Abstract:
:We report a patient with the extremely rare familial multiple lipomatosis syndrome, who developed the uncommon autoimmune disease cytophagic histiocytic panniculitis, manifested as inflammation of preexisting lipomas. Despite his initial critical condition and unsuccessful treatment with steroids, he responded to cyclosporin and remains well 15 years after diagnosis. In contrast with most previous reports, our patient stays dependent on cyclosporin; repeated attempts of discontinuing or substituting treatment were quickly followed by relapse. Haemophagocytic panniculitis is considered as a T-cell disorder, but its exact pathophysiological mechanism has not been clarified. Differential diagnosis of cytophagic histiocytic panniculitis mainly includes malignant histiocytosis, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and lupus erythematosus panniculitis (lupus profundus). We discuss the main clinical features, diagnostic challenges and treatment issues of this usually benign, but at times life-threatening autoimmune condition.
journal_name
Mod Rheumatoljournal_title
Modern rheumatologyauthors
Krilis M,Miyakis Sdoi
10.1007/s10165-011-0487-7subject
Has Abstractpub_date
2012-02-01 00:00:00pages
158-62issue
1eissn
1439-7595issn
1439-7609journal_volume
22pub_type
杂志文章,评审abstract::Abstract An accumulating body of evidence shows that gene therapy can be successfully used to treat animal models of arthritis. Based on this success, a clinical trial of gene therapy for rheumatoid arthritis has been initiated. We review the methods and genes used for the previous gene transfer experiments, including...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/s101650050003
更新日期:2000-06-01 00:00:00
abstract::Adult-onset Still's disease (AOSD) is a rheumatoid disorder characterized by high fever, polyarthritis, leukocytosis, hyperferritinaemia, and mild liver involvement. We describe the case of a patient with AOSD with severe liver dysfunction. His serum levels of interleukin-10 and 18 showed a similar trend to his diseas...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-008-0065-9
更新日期:2008-01-01 00:00:00
abstract::Abstract A woman with Sjögren's syndrome concurrently suffered bilateral fractures of the femoral neck without any proximate cause, while she had aseptic necrosis of the femoral head resulting from the treatment for Sjögren's syndrome. According to previous reports, fractures of the femoral neck following aseptic necr...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/s10165-004-0346-x
更新日期:2004-12-01 00:00:00
abstract::A 33-year-old woman complaining of severe anemia was admitted to our hospital for polyclonal hyperglobulinemia. She was diagnosed with pure red cell aplasia (PRCA) associated with Evans syndrome. Initially, the presence of human parvovirus B19 (HPV B19) IgM appeared to indicate that the cause of PRCA was HPV B19 infec...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-007-0584-9
更新日期:2007-01-01 00:00:00
abstract::Bucillamine (Buc) is a disease-modifying antirheumatic drug (DMARD) developed in Japan, which has been used as one of the first-line DMARDs for the treatment of rheumatoid arthritis (RA) in Japan. However, direct comparison of this drug with standard DMARDs including sulfasalazine (SASP) and methotrexate (MTX) has bee...
journal_title:Modern rheumatology
pub_type: 临床试验,杂志文章
doi:10.1007/s10165-006-0527-x
更新日期:2006-01-01 00:00:00
abstract:OBJECTIVES:The introduction of powerful antirheumatic drugs has dramatically improved the treatment of rheumatoid arthritis (RA), leading clinicians to reconsider the benefits of joint preservation for rheumatoid forefoot deformities. We have employed joint-preserving forefoot surgeries, including rotational closing-we...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-012-0776-9
更新日期:2013-09-01 00:00:00
abstract:OBJECTIVE:To clarify polyarticular juvenile idiopathic arthritis (pJIA) patients who failed to maintain prolonged remission with the first biologic agent. METHODS:Fourteen pJIA patients were observed for 47.5 months (median) after initiating the first biologic agent. RESULTS:Eight maintained sustained clinical remiss...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/14397595.2015.1083147
更新日期:2016-01-01 00:00:00
abstract::We report a case of a 60-year-old female with cutaneous polyarteritis nodosa (CPN) of the left ankle, accompanied by elevated serum interleukin (IL)-6 levels. Computed tomographic angiography revealed severe narrowing of medium-sized arteries in her left leg. Destructive arthropathy in the left ankle was identified by...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/14397595.2013.852840
更新日期:2014-01-01 00:00:00
abstract::Synovial vascularization in the knee joints of six patients with rheumatoid arthritis who were treated with infliximab was evaluated by Doppler sonography. Power Doppler sonography demonstrated a significant reduction of color flow signals (P < 0.05), and spectral Doppler sonography demonstrated a significant increase...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-004-0359-5
更新日期:2005-01-01 00:00:00
abstract::To evaluate the efficacy of high-dose leukocytapheresis (LCAP) using a large filter in patients with refractory rheumatoid arthritis (RA), we conducted a multicenter, nonrandomized, open-label clinical study. Thirty patients with highly active RA were treated with high-dose LCAP performed 3-5 sessions at 1-week interv...
journal_title:Modern rheumatology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s10165-007-0630-7
更新日期:2007-01-01 00:00:00
abstract:OBJECTIVES:Psoriasis is a chronic autoimmune disease involving a complex network of cytokines such as interleukin (IL)-6. We tested the hypothesis that serum IL-6 level is a useful indicator of disease activity and predicts the treatment response to biologics in patients with psoriasis. METHODS:We analyzed 113 psorias...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/14397595.2016.1174328
更新日期:2017-01-01 00:00:00
abstract:OBJECTIVE:Upper limit of methotrexate (MTX) for patients with rheumatoid arthritis (RA) was recently increased from 8 to 16 mg/week in Japan. We therefore examined the effect of concomitant MTX dose on the efficacy of adalimumab (ADA) in clinical practice. METHOD:Sixty-one consecutive RA patients treated with ADA were...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/14397595.2016.1170958
更新日期:2017-01-01 00:00:00
abstract::We investigated the clinical efficacy and safety of tocilizumab (a humanized anti-IL-6 receptor antibody) monotherapy in active rheumatoid arthritis (RA) patients with an inadequate response to low dose methotrexate (MTX). In a multicenter, double-blind, randomized, controlled trial, 125 patients were allocated to rec...
journal_title:Modern rheumatology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s10165-008-0125-1
更新日期:2009-01-01 00:00:00
abstract::A 68-year-old Japanese male presented with atrophic erythematous white lesions with peripheral dark reddish rims on his back. Multiple ulcers were detected from his stomach to his large intestine using endoscopy. Although the patient was given high doses of a steroid, aspirin, dipyridamole, and intravenous immunoglobu...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/14397595.2013.874761
更新日期:2015-05-01 00:00:00
abstract::The purpose of this study was to describe the prevalence of total joint arthroplasty (TJA) in Japanese rheumatoid arthritis (RA) patients undergoing conventional drug treatment in a large observational cohort in Japan. A total of 5,177 RA patients were studied for the prevalence of TJA, who were enrolled in the NinJa ...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-009-0163-3
更新日期:2009-01-01 00:00:00
abstract::We examined the therapeutic effect of high molecular weight hyaluronic acid (HA) on the progression of joint pain and cartilage degeneration in a rabbit osteoarthritis (OA) model. The OA model was induced by partial meniscectomy. In the time course study, cartilage degeneration was assessed at 3, 7 and 14 days after o...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-010-0299-1
更新日期:2010-10-01 00:00:00
abstract::We report two cases of clinically amyopathic dermatomyositis (CADM) complicated by rapid progress interstitial lung disease (RPILD). The diagnosis of CADM was based on Gottron's papule and RPILD, and the absence of muscle weakness. The female patients were treated with early immunosuppressive therapies including stero...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-012-0637-6
更新日期:2013-01-01 00:00:00
abstract::For over 10 years there have been no clinical studies about adult-onset Still's disease (AOSD) in Japan. We aimed to investigate recent clinical features and treatment of AOSD and to evaluate the efficacy of cyclosporin A (CyA) in the treatment of AOSD. The data from 34 patients with AOSD who were admitted to our hosp...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-008-0126-0
更新日期:2009-01-01 00:00:00
abstract::We performed this study to investigate the differences in radiological and laboratory findings between systemic juvenile idiopathic arthritis (s-JIA) and polyarthritis (p-JIA). Twenty-two patients with s-JIA and 18 with p-JIA were enrolled. Their laboratory findings and radiographs were examined retrospectively. Plain...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-011-0540-6
更新日期:2012-08-01 00:00:00
abstract::Interstitial lung disease (ILD) is a noteworthy condition in the treatment of systemic sclerosis (SSc) because of its associated mortality and morbidity; however, the efficacy of various treatments for ILD has been controversial in previous reports. In this study, we examined the efficacy and safety of intravenous cyc...
journal_title:Modern rheumatology
pub_type: 临床试验,杂志文章
doi:10.1007/s10165-010-0403-6
更新日期:2011-06-01 00:00:00
abstract::Abstract Little is known about the specific effects of the ultra-high molecular weight polyethylene (UHMWP) debris that is obtained from human interface membranes after revision for a failed total hip arthroplasty. This paper reports the effects of retrieved polyethylene particles on human monocyte/macrophages (M/M). ...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/s101650070035
更新日期:2000-03-01 00:00:00
abstract::Sarcoidosis is a multisystem disease related to helper T cell responses. We recently experienced the case of a 57-year-old woman with sarcoidosis complicated by crescentic glomerulonephritis with low levels of myeloperoxidase-antineutrophil cytoplasmic antibody. We herein describe the details of her clinical course an...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-012-0614-0
更新日期:2013-01-01 00:00:00
abstract::Abstract This study examined the pathogenesis of large pseudocysts adjacent to knee joints in rheumatoid arthritis (RA). The radiological and histopathological features of 17 large subarticular pseudocysts in 12 knee joints of 10 patients were analyzed. Nine of the 10 patients were classified as class 2 according to ...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/s101650200022
更新日期:2002-06-01 00:00:00
abstract::Abstract To determine the specificities of autoantibodies targeting cell cycle-associated antigens in rheumatic diseases, we studied 30 sera which were obtained from patients visiting our hospital and which exhibited a variegated speckled pattern in indirect immunofluorescence (IF). The immunoreactivities of the sera ...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/s101650170008
更新日期:2001-09-01 00:00:00
abstract::Anti-tumor necrosis factor (anti-TNF) biologics are effective in the treatment of rheumatoid arthritis (RA); however, it is still not clear whether this treatment promotes the development of malignancies such as lymphoma. Human T-lymphotropic virus type 1 (HTLV-1), which is a causative agent of adult T-cell lymphoma (...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/14397595.2013.844389
更新日期:2015-09-01 00:00:00
abstract::We investigated the association between cigarette smoking and the risk of developing rheumatoid arthritis (RA) in the Malaysian population. A total of 1,056 RA patients and 1,416 matched controls aged 18-70 years within a defined area of Peninsular Malaysia were evaluated in a case-control study between August 2005 an...
journal_title:Modern rheumatology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10165-011-0544-2
更新日期:2012-08-01 00:00:00
abstract:OBJECTIVE:Avascular necrosis of bone (AVN) is an important complication of systemic lupus erythematosus (SLE). Corticosteroid therapy has been underlined as a main risk factor for osteonecrosis. However, AVN development in patients who have never received corticosteroid and the absence of AVN in the majority of the pat...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.3109/14397595.2014.987366
更新日期:2015-07-01 00:00:00
abstract::Clinical squeal of the treatment of rheumatoid arthritis patients with methotrexate (MTX) according to the Japanese government recommended dose of 8 mg/week was evaluated prospectively. A total of 176 patients with active RA attending Konan Kakogawa Hospital and Kobe University Hospital were enrolled. Patients' profil...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-009-0205-x
更新日期:2009-01-01 00:00:00
abstract::Rheumatoid arthritis (RA) is a complex process of chronic and progressive inflammation associated with activation of numerous signaling molecules and transcription factors and hyperproliferation of synoviocytes of the affected joints, although the greater part of its pathophysiological process is explained by activati...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-004-0372-8
更新日期:2005-01-01 00:00:00
abstract::We report a novel case of eosinophilic orbital myositis associated with Churg-Strauss syndrome. A 56-year-old man with a 20-year history of chronic sinusitis and seasonal allergic rhinitis was admitted because of fever, swelling of cheeks and extremities, diplopia, and eosinophilia. With findings from gadolinium-enhan...
journal_title:Modern rheumatology
pub_type: 杂志文章
doi:10.1007/s10165-009-0252-3
更新日期:2010-04-01 00:00:00