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ID PMID Title PublicationDate abstract
23596590 Current status of registry of vaccine clinical trials conducted by Korean investigators in 2013 Jan PURPOSE: PubMed is not only includes international medical journals but also has a registration site for the ongoing clinical trials, such as ClinicalTrials.gov, under the supervision of US National Institutes of Health. We analyzed current status of vaccine clinical trials conducted by Korean investigators in database of ClinicalTrial.gov. MATERIALS AND METHODS: As of October 2012, there are total of 72 trials found on registry of vaccine clinical trials conducted by Korean investigators in database of ClinicalTrial.gov. These trials were analyzed and classified by conditions of vaccine clinical trials, biologicals or drugs used in vaccine clinical trials, status of proceeding research, and list of sponsor and collaborators. RESULTS: Total 72 trials of vaccine clinical trials conducted by Korean investigators are classified by groups of infection (64 trials), cancer (4 trials), and others (4 trials). Infections group shown are as follows: poliomyelitis, pertussis, diphtheria, tetanus, and Haemophilus influenzae type b (10), influenza (9), human papillomavirus infection (8), pneumococcal vaccine (6), herpes zoster (4), smallpox (4), hepatitis B (4), etc. One trial of each in lung cancer, breast cancer, prostate cancer, and colorectal cancer are shown in cancer group. One trial of each in Crohn's disease, ulcerative colitis, renal failure, and rheumatoid arthritis are shown in other group. CONCLUSION: Vaccine clinical trials conducted by Korean investigators in ClinicalTrial.gov reflects the current status of Korean research on vaccine clinical trials at the international level and can indicate research progress. It is hoped that this aids the development of future vaccine clinical trials in Korea.
23556512 Pulmonary manifestations in Behçet disease: impaired natural killer cells activity. 2013 Apr 4 BACKGROUND: Behçet's disease (BD) is a systemic vasculitis with unknown aetiology, where, besides genetic predisposition, an immune dysregulation involving T and B lymphocytes and hyperactive neutrophils contribute to disease pathogenesis. The aim of this study was to determine the cytotoxicity of natural killer (NK) cells in bronchoalveolar lavage (BAL) from BD patients with pulmonary manifestations. METHODS: BAL was performed in 27 patients with BD and pulmonary manifestations, 14 patients with Rheumatoid Arthritis (RA) and 23 healthy controls (HC). Related orphan receptor C (RORC) and forkheadbox P3 (FOXP3) mRNA transcript were determined in BAL by reverse transcription-polymerase chain reaction (RT-PCR). NK cells, NK cell cytotoxicity, and lymphokine-activated killer (LAK) activity against K562 cells were measured by flow cytometry. Proportions of NK precursors and expression of genes for IL-2 receptor β (IL-2Rβ; CD122), perforin, and granzyme in NK cells were measured by flow cytometry or RT-PCR. RESULTS: The analysis of transcription factors revealed an increase in the RORC/FOXP3 ratio (Th17/Treg cells) in BAL from BD patients. Percentages of NK were significantly lower in BD than in RA patients and healthy controls. Purified NK cells derived from BD patients were found to have lower cytotoxicity and LAK activity than those from controls. This defect of NK cells in BD patients was related to down-regulation of perforin and granzyme expression in NK cells. CONCLUSION: In BD patients, the increased RORC/FOXP3 ratio indicated an inflammatory state of the lung. NK cells were decreased together with an impairment of their activity due to a defective expression of granzyme and perforin. These abnormalities possibly contribute to immune system dysregulation found in BAL of BD patients with pulmonary manifestations.
23549045 The Prostate Cancer Patient Had Higher C-Reactive Protein Than BPH Patient. 2013 Feb PURPOSE: C-reactive protein (CRP) is a general marker for inflammation and it has been associated with prostate cancer. We hypothesized that a correlation may exist between CRP and prostate cancer in patients undergoing transrectal biopsy of the prostate because of rising prostate-specific antigen (PSA) levels. MATERIALS AND METHODS: From January 2009 to March 2012, we retrospectively reviewed 710 patients who visited our urology department and were diagnosed as having a PSA value over 4.0 ng/mL. Patients with acute infections, rheumatoid arthritis, gout, asthma, chronic lung disease, myocardial infarction, or apoplexy and those who had taken nonsteroidal anti-inflammatory drugs were exempted from the research because these variables could have impacted CRP. After we applied the exclusion criteria, we selected 63 patients with prostate cancer and 140 patients with benign prostatic hyperplasia (BPH). RESULTS: A total of 203 patients were observed: 140 patients had BPH, and 63 patients had prostate cancer. Prostate cancer patients were divided into two groups by tumor-node-metastasis classification. The patients below T2 were group A, and those above T3 were group B. The natural logarithm of C-reactive protein (lnCRP) differed between the BPH group and the prostate cancer group. The lnCRP also differed between the BPH group and prostate cancer groups A and B (p<0.05). CONCLUSIONS: The serum CRP level of the prostate cancer group was higher than that of the BPH group. Inflammation may be correlated with prostate cancer according to the serum CRP level.
23524900 [Development of sarcoidosis during treatment for chronic hepatitis C with pegylated interf 2013 Mar Sarcoidosis is a chronic inflammatory multisystem disease of unknown aetiology. IFN-alfa is an immunomodulatory cytokine, currently used with ribavirin in the treatment of chronic hepatitis C infection. Due to its effects on the immune system, IFN-? may lead to the induction or exacerbation of autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis and sarcoidosis. We report on a 37-year-old man, presenting with sarcoidosis during a six-month course of PEG-IFN alfa-2a and ribavirin for chronic hepatitis C virus genotype 3 infection.
23505390 Ubiquitous polygenicity of human complex traits: genome-wide analysis of 49 traits in Kore 2013 Recent studies in population of European ancestry have shown that 30% ~ 50% of heritability for human complex traits such as height and body mass index, and common diseases such as schizophrenia and rheumatoid arthritis, can be captured by common SNPs and that genetic variation attributed to chromosomes are in proportion to their length. Using genome-wide estimation and partitioning approaches, we analysed 49 human quantitative traits, many of which are relevant to human diseases, in 7,170 unrelated Korean individuals genotyped on 326,262 SNPs. For 43 of the 49 traits, we estimated a nominally significant (P<0.05) proportion of variance explained by all SNPs on the Affymetrix 5.0 genotyping array ([Formula: see text]). On average across 47 of the 49 traits for which the estimate of h(G)(2) is non-zero, common SNPs explain approximately one-third (range of 7.8% to 76.8%) of narrow sense heritability. The estimate of h(G)(2) is highly correlated with the proportion of SNPs with association P<0.031 (r(2) = 0.92). Longer genomic segments tend to explain more phenotypic variation, with a correlation of 0.78 between the estimate of variance explained by individual chromosomes and their physical length, and 1% of the genome explains approximately 1% of the genetic variance. Despite the fact that there are a few SNPs with large effects for some traits, these results suggest that polygenicity is ubiquitous for most human complex traits and that a substantial proportion of the "missing heritability" is captured by common SNPs.
23471975 Atypical femoral fractures shortly after osteonecrosis of the jaw in a postmenopausal woma 2013 Apr CONTEXT: Bisphosphonates effectively increase bone mineral density and reduce fracture risk in patients with osteoporosis, but there are concerns about osteonecrosis of the jaw (ONJ) and atypical femoral fractures (AFFs) in the long-term users. So far both complications have not been reported as occurring simultaneously in an osteoporotic individual on oral alendronate. OBJECTIVE: The aim of this study was to report a postmenopausal woman presenting with concomitant ONJ and AFF on oral alendronate treatment. SUBJECT, MEASURES, AND RESULT: The patient was a 63-year-old woman with a history of rheumatoid arthritis for 30 years and diabetes for 3 years. Spinal compression fractures at levels L3 and L4 were documented, and she took alendronate 70 mg weekly for 7 years. She is the first case whose dental periapical imaging and pelvic radiography documented her ONJ and AFF, which developed subsequently within 6 months. CONCLUSIONS: This case report supports the association of both ONJ and AFF with long-term oral bisphosphonate therapy.
23402800 Pesticides and human chronic diseases: evidences, mechanisms, and perspectives. 2013 Apr 15 Along with the wide use of pesticides in the world, the concerns over their health impacts are rapidly growing. There is a huge body of evidence on the relation between exposure to pesticides and elevated rate of chronic diseases such as different types of cancers, diabetes, neurodegenerative disorders like Parkinson, Alzheimer, and amyotrophic lateral sclerosis (ALS), birth defects, and reproductive disorders. There is also circumstantial evidence on the association of exposure to pesticides with some other chronic diseases like respiratory problems, particularly asthma and chronic obstructive pulmonary disease (COPD), cardiovascular disease such as atherosclerosis and coronary artery disease, chronic nephropathies, autoimmune diseases like systemic lupus erythematous and rheumatoid arthritis, chronic fatigue syndrome, and aging. The common feature of chronic disorders is a disturbance in cellular homeostasis, which can be induced via pesticides' primary action like perturbation of ion channels, enzymes, receptors, etc., or can as well be mediated via pathways other than the main mechanism. In this review, we present the highlighted evidence on the association of pesticide's exposure with the incidence of chronic diseases and introduce genetic damages, epigenetic modifications, endocrine disruption, mitochondrial dysfunction, oxidative stress, endoplasmic reticulum stress and unfolded protein response (UPR), impairment of ubiquitin proteasome system, and defective autophagy as the effective mechanisms of action.
23340909 [Tubulointerstitial nephritis with uveitis (TINU) syndrome. A relatively rare rheumatologi 2013 May The tubulo-interstitial nephritis and uveitis (TINU) syndrome, first described in 1975, is a rare disease most probably of autoimmune origin that is characterized by unilateral or bilateral uveitis and tubulointerstitial nephritis. Most patients are adolescents and it is sometimes associated with other autoimmune diseases, such as spondyloarthritis, rheumatoid arthritis and hyperthyroidosis. This article reports the case of a 43-year-old female patient who presented with refractory recurrent bilateral uveitis despite therapy with high doses of corticosteroids in combination with cyclosporin. When the patient was referred to this hospital for rheumatological examination after almost 1 year of therapy, mild renal insufficiency and proteinuria were found. The kidney biopsy revealed interstitial nephritis, partly crescent-shaped and partly chronic. A diagnosis of TINU syndrome was made and treatment with adalimumab in combination with methotrexate was started. The favorable clinical outcome indicated that tumor necrosis factor (TNF) alpha may play an important role in the pathogenesis of TINU syndrome.
23325330 The hygiene theory harnessing helminths and their ova to treat autoimmunity. 2013 Oct The incidence of autoimmune diseases is increasing in Western countries, possibly due to the improved sanitary conditions and reduced exposure to infections in childhood (the hygiene hypothesis). There is an ongoing debate whether infection prevents or precipitates autoimmune diseases. Various helminths species used in several animal models were shown to limit inflammatory activity in a variety of diseases including inflammatory bowel disease, multiple sclerosis, type 1 diabetes, rheumatoid arthritis, and systemic lupus erythematosus. At present the scientific data is based mostly on experimental animal models; however, there is an increasing body of evidence in a number of clinical trials being conducted. Herein we review several clinical trials evaluating the anti-inflammatory effects of helminths and assessing their association with different autoimmune diseases, including inflammatory bowel disease, multiple sclerosis, and autoimmune liver diseases. We also describe the common pathways by which helminths induce immune modulation and the key changes observed in the host immune system following exposure to helminths. These common pathways include the inhibition of IFN-γ and IL-17 production, promotion of IL-4, IL-10 and TGF-β release, induction of CD4(+) T cell FoxP3(+) expression, and generation of regulatory macrophages, dendritic cells, and B cells. Helminths products are becoming significant candidates for anti-inflammatory agents in this context. However, further research is needed for synthetic analogues of helminths' potent products that mimic the parasite-mediated immunomodulation effect.
23253448 P2X receptors as drug targets. 2013 Apr The study of P2X receptors has long been handicapped by a poverty of small-molecule tools that serve as selective agonists and antagonists. There has been progress, particularly in the past 10 years, as cell-based high-throughput screening methods were applied, together with large chemical libraries. This has delivered some drug-like molecules in several chemical classes that selectively target P2X1, P2X3, or P2X7 receptors. Some of these are, or have been, in clinical trials for rheumatoid arthritis, pain, and cough. Current preclinical research programs are studying P2X receptor involvement in pain, inflammation, osteoporosis, multiple sclerosis, spinal cord injury, and bladder dysfunction. The determination of the atomic structure of P2X receptors in closed and open (ATP-bound) states by X-ray crystallography is now allowing new approaches by molecular modeling. This is supported by a large body of previous work using mutagenesis and functional expression, and is now being supplemented by molecular dynamic simulations and in silico ligand docking. These approaches should lead to P2X receptors soon taking their place alongside other ion channel proteins as therapeutically important drug targets.
23201917 Cartilage as a target of autoimmunity: a thin layer. 2013 Mar The articular cartilage is an important component of human organism that has elasticity, low-friction surface, and ability to withstand great physical forces. The structure consists of collagens and proteoglycans, whereas non-collagenous proteins are needed for the organization and modulation of the molecular networks. The structural elements of the cartilage are typical to that tissue and could, in part, account for the localization of the inflammatory response to the joint. For this reason cartilage is of particular interest in autoimmunity as it may represent a source of antigens. It is well known that sensitization with collagens can produce autoimmune rheumatic diseases in experimental models. So far, the cartilage proteins that have been clearly characterized to be arthritogenic in experimental models involve types II and XI collagen, cartilage oligomeric matrix protein, and aggrecan. It is likely that these proteins are also recognized at different stages in the development of rheumatoid arthritis and in other autoimmune diseases. The mechanisms determining the trigger of a cartilage-specific immune response, its development and outcome are poorly understood. Most likely, the distribution and concentration of a specific cartilage protein may play a role by eliciting an autoimmune response. Indeed, the inflammatory processes lead to tissue damage mediated by the intervention of several factors such as autoantibodies, cytokines as well as cells of the innate an adaptive immunity. For this reason, even previously-considered degenerative diseases, such as osteoarthritis, should now be re-evaluated as at least partly inflammatory-driven. Thus, the objective of this review is to describe the clinical conditions sustained by the immune-mediated reactions to cartilage, which represents the target organ in a number of autoimmune diseases.
23181348 Hormones and AID: balancing immunity and autoimmunity. 2013 Mar The human immune system is a complex dynamic network of soluble factors and specialized cells that can and need to act in an instance or keep a lifelong protection, with the consequence that health has to be maintained through genetic and environmental stimuli. Autoimmunity is a multifactorial disease, where this combination of genetic predisposition and environmental factors lead to disease etiology. As some autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) or other B cell autoimmunities have a very strong female gender bias, hormones, especially estrogen, have been implicated as environmental factors in driving the disease. One of the key regulators of B cell development is activation-induced deaminase (AID), as its molecular mechanism of cytosine deamination induces immunoglobulin affinity maturation and antibody class switching. In this review we will highlight some of the recent findings of how estrogen directly and indirectly activates AID expression, which in turn can lead to immune hyper-stimulation. Those regulatory pathways can be direct when the estrogen receptor (ER) binds the AID promoter, or indirect via activation of transcription factors that enhance AID expression (e.g., HoxC4). Estrogen's influence on AID will also be discussed in terms of microRNA processing for miRNA-155 and miRNA-181b. Important other external stimuli, such as EBV virus, in conjunction with estrogen can add another layer of regulation during autoimmune disease progression. Understanding these pathways will become more important as AID has now been implicated to play an important role in immune tolerance and actual elimination of autoantibodies.
23177218 Aurin tricarboxylic acid self-protects by inhibiting aberrant complement activation at the 2013 May Aberrant complement activation is known to exacerbate the pathology in a spectrum of degenerative diseases of aging. We previously reported that aurin tricarboxylic acid (ATA) is an orally effective agent which prevents formation of the membrane attack complex of complement. It inhibits C9 attachment to tissue bound C5b678 and thus prevents bystander lysis of host cells. In this study, we investigated the effects of ATA on the alternative complement pathway. We found that ATA prevented cleavage of the tissue bound properdin-C3b-Factor B complex into the active C3 convertase enzyme properdin-C3b-Factor Bb. This inhibition was reversed by adding Factor D to the serum. Using enzyme-linked immunosorbent type assays, we established that ATA binds directly to Factor D and C9 but not to properdin or other complement proteins. We conclude that ATA, by inhibiting at two stages of the alternative pathway, might be a particularly effective therapeutic agent in conditions such as macular degeneration, paroxysmal nocturnal hemoglobinemia, and rheumatoid arthritis, in which activation of the alternative complement pathway initiates self damage.
23022892 Peptidylarginine deiminase and protein citrullination in prion diseases: strong evidence o 2013 Jan The post-translational citrullination (deimination) process is mediated by peptidylarginine deiminases (PADs), which convert peptidylarginine into peptidylcitrulline in the presence of high calcium concentrations. Over the past decade, PADs and protein citrullination have been commonly implicated as abnormal pathological features in neurodegeneration and inflammatory responses associated with diseases such as multiple sclerosis, Alzheimer disease and rheumatoid arthritis. Based on this evidence, we investigated the roles of PADs and citrullination in the pathogenesis of prion diseases. Prion diseases (also known as transmissible spongiform encephalopathies) are fatal neurodegenerative diseases that are pathologically well characterized as the accumulation of disease-associated misfolded prion proteins, spongiform changes, glial cell activation and neuronal loss. We previously demonstrated that the upregulation of PAD2, mainly found in reactive astrocytes of infected brains, leads to excessive citrullination, which is correlated with disease progression. Further, we demonstrated that various cytoskeletal and energy metabolism-associated proteins are particularly vulnerable to citrullination. Our recent in vivo and in vitro studies elicited altered functions of enolase as the result of citrullination; these altered functions included reduced enzyme activity, increased protease sensitivity and enhanced plasminogen-binding affinity. These findings suggest that PAD2 and citrullinated proteins may play a key role in the brain pathology of prion diseases. By extension, we believe that abnormal increases in protein citrullination may be strong evidence of neurodegeneration.
22982753 Prostaglandin E2 inhibits IL-23 and IL-12 production by human monocytes through down-regul 2013 Mar The heterodimeric cytokine IL-23 is important for the maintenance of Th17 cells, which are pivotal mediators of autoimmune diseases like rheumatoid arthritis, colitis, and multiple sclerosis. Prostaglandin E2 (PGE2) is a soluble regulator of inflammation that has both pro- and anti-inflammatory properties. PGE2 has been shown to elevate the IL-23 production by dendritic cells (DC). Monocytes are also producers of IL-23 but the effect of PGE2 on IL-23 production by human monocytes has hardly been investigated. We show here that PGE2 blocks the production of IL-23 by LPS-stimulated monocytes in an IL-10 and IL-1β independent manner. This effect was due to the down-regulation of the p40 subunit of IL-23 on mRNA and protein level. The p40 subunit is shared by IL-12 and, consistently, PGE2 also lowered the IL-12 production by monocytes. These effects of PGE2 were cAMP-dependent since the cAMP enhancer forskolin strongly reduced IL-23 and IL-12 production by monocytes. Taken together, PGE2 acts in an anti-inflammatory manner by lowering IL-23 production by monocytes while it has the opposite effect in DC. Our data may help to reconcile controversial point of views on the pro- and anti-inflammatory nature of PGE2 by making a strong case for a cell type-dependent function.
22956345 Use of vitamin D in various disorders. 2013 Mar Approximately 1 billion people worldwide have been identified as vitamin D deficient in the 21st century, and the number is on the rise; non-classical actions of vitamin D were initially recognized around 30 y ago when receptors for vitamin D were detected in neoplastic cells lines. The aim of this review is to provide a brief overview of the non-classical actions of vitamin D. Reports describing the associations of non skeletal actions of vitamin D, especially pertaining to the immune system, inflammatory disorders, cancers and cardiovascular disease have been summarized in this paper. Reports support a role for the active form of vitamin D in mediating normal function of both the innate and adaptive immune systems. Studies also suggest a link between vitamin D deficiency and autoimmune diseases, such as rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus and type 1 diabetes. There is believed to be an inverse association between serum 25-hydroxyvitamin D concentrations and the incidence of colorectal cancer, sporadic colorectal adenoma and breast cancer. Vitamin D deficiency has been linked with various cardiovascular diseases such as hypertension, myocardial infarction, and stroke. Several epidemiological and genetic studies suggest a strong association between vitamin D and non skeletal acute and chronic disorders. However, currently, robust clinical data are still lacking to support raising intake requirements and target vitamin D plasma levels. Nonetheless, the high prevalence of vitamin D deficiency is alarming and requires implementation of clear supplementation guidelines.
22905729 Incorporating gold into nanocrystalline silver dressings reduces grain boundary size and m 2013 Dec Nanocrystalline silver dressings are widely known to be potent antimicrobial and anti-inflammatory agents and have long been used to treat topical wounds. Gold is known to be a strong anti-inflammatory agent and has been used in the treatment of rheumatoid arthritis for >70 years. The purpose of this work was to study the effect of incorporating gold into nanocrystalline silver dressings from antimicrobial and anti-inflammatory perspectives. Gold and silver dressing alloys were created by direct current magnetron sputtering and compared with pure silver nanocrystalline dressings using conventional biological (log reduction and corrected zone of inhibition) and physical (X-ray diffraction, X-ray photoelectron spectroscopy, energy-dispersive X-ray spectroscopy, atomic absorption spectroscopy, atomic force microscopy and scanning electron microscopy) characterisation techniques. While the gold/silver dressings were slightly weaker antimicrobials than the pure silver nanocrystalline structures, the addition of gold to the nanostructure reduces the minimum crystallite size from 17 to 4 nm. This difference increases the number of grain boundary atoms from 12% to 40% which could augment the anti-inflammatory properties of the dressings. The formation of gold oxide (Au2O3) was thought to be responsible for the observed decrease in crystallite size.
24712219 Bilateral pulmonary nodules and mediastinal lymphadenopathy in a patient with Sjogren's sy 2014 Feb Bronchus-associated lymphoid tissue is a normal component of the lung's immune system and may be analogous to gut-associated lymphoid tissue, a form of mucosa-associated lymphoid tissue. Bronchial-associated lymphoid tissue lymphoma is a distinct subgroup of low-grade B-cell extranodal non-Hodgkin lymphoma, classified as marginal-zone lymphoma. It is a rare disorder and appears with a distinct clinical and radiological presentation. We report a case of a patient with a history of Sjogren's syndrome who presented with bilateral pulmonary nodules and mediastinal lymphadenopathy, and who was diagnosed as having bronchus-associated lymphoid tissue lymphoma.
24692530 Increased risks of deep vein thrombosis and pulmonary embolism in Sjögren syndrome: a nat 2014 May OBJECTIVE: Studies of the risks of deep vein thrombosis (DVT) and pulmonary embolism (PE) in patients with Sjögren syndrome (SS) in Asia are scant. We evaluated the effect of SS on the incidences of DVT and PE in a nationwide, population-based cohort in Taiwan. METHODS: We identified patients in Taiwan diagnosed with SS between 1998 and 2008 in the Catastrophic Illness Patient Database and the National Health Insurance Research Database. Each patient with SS was matched to 4 control patients based on age, sex, and index year, and all patients were followed up from the index date to December 31, 2010. We calculated the hazard ratios (HR) and 95% CI of DVT and PE in the SS and comparison cohorts by using Cox proportional hazards regression models. RESULTS: We followed 8920 patients with SS and a comparison cohort of 35,680 for about 50,000 and 200,000 person-years, respectively. The mean age of the SS and comparison cohorts was 53.5 and 53.1 years, respectively, and 88.9% of the patients were women. The risks of DVT and PE among the patients with SS were a 1.83-fold and 3.29-fold greater, respectively, than those for the general population after adjusting for age, sex, comorbidities, and frequency of hospitalization. The patients with a secondary SS had a greater risk of PE (adjusted HR: 5.06; 95% CI: 1.22-21.1) than those with a primary SS (adjusted HR: 3.21; 95% CI: 1.96-5.23). CONCLUSION: Patients with SS have a significantly greater risk of developing DVT or PE than the general population.
24623571 Anaplastic large cell lymphoma involving the urinary bladder: a case report and review of 2015 Jan T cell-derived malignant lymphoma is rarely detected as a bladder neoplasm. A literature review for anaplastic large cell lymphoma (ALCL) involving urinary bladder reveals only seven previously reported cases. Here, we report a case of a 59-year-old HIV-negative man with ALK-positive ALCL. He presented an unusual clinical course with initial consideration of adult onset Still's Disease (AOSD) due to his negative results searching for malignancy and infectious diseases. He rapidly developed macrophage activation (hemophagocytic) syndrome and experienced an unusual rapid disease progression and died in 39 days after onset of symptoms. Compared to previously reported cases, the current case of ALK-1-positive ALCL is a rare case with an unusual presentation. From this case, we learned that ALCL is one malignancy that should be considered and screened in patients with suspected AOSD. Also, T-cell lymphoma associated hemophagocytic syndrome should be considered in a patient with sustained corticosteroid-resistant spike fever, high serum ferritin, and rapid exacerbation of the disease course.