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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four new cases of adult Still's disease have been observed in the past three years. Clinical and biological features are high fever, polyarthralgia, macular rash on forearm, pericarditis, splenomegaly, lymphadenopathy, alopecia, anemia, hyper-leucocytosis, raised ESR. Transient neurological findings may also be observed with abnormal reflexes, cranial nerve paralysis. High dose steroids treatment can be used in case of systemic involvement. The long term prognosis is usually good with treatment. In conclusion, the diagnosis of Still's disease is essentially clinical and should not be mistaken for septicemia.
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PMID:[Still's disease in the adult (author's transl)]. 627 24

Lymphohistiocyte activation syndromes associated with the Epstein-Barr virus and characterized by activation and proliferation of T-cells and macrophages reflecting an inappropriate response of the host to the viral infection. The clinical manifestations are often alarming symptoms suggestive of severe sepsis. Most patients have a known underlying disease (hemopathy, lupus, Still's disease, HIV infection, bone marrow or organ transplantation). In the few cases that occur in the apparent absence of any risk factors, investigations should be done to look for a predisposing inherited disease, such as familial lymphohistiocytosis of Purtilo syndrome in boys. The treatment rests on immunosuppressive agents followed by bone marrow transplantation, which can provide a definitive cure in genetic forms.
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PMID:[Lymphohistiocyte activation syndromes associated with Epstein-Barr virus]. 924 35

In this study we aimed to investigate the findings in patients with adult-onset Still's disease (AOSD) admitted with fever of unknown origin (FUO) during the last 18 years in our unit, in order to discover the ratio of such patients to all patients with FUO during the same period, and to determine the clinical features of AOSD in FUO. The number and the aetiologies of the patients with FUO diagnosed between 1984 and 2001, and the clinical features of those with AOSD, were taken from the patient files. The diagnosis of AOSD was reanalysed according to the diagnostic criteria of Cush et al. [11]. The presumed diagnoses before a diagnosis of AOSD was established were also noted. The chi(2) and Fisher's exact tests were used for statistical analysis. We studied 130 patients with a diagnosis of FUO, 36 (28%) of whom had collagen vascular diseases. Of these 36 patients, 20 (56%, 12 female, 8 male, mean age 34 years, range 16-65) had AOSD. Clinical and laboratory findings were as follows: fever (100%), arthralgia (90%), rash (85%), sore throat (75%), arthritis (65%), myalgia (60%), splenomegaly (40%), hepatomegaly (25%), lymphadenopathy (15%), anaemia (65%), neutrophilic leukocytosis (90%), increased erythrocyte sedimentation rate (100%), elevated transaminase levels (65%), a negative RF (100%), and a negative FANA (80%). Antibiotics had been prescribed in 18 (90%) of cases. The presumed infectious diagnoses were streptococcal tonsillitis/pharyngitis (50%), infective endocarditis (four patients), sepsis (two patients) and acute bacterial meningitis (two patients). The presumed non-infectious diagnoses were acute rheumatic fever (three patients), seronegative rheumatoid arthritis (two patients) and polymyositis (two patients). Sixteen patients were followed for a mean duration of 30 months (range 2-59). A remission was obtained with indomethacin in three cases (19%), and with prednisolone in the remainder. Relapse was detected in three cases (19%). AOSD is one of the most frequent aetiologies of FUO. During the diagnostic course of a patient with FUO, a maculopapular rash and/or arthralgia and/or sore throat should raise the suspicion of AOSD. Because the disease has heterogeneous clinical findings, certain bacterial infections (e.g. streptococcal pharyngitis and sepsis) are generally considered and the prescribing of antibiotics is common.
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PMID:Fever of unknown origin: a review of 20 patients with adult-onset Still's disease. 1274 Jun 70

Lymphohistiocytic activation syndromes are characterized by activation and proliferation of T cells and macrophages usually reflecting an inappropriate response of the host to an infection. The clinical manifestations are often alarming symptoms suggestive of severe sepsis. Most patients have a known underlying disease (hemopathy, lupus, Still's disease, HIV infection, bone marrow or organ transplantation). In the few cases that occur apparently without any risk factors, investigations should be done to look for a predisposing inherited disease such as familial lymphohistiocytosis or Purtilo syndrome in boys. The treatment relies on immunosuppressive agents followed by bone marrow transplantation, which can provide a definitive cure in genetics forms.
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PMID:[Hemophagocytic syndromes and primary immunodeficiencies]. 1476 36

Severe systemic manifestations of adult onset Still's disease (AOSD) are often fatal and occasionally related to hemophagocytic syndrome (HS). We describe the case of a 49-yr-old woman with AOSD presenting with non-remitting high fever, confusion, jaundice, hepatosplenomegaly, serositis, azotemia, pancytopenia, coagulopathy with disseminated intravascular coagulation (DIC), hyperferritinemia, acute acalculous cholecystitis and ileocolitis noted in computed tomographic images. The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain. Although bone marrow examination was not performed due to hemorrhagic diathesis, we suspected AOSD-associated HS on the basis of clinical course without detectable infectious agents in cultures or serologic studies. Intravenous immunoglobulin, pulse methylprednisolone, oral cyclosporine A (CsA) and ceftriaxone brought about transient improvement of fever and confusion, but the disease progressed. After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly. Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.
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PMID:Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A. 1496 57

The authors report three cases of adult-onset Still's disease with severe hypoxemic pulmonary involvement, mimicking severe pulmonary sepsis. Clinicians must be aware of this rare form of such disease. Low (<20%) glycosylated ferritin level in the presence of unexplained prolonged fever with leukocytosis can help in the diagnosis.
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PMID:Acute respiratory failure revealing adult-onset Still's disease: diagnostic value of low glycosylated ferritin level. 1628 19

We report the case of a 60-year-old woman who had recently been examined for fever of unknown origin and who presented to our hospital with high fever and myalgia, weakness, sore throat, and rash. The patient had a markedly elevated serum ferritin concentration of 40,000 ng/mL and positive antinuclear antibodies (ANA) with a titer of 1/200. Despite the presence of positive ANA, the patient was diagnosed as having adult Still's disease (ASD). High-dose steroid therapy resulted in a remarkable clinical improvement. Such a severe case of systemic inflammatory response syndrome, masquerading as sepsis with a positive ANA test, has not been reported previously, at least not in the last 15 years.
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PMID:Adult Still's disease despite the presence of positive antinuclear antibodies. 1733 72

The assessment of systemic inflammation by means of laboratory tests often complements the results of medical examination. Traditionally, the erythrocyte sedimentation rate and leukocytosis with left shift are diagnostic markers for inflammatory and infectious diseases. The levels of acute-phase proteins--especially C-reactive protein--are used to assess both the presence of inflammation and any response to treatment. The determination of C-reactive protein levels may be advised in three types of pathological situation: infection, acute or chronic inflammation, and evaluation of metabolic risk. Procalcitonin is useful as a marker of sepsis and severe infection. The concentration of serum amyloid A predicts the chances of survival of patients with secondary (AA) amyloidosis. Ferritin and its glycosylated form are of interest in the study of specific diseases such as adult-onset Still's disease. Markers of cartilage and bone turnover are complementary to these markers of inflammation. Although cytokine serum levels are transiently crucial to the generation of inflammation, their usefulness in the clinic is still under investigation. Serum concentrations of cytokine inhibitors or soluble cytokine receptors, as well as the clinical response of patients to treatment with cytokine antagonists, might generate important information for monitoring autoinflammatory diseases.
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PMID:Primer: the practical use of biological markers of rheumatic and systemic inflammatory diseases. 1776 50

Adult-onset Still's disease (AOSD) is characterized by fever, rash, and joint pain and may lead to chronic arthritis. The cause of AOSD is unknown, and it is rare. In children, Still's Disease is called systemic juvenile rheumatoid arthritis. We encountered a patient with adult-onset Still's disease following a severe sore throat and fever. The patient was a 17-year-old woman who consulted our hospital because of a sore throat and fever. She was admitted and treated with antibiotics, but the fever persisted. Laboratory parameters of inflammatory activity increased at an accelerated rate, and after ruling out sepsis, EBV-associated disease, and malignant lymphoma, a diagnosis of AOSD was made. Steroid therapy was very effective. When acute pharyngitis is observed in association with significant changes in laboratory parameters despite mild local symptoms, or when pharyngitis is observed in association with joint pain, continuous fever, and a rash, it is important to consider AOSI).
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PMID:[Adult-onset Still's disease following severe sore throat and fever. Case report]. 1826 Mar 1

The role of the potent proinflammatory cytokine IL-1 in disease could clinically be investigated with the development of the IL-1 blocking agent anakinra (Kineret), a recombinant IL-1 receptor antagonist. It was first tested in patients with sepsis without much benefit but was later FDA approved for the treatment of patients with rheumatoid arthritis. More recently IL-1 blocking therapies are used successfully to treat a new group of immune-mediated inflammatory conditions, autoinflammatory diseases. These conditions include rare hereditary fever syndromes and pediatric and adult conditions of Still's disease. Recently the FDA approved two additional longer acting IL-1 blocking agents, for the treatment of cryopyrin-associated periodic syndromes (CAPS), an IL-1 dependent autoinflammatory syndrome. The study of autoinflammatory diseases revealed mechanisms of IL-1 mediated organ damage and provided concepts to a better understanding of the pathogenesis of more common diseases such as gout and Type 2 diabetes which show initial promising results with IL-1 blocking therapy.
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PMID:Blocking interleukin-1 in rheumatic diseases. 2007 80


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