Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Still's disease was reported to be a type of Juvenile Rheumatoid Arthritis (JRA) by Still in 1897. Adult-onset Still's disease is an important clinical entity inducing fever, skin rash and polyarthritis. Spiking fever and rash are characteristic features for early diagnosis. Although chronic polyarthritis is similar to RA, ankylosis of hand joint is characteristic for Still's disease rather than destructive change. Increased ESR, negative autoantibodies, leukocytosis, liver dysfunction and hyperferritinemja are major laboratory findings. A markedly increased level of serum ferritin can be used, not only as an indicator of disease activity, but also as a diagnostic marker of the disease. For therapy, a moderate dose of steroid is the most effective.
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PMID:[Adult Still's disease]. 158 58

Adult-onset Still's disease (AOSD) is a rare syndrome of unknown origin consisting of recurrent fever, urticarial rash, arthralgias or arthritides, lymphadenopathia, splenomegalia and nonspecific sore throat. It is always linked with leucocytosis and negative blood cultures; rheumatic and antinuclear factors are mostly negative. Very high ferritin levels are frequent and could be a marker of the disease. We report on two cases and give a review of the literature.
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PMID:[Still's syndrome in the adult (adult-onset Still's disease). 2 case reports and review of the literature]. 800 40

Adult-onset Still's disease, the adult variant of the systemic form of juvenile arthritis, is an uncommon systemic inflammatory disorder of unknown etiology characterized by high spiking fevers, neutrophilic leukocytosis, arthritis, and an evanescent rash. There is often a delay in reaching a firm diagnosis. Differential diagnoses include infection, malignancy, and various immunologic disorders. Increased ferritin levels are of particular value in establishing the diagnosis. Clinical response to high-dose corticosteroids may be dramatic. We report a case of a 29-year-old woman who had recently been investigated for fever of unknown origin, and who presented to our hospital with high fever and hypotension. Her condition rapidly deteriorated with the development of ARDS, disseminated intravascular coagulation, and shock. The patient had a markedly elevated serum ferritin concentration of 26,000 ng/mL. High-dose pulse methylprednisolone therapy resulted in a remarkable clinical improvement. Such a severe case of systemic inflammatory response syndrome, masquerading as septic shock, has not been reported previously.
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PMID:Severe systemic inflammatory response syndrome with shock and ARDS resulting from Still's disease: clinical response with high-dose pulse methylprednisolone therapy. 1037 76

In three patients, two women aged 70 and 19 years and a man aged 33 years with long-lasting fever no diagnosis was made after extensive diagnostic work-up. After exclusion of infectious, malignant and rheumatic diseases, adult-onset Still's disease was diagnosed in all three patients on the basis of clinical and laboratory criteria. Adult-onset Still's disease is an important but less well known cause of fever. Clinically, adult-onset Still's disease is characterized by the triad of fever, skin rash and arthritis/arthralgia. A greatly elevated serum ferritin level proved to be an additional valuable diagnostic clue. Treatment consists of non-steroidal anti-inflammatory drugs, corticosteroids or immunosuppressive agents. The long-term prognosis is usually good, but severe joint destruction may occur. All three patients recovered.
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PMID:[Fever of unknown origin caused by the adult form of Still's disease]. 1092 51

Adult-onset Still's disease (AOSD) is characterized by a spiking fever and diverse clinical findings; the diagnosis is often delayed for months or even years. The only positive laboratory finding is neutrophilic leukocytosis. Since 1987, the diagnostic importance of elevated serum ferritin levels has been discussed in numerous papers, but is not yet among the diagnostic criteria for AOSD. We describe a case in which the finding of extreme hyperferritinemia enabled prompt diagnosis and treatment.
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PMID:[Ferritin levels in adult Still's disease]. 1095 21

Adult-onset Still's disease (AOSD) is a rare disorder of unknown aetiology, characterised by high spiking fever, an evanescent, erythematous, maculopapular rash, arthralgia or arthritis, lymphadenopathy, hepatosplenomegaly, sore throat and serositis. It is associated with marked leukocytosis, high erythrocyte sedimentation rate, increased level of serum ferritin and negative rheumatoid factor and antinuclear antibody tests. Here we report a patient in whom an urticaria-like rash was an uncommon presenting clinical feature of AOSD. To our knowledge, this association has only been reported once before.
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PMID:Urticaria as a presenting manifestation of adult-onset Still's disease. 1105 30

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology characterized by an evanescent rash, intermittent spiking high fever, arthralgia, and a variety of systemic features. We describe a 46-yr-old woman with Still s disease who presented with a 2-month history of a symmetrical vesiculopustular eruptions only on both hands and feet. Skin biopsy specimens of both vesicle and pustule revealed fibrin thrombi deposition in the small dermal vessels with little inflammation, subepidermal bulla, and ischemic necrosis of the overlying epidermis, which were consistent with a vasculopathic reaction. Her skin lesions gradually disappeared in response to conventional AOSD therapy, as elevated serum ferritin levels, an index of disease activity, decreased. To the best of our knowledge, this is the first case of vesiculopustular lesions as a skin manifestation of AOSD.
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PMID:Adult-onset Still's disease with vesiculopustules on the hands and feet. 1248 16

Adult onset Still's disease (AOSD) is a rheumatoid disorder characterized by polyarthritis, intermittent high fever, and salmon colored rashes. Liver dysfunction is usually mild and fulminant liver failure is rare. We describe a 20-year-old woman with AOSD and severe hepatic necrosis leading to hepatic failure requiring liver transplant. This severe liver disorder developed after decreases in fever, arthritis, and C-reactive protein. Interleukin 18 (IL-18), but not ferritin, increased in association with liver dysfunction. Hepatocyte growth factor (HGF) increased at the time of hepatic failure. IL-18 and HGF elevation may have contributed to this rare severe liver injury in AOSD.
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PMID:Interleukin 18 and hepatocyte growth factor in fulminant hepatic failure of adult onset Still's disease. 1273 13

A TRIAD OF FEATURES: Adult onset Still's disease (ASD) is an uncommon disorder usually associating high spiking fever, evanescent skin rash constituted of small salmon pink macules, and arthritis. NUMEROUS SYSTEMIC MANIFESTATIONS: A sore throat is common and often misleading. More than 60% of the patients develop mobile and indolent lymph nodes, usually in the cervical area. Liver involvement is common and usually limited to a mild or moderate cytolysis. However, several observations of severe hepatitis have been reported justifying strict monitoring of the liver biology in these patients. Amongst the other numerous systemic manifestations that have been reported, pericarditis is common and sometimes responsible for tamponade, the pulmonary involvement may lead to an acute respiratory distress, and the rare neurological manifestations include aseptic meningitis or cranial nerve palsy. FROM A BIOLOGICAL POINT OF VIEW: The sedimentation rate is consistently elevated and there is usually a marked elevation in the polymorphonuclears. The bacteriological survey is negative as are the immunological tests. An increase in the serum level of IL-18 might be both diagnostic and prognostic. It is the increase of the serum level of ferritin and the marked decrease in its glycosylated fraction below 20% that seem to be of more potent diagnostic value.
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PMID:[Clinical and biological manifestations of adult-onset Still's disease]. 1552 51

The subject was a 22-year-old woman who developed high fever and arthralgias and eruptions in the extremities around June 2005. She sought medical advice at a nearby dermatology clinic, where hepatic dysfunction was noted on blood testing. The patient was thus hospitalized the next day. Although CRP levels were significantly high, no sign of infection was observed and bone marrow cell differentiation was normal. Adult onset Still's disease was diagnosed based on the observation of persistent high fever >39 degrees C, eruptions, increased leukocytes, pharyngeal pain, splenomegaly, hepatic dysfunction, negative autoantibody results from blood testing, and high serum ferritin levels. Administration of prednisolone 30 mg/day was initiated, but proved ineffective. Steroid pulse therapy was conducted, and the subject was transferred to our medical facility for continued treatment. Attempts were made to control the disease using combined steroid and cyclosporine administration; but exacerbation of high serum ferritin levels and hepatic dysfunctions were observed, so a second course of steroid pulse therapy was conducted. Symptoms improved temporarily, but steroid levels were difficult to reduce. Cyclosporine was therefore replaced by methotrexate, and administration of infliximab was initiated. In the course of treatment, administration of a sulfamethoxazole/trimethoprim combination was initiated, but was discontinued due to suspicion of drug-induced hepatic injury. A second administration of infliximab was conducted in late August, and rapid improvements in clinical symptoms and abnormal test values was observed. However, high fever and headache developed suddenly in early September. Based on the results of spinal fluid testing, blood and spinal fluid cultures and MRI of the head, Listeria meningoencephalitis was diagnosed. Diplopia and impaired consciousness occurred during the disease course, and formation of a brain abscess was observed on imaging. However, symptoms were controlled by long-term combination administration of ampicillin and gentamicin. Administration of infliximab was discontinued for treatment of adult onset Still's disease, and steroid levels were reduced following double-membrane filtration plasma exchange. On follow-up, no relapse of symptoms or abnormalities in blood test values were observed, so the subject was discharged from our medical facility in December 2005. In treatment for rheumatic diseases, a dramatic improvement in treatment results for pathologies displaying tolerance against conventional treatments has been acquired with the development of biological drugs. However, opportunistic infections represent a serious problem, and appropriate preventative measures are required. The present report describes a case in which the subject was affected by Listeria meningoencephalitis during administration of infliximab for steroid-dependent adult Still's disease. Since listeriosis is one of the complications, along with tuberculosis, that warrants precautionary measures, this case is reported and discussed.
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PMID:[A case in which the subject was affected by Listeia meningoencephalitis during administration of infliximab for steroid-dependent adult onset Still's disease]. 1681 65


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