Abstract：
:Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years. More SJIA patients had MAS at disease onset than SLE patients (53.6 vs. 16.7 %). Fever, high C-reactive protein and hyperferritinemia were present in all MAS episodes. Rash was less (p = 0.03), and fatigue was more frequent (p = 0.042) in SLE than SJIA patients. All received corticosteroids. Cyclosporine was given in 74.2 % of SJIA-MAS; 66.7 % of SLE-MAS episodes. Intravenous immunoglobulin, anakinra, or etoposide was administered during 67.7; 41.9; 32.3 % of SJIA-MAS and 33.3; 33.3; 50 % of SLE-MAS episodes, respectively. Plasmapheresis was performed during 41.9 % of SJIA-MAS and 33.3 % of SLE-MAS episodes. The mortality rate was 11.8 % (n = 4;3 SJIA, 1 SLE). Hepatosplenomegaly was more frequent (p = 0.005), and plasmapheresis was performed more frequently (p = 0.021) in the patients who died compared to the cured patients. The median duration between symptom onset and admission to our hospital was longer among the patients who died (16.5 vs. 7 days; p = 0.049). Our patients' characteristics were similar to the reported cases, but our mortality rate is slightly higher probably due to late referral to our center. Early diagnosis and effective treatment are crucial to prevent mortality.
journal_name
Rheumatol Intjournal_title
Rheumatology internationalauthors
Aytaç S,Batu ED,Ünal Ş,Bilginer Y,Çetin M,Tuncer M,Gümrük F,Özen Sdoi
10.1007/s00296-016-3545-9subject
Has Abstractpub_date
2016-10-01 00:00:00pages
1421-9issue
10eissn
0172-8172issn
1437-160Xpii
10.1007/s00296-016-3545-9journal_volume
36pub_type
杂志文章abstract：:Osteopoikilosis, a form of osteosclerotic disorders, is a rare bone dysplasia with typical radiologic findings. The pathology, which sometimes requires attention in differential diagnosis due to its radiologic appearance, has been reported previously with other diseases. In this report, a case diagnosed with both reac...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-005-0037-8
更新日期：2006-05-01 00:00:00
abstract：:In a prospective study 222 sera from 56 patients with systemic lupus erythematosus (SLE) were tested for antibodies to poly(A), poly dAT and histones using enzyme-linked immunosorbent assay (ELISA). Patients with active disease had significantly more often antibodies to poly(A), poly dAT and histones than patients wit...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/BF00541200
更新日期：1984-01-01 00:00:00
abstract：:Fifty-five patients with systemic lupus erythematosus (SLE) were examined for antineutrophil cytoplasmic antibodies (ANCA) by indirect immunofluorescence (IIF). Enzyme-linked immunosorbent assay (ELISA) for ANCA against myeloperoxidase (MPO), lactoferrin (LF), proteinase 3 (PR3), elastase (HLE), and bactericidal/perme...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s002960100108
更新日期：2001-07-01 00:00:00
abstract：:Although arthroscopic surgery (AS) for knee osteoarthritis has been widely employed, scientific evidence is lacking. The purpose of this study was to investigate temporal changes in synovial fluid levels of biochemical markers associated with cartilage metabolism following AS. Twenty-five knees of 24 patients with med...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-012-2374-8
更新日期：2013-01-01 00:00:00
abstract：:Systemic lupus erythematosus (SLE) is a chronic and complex autoimmune disease characterized by the production of autoantibodies against a spectrum of nuclear antigens. RANTES and its receptor CCR5 have been associated with the pathogenesis of SLE. The objective of this study is to analyze autoantibodies (DNA/RNA), al...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-013-2812-2
更新日期：2013-11-01 00:00:00
abstract：:Familial Mediterranean fever is a chronic inflammatory disease characterized by periodic and self-limited episodes of fever and aseptic polyserositis. Although colchicine treatment has altered the course of the disease, it is believed that subclinical inflammation is still present, leading to endothelial dysfunction a...
journal_title：Rheumatology international
pub_type： 杂志文章,评审
doi：10.1007/s00296-019-04457-8
更新日期：2020-01-01 00:00:00
abstract：:Different genetic variants in estrogen receptor alpha (ESR1) have been shown to influence bone phenotypes including quantitative bone ultrasound in elderly. We aimed to investigate the role of ESR1 polymorphisms in bone mass assessed by calcaneal quantitative ultrasound (QUS) in a population of young adults. The study...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-017-3748-8
更新日期：2017-08-01 00:00:00
abstract：:In a study of synovial fluid from 110 patients with various forms of arthritis, platelets were identified in the synovial fluid of all the 50 rheumatoids, in 18 out of the 25 (72%) with osteoarthritis and in all 35 of those with other forms of inflammatory osteoarthrosis. Identification of platelets by light microscop...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/BF00683878
更新日期：1984-01-01 00:00:00
abstract：:The aim of this study was to evaluate the effectiveness of intravenous corticosteroid therapy when Henoch-Schönlein purpura (HSP) patients are unable to tolerate oral medications due to abdominal pain. We retrospectively analyzed 111 children with a diagnosis of HSP (mean age 6.9 ± 2.3 years, male:female = 54:57) from...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-010-1507-1
更新日期：2011-11-01 00:00:00
abstract：:Anti-TNF drugs may increase lymphoma risk in autoimmune rheumatic diseases, such as rheumatoid arthritis, but there have been no reports stating increased risk of lymphoma in ankylosing spondylitis (AS). Before 2 years, we had presented a case with AS developing Hodgkin's lymphoma following 6 months of etanercept trea...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-009-1265-0
更新日期：2011-12-01 00:00:00
abstract：:This study evaluates prospectively whether baseline scores [Health Assessment Questionnaire (HAQ) and SF-36] can predict clinical and radiographic evolution in a cohort of early rheumatoid arthritis (RA) during a 3-year follow-up. Forty consecutive early RA patients were followed for 3 years, while receiving standardi...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-011-2261-8
更新日期：2012-12-01 00:00:00
abstract：:The current study was to investigate the quantity and quality of researches in the field of ankylosing spondylitis, and to reveal the characteristics of worldwide productivity on this disease. This was a bibliometric study on ankylosing spondylitis using Web of Science. The numbers of papers, citations, research outpu...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-019-04308-6
更新日期：2019-06-01 00:00:00
abstract：:The aim of our study was to evaluate effectiveness of ultrasound treatment applied with exercise therapy in patients with ankylosing spondylitis. Fifty-two patients, who were diagnosed according to modified New York criteria, were aged 25-60, and have spine pain, were randomly assigned to two groups. Ultrasound (US) a...
journal_title：Rheumatology international
pub_type： 杂志文章,随机对照试验
doi：10.1007/s00296-016-3441-3
更新日期：2016-05-01 00:00:00
abstract：:Fibrosis is unregulated tissue repair that may cause impairment of organ function, especially in end-organ damage. Systemic sclerosis (SSc) is the prototype systemic fibrosing disorder. Classical targets for fibrosis in SSc like transforming growth factor Beta (TGF-β), Interleukin-6 (IL-6), and multiple tyrosine kinas...
journal_title：Rheumatology international
pub_type： 杂志文章,评审
doi：10.1007/s00296-019-04317-5
更新日期：2019-07-01 00:00:00
abstract：:The objective of the study was to assess the ESSDAI index characteristics in the SJÖGRENSER cohort (Spanish Rheumatology Association's registry of patients with Primary Sjögren Syndrome [PSS]). SJÖGRENSER is a prospective multicentric study on a cohort of Spanish patients with PSS who meet the 2002 American-European c...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-019-04285-w
更新日期：2019-06-01 00:00:00
abstract：:Microscopic polyangiitis (MPA) is a systemic necrotizing vasculitis affecting small vessels without necrotizing granulomatous inflammation and is commonly associated with necrotizing glomerulonephritis. Diagnosis is based on typical clinical features, the presence of antimyeloperoxidase-antineutrophil cytoplasmic anti...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-006-0127-2
更新日期：2006-08-01 00:00:00
abstract：:The objective of this study was to search for proteomic patterns distinguishing patients with rheumatoid arthritis (RA) from healthy controls, biomarker candidates specific for early RA, and proteins reflecting disease activity, by profiling of serum proteins using magnetic bead-based (MB) separation and matrix-assist...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-010-1407-4
更新日期：2011-08-01 00:00:00
abstract：:We have examined the way in which products of cultured human blood mononuclear cells activate human articular chondrocytes. Conditioned medium from mononuclear cells enhanced the production of prostaglandin E by cultured human chondrocytes and also stimulated fibrinolytic activity in these cultures. These two effects ...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/BF00541204
更新日期：1984-01-01 00:00:00
abstract：:The association of familial Mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established. These patients have been reported to have an overall better prognosis than other PAN patients. Herein we report a patient with FMF and PAN who died of sepsis following a severe course of recurrent bleeding e...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-003-0424-y
更新日期：2005-01-01 00:00:00
abstract：:Schistosomiasis or bilharzia is a parasitic disease found in tropical countries. Most infections are subclinical but may progress to chronic form characterized most frequently by the presence of liver involvement and portal hypertension. We report a patient that presented chronic polyarthritis with positive rheumatoid...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-010-1562-7
更新日期：2013-05-01 00:00:00
abstract：:Systemic sclerosis is a connective tissue disease, which may lead to elevated pulmonary arterial pressure due to pulmonary arterial hypertension and/or left ventricular diastolic dysfunction. Uric acid (UA) has been shown to be elevated in patients with pulmonary hypertension (PH) and heart failure. We aimed to invest...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-010-1557-4
更新日期：2011-02-01 00:00:00
abstract：:We aimed to investigate how orbital blood flow rates in patients with rheumatoid arthritis (RA) are affected by the active and remission phase of the disease. This prospective study included a total of 56 patients with RA (study group) and 24 control individuals (control group). All RA patients were divided into two g...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-019-04275-y
更新日期：2019-06-01 00:00:00
abstract：:It is known that patients with rheumatoid arthritis (RA) have an increased risk for non-Hodgkin's lymphomas in comparison with the general population. Although increased risk of lymphoma is attributed to the disease activity, the drugs used in the therapy of RA may also cause increased risk of malignancy. Herein, we r...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-009-1184-0
更新日期：2011-04-01 00:00:00
abstract：:The objective of this research was to investigate the cellular source of soluble ICAM-1 (siCAM-1) from rheumatoid synovial tissue (RS) and its relation to sICAM-1 in synovial fluid (SF) and serum, and to study the expression of ICAM-1 in isolated cells of RS. sICAM-1 was determined by using the enzyme-linked immunosor...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/pl00006846
更新日期：1997-01-01 00:00:00
abstract：OBJECTIVES:To identify recommendations on the diagnosis and management of rheumatoid arthritis (RA) supported by low recommendation grades, to study the causes of this low grading, and to propose solutions. METHODS:A group of six rheumatologists, with extensive experience in the development of systematic reviews, revi...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-020-04724-z
更新日期：2021-01-01 00:00:00
abstract：:The objective of the study was to determine the clinical features and treatment course in Canadian patients with dermatomyositis (DM) associated with the anti-melanoma differentiation-associated gene 5 antibody (MDA5). A retrospective chart review of consecutive patients with anti-MDA5 antibody DM from two Canadian te...
journal_title：Rheumatology international
pub_type： 杂志文章,评审
doi：10.1007/s00296-019-04398-2
更新日期：2019-11-01 00:00:00
abstract：:During the coronavirus disease-2019 (COVID-19) pandemic there were several barriers to treatment access and medication adherence in rheumatoid arthritis (RA) patients. There is no information regarding the RA patient health status in Egypt during the COVID-19. Thus,the aim of this work was to study the impact of the p...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-020-04736-9
更新日期：2021-02-01 00:00:00
abstract：:The aim of this study was to investigate the frequency of patients with rheumatoid arthritis (RA) who have inflammatory back pain (IBP) and meet the existing classification criteria for ankylosing spondylitis (AS) and spondyloarthritis (SpA). We included 167 patients fulfilling the ACR 1987 revised criteria for RA. Af...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s00296-012-2553-7
更新日期：2013-05-01 00:00:00
abstract：:A 50-year-old man presented with a complaint of low-back pain and widespread joint pain for the previous 20 years. Conventional radiography revealed wide areas of calcification in the intervertebral discs and degenerative changes in the peripheral joints. A diagnosis of ochronosis was made by the observation of bluish...
journal_title：Rheumatology international
pub_type： 杂志文章,评审
doi：10.1007/s00296-007-0381-y
更新日期：2007-11-01 00:00:00
abstract：:Aseptic loosening is the predominant cause of total hip implant failure. It has been assumed that a layer or membrane, containing macrophages, fibroblasts and vascular endothelial cells, of synovial-like tissue develops at the implant-to-bone interface almost invariably and, with time, somehow leads to loosening of th...
journal_title：Rheumatology international
pub_type： 杂志文章
doi：10.1007/s002960050037
更新日期：1998-01-01 00:00:00
