanocytes. We propose that this method using iPS cells established from T cells in the blood of normal human volunteers could be applied clinically to develop regenerative medicine and cell therapies for various forms of human pigmentation disorders.
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http://dx.doi.org/10.1016/j.jid.2017.07.849DOI Listing
January 2018

Nerve conduction study of lower extremities in cutaneous arteritis patients with neurological manifestations.

J Dermatol 2017 Nov 30;44(11):1299-1302. Epub 2017 Jun 30.

Department of Internal Medicine, Division of Neurology, St Marianna University School of Medicine, Kawasaki, Japan.

Some patients originally diagnosed with cutaneous arteritis (CA) could develop additional disease manifestations, including peripheral neurological involvement. We evaluated the biological neurological parameters among CA patients who underwent nerve conduction studies for neurological involvement in the lower extremities. We reviewed 164 patients who were originally diagnosed with CA at our dermatology department between 2004 and 2015. Seventeen (10.4%) of the CA patients underwent further nerve conduction studies to determine their peripheral neurological manifestations, primarily in the lower extremities, in our neurology division. The frequency of low compound muscle action potential (CMAP) was significantly higher compared with that of delayed latency in both the peroneal nerve and sural nerve based on nerve conduction studies. The frequency of low CMAP was significantly higher compared with that of prolonged distal latency in both the peroneal and sural nerves. We suggest that impairment of the nerve axon pathways in the peroneal and sural nerves could result in the peripheral neurological manifestations in the lower extremities in CA patients.
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http://dx.doi.org/10.1111/1346-8138.13946DOI Listing
November 2017

Elevated levels of serum IgM anti-phosphatidylserine-prothrombin complex antibodies in patients with cancer-associated vasculitis.

Int J Dermatol 2017 10 19;56(10):e203-e204. Epub 2017 Jun 19.

Department of Dermatology, St. Marianna University School of Medicine, Kawasaki, Japan.

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http://dx.doi.org/10.1111/ijd.13689DOI Listing
October 2017
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