Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P14784 (IL-2 receptor)
 3,849 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied clinical manifestations, laboratory results, treatment, recurrence, and complications in 69 patients diagnosed with Kikuchi's disease by lymph node pathology from January 1, 1998, to December 31, 2007. Subjects were 34 men and 35 women (median age: 28 years, range: 12 to 58 years). Of the 69, 67 were Japanese and 2 were Korean. Major clinical symptoms and signs were fever >37 degrees (71%) and lymphadenopathy (100%). Lymphadenopathy was cervical in 93% and systemic in 3%. Some experienced night sweats and weight loss. The median white blood cell count was 3800 microL (1,700-9,300 microl), and 50.7% of subjects had leukocytopenia (<4,000 microL). The median serum LDH concentration was 245U/L (129-923 U/L). The median ferritin concentration, measured in 26 cases, was 769 ng/mL (4.5-2,580 ng/mL). The median concentration of soluble IL-2 receptor, measured in 27 cases, was 639 U/mL (0.5-4,000 U/ml). Having observed several cases with abnormally high ferritin and soluble IL2 receptor, we note the importance of carefully considering differential diagnosis from Still's disease and malignant lymphoma. Treatment included no medication in 30% of subjects, nonsteroidal anti-inflammatory drugs alone in 37.7%, steroids alone in 7%, and combined nonsteroidal anti-inflammatory drugs and steroids in 22%. Of the 29% administered steroids, we mostly used prednisolone (0.5-1.0 mg/kg), tapering the dose as clinical features improved. Two developed aseptic meningitis and 2 systemic lupus erythematosus. In total, 75% improved in less than 3 months, whereas 6% showed improvement only after at least 6 months of continued treatment. All were cured, but the condition recurred in 8%. Recurrence was not associated initial the disease duration. Recurrence cannot be predicted but can occur. In some cases, we could not distinguish lymphadenitis from malignant lymphoma or tuberculous lymphadenitis based on clinical features or laboratory data. Diagnosis must thus be based on lymph node pathology. We also must consider the possibility of recurrence or attacks of systemic lupus erythematosus.
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PMID:[Medical study of 69 cases diagnosed as Kikuchi's disease]. 1969 71

To investigate the characteristics of patients with adult-onset Still's disease (AOSD), serum cytokines and chemokines were measured to examine their associations with systemic manifestations of AOSD, especially hemophagocytic syndrome (HPS). Nineteen patients diagnosed with AOSD were enrolled. Serial serum samples were obtained from patients with AOSD in both active and inactive stages and controls. The concentrations of cytokines and chemokines, including IL-18, soluble IL-2 receptor (sIL-2R), CX3CL1, CXCL8, CXCL10, CCL2, and CCL3, were determined using enzyme-linked immunosorbent assay. Multivariate analysis was used to evaluate the correlations among serum chemokine levels, disease activity, and the clinical features of AOSD. Significantly higher serum levels of all cytokines and chemokines were observed in patients with active, untreated AOSD than in controls. The level of CX3CL1, but not other chemokines, was elevated in AOSD patients and was positively correlated with clinical activity and the levels of CRP, ferritin, IL-18, and sIL-2R. Among the 19 patients with AOSD, four patients also had HPS. Serum CX3CL1 and ferritin were significantly higher in AOSD patients with HPS than in those without HPS. The serum CX3CL1 level may be used as a clinical marker to assess the disease activity of AOSD, and high serum CX3CL1 and ferritin in patients with AOSD reflected the presence of HPS. The association between the chemokine profile and distinct clinical manifestations or various patterns of disease progression indicates that the pathogenesis of AOSD is heterogeneous.
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PMID:Correlation of serum CX3CL1 level with disease activity in adult-onset Still's disease and significant involvement in hemophagocytic syndrome. 2232 7

Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still's disease (Still's disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still's disease, whether it is therapeutic for MAS associated with Still's disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation. Striking hyperferritinemia was noted without evidence of infection, malignancy, or hemophagocytosis on bone marrow biopsy. NK cells were undetectable in the peripheral blood, whereas soluble IL-2 receptor was elevated. His multiorgan disease resolved in association with methylprednisolone pulse therapy, Anakinra, and a tapering course of prednisone. This case reinforces the notion that Still's disease is inherently poised to manifest MAS as one of the clinical phenotypes by shedding light on the role of IL-1 underlying both Still's disease and related MAS.
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PMID:Macrophage Activation Syndrome Associated with Adult-Onset Still's Disease Successfully Treated with Anakinra. 2781 26