Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0242429 (sore throat)
 2,760 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adult onset Still's disease is a variant of systemic juvenile chronic arthritis in adulthood. The clinical picture is characterized by high spiking fever, arthralgia/arthritis, transient erythema, acute-phase reaction including elevated ESR, CRP and neutrophilia, resembling acute bacterial infections. Hyperferritinaemia and hepatic dysfunction are usually present, and the patients frequently have a sore throat. Extraarticular features, such as splenomegalia, serositis and pericarditis may be parts of this disease as well. Two cases are described, who were admitted to the Department of Internal Medicine of a small Norwegian hospital. Both patients were subjected to exhaustive and laborious investigations for the purpose of disclosing malignancy and/or septicaemia. Following adequate glucocorticoid therapy, both were asymptomatic after less than a week's treatment and after five months' follow-up. Two sets of diagnostic criteria are presented, having different sensitivity, although almost equal specificity. Still's disease in the adult may be an underdiagnosed clinical entity, but should definitely be considered to be a possible differential diagnosis when investigating suspected malignancy, including lymphoma and febrile conditions suspected of septicaemia.
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PMID:[Adult-onset Still's disease. An underdiagnosed condition?]. 853 15

Adult Still's disease is a chronic, systemic disease of unknown origin. We describe the case of an otherwise healthy man with an uncommon presentation of Still's disease. A 38-year-old man presented with sore throat, fever, rash and arthritis. Laboratory findings showed that both erythrocyte sedimentation rate and ferritin had increased. Transoesophageal echocardiography revealed a vegetation involving the aortic leaflet. The diagnosis of Still's disease was made after the exclusion of infectious endocarditis, based upon the clinical picture, the high level of ferritin and the follow-up. The patient markedly improved after treatment with prednisone 1 mg. kg-1. This controlled and then progressively reduced the disease; the drug was then withdrawn. This case illustrates that Still's disease can present with endocardial involvement mimicking acute bacterial endocarditis as a first clinical manifestation. The observation suggests that the presence of high ferritinaemia in a patient with some clinical criteria of Still's disease could lead to an early diagnosis.
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PMID:Atypical presentation of adult Still's disease mimicking acute bacterial endocarditis. 874 17

We describe our experience with 3 cases and a literature review examining whether sore throat is an early manifestation of adult onset Still's disease (AOSD). From our review of 341 cases in the English literature we noted that 69% of all reported patients displayed sore throat early in the disease course. We propose that sore throat be considered an important early diagnostic manifestation of AOSD because it appears useful in identifying patients in their first month of illness.
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PMID:Severe sore throat as a presenting symptom of adult onset Still's disease: a case series and review of the literature. 905 72

This retrospective descriptive study aims to characterise and compare the clinical manifestations, course and outcome of 16 Oriental patients with adult-onset Still's disease diagnosed in the last 4 years with published data based on Western populations and another Oriental (Japanese) series. Like the Japanese, we found a female preponderance, an older age at onset, and fewer patients with abdominal pain, myalgia, sore throat and serositis compared to the Western series. A longer delay in diagnosis occurred in patients lacking either arthritis or rash at presentation. Most patients had mild hyponatraemia and 2 patients had overt syndrome of inappropriate anti-diuretic hormone secretion. All patients showed a dissociation of elevated aldolase with normal to low creatine kinase levels. Over 50% relapsed within a year from diagnosis and needed slow-acting anti-rheumatic drugs as steroid-sparing agents. Two were given intravenous pulse cyclophosphamide therapy for progressive pneumonitis. Outcome was generally good with minimal functional impairment and no mortality.
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PMID:Adult-onset Still's disease in an oriental population: manifestations, course and outcome in 16 patients. 958 68

The clinical manifestations, treatment and course, and articular outcomes of 24 children with juvenile-onset Still's disease (JOSD) and 21 adults with adult-onset Still's disease (AOSD) were compared retrospectively. There was no significant difference in the initial clinical and laboratory manifestations except that more adults presented with a sore throat (81% vs. 46%, p = 0.03). Although serum ferritin was almost always elevated in both diseases, adults had significantly higher serum ferritin concentrations compared with those of children. Steroid treatment was required in 71% of children and 52% of adults, while disease-modifying antirheumatic drugs were used in 42% of children and 24% of adults during the course. Chronic arthritis (>6 months) occurred in comparable proportions of patients with JOSD and AOSD (46% vs 38%, p = 0.82), irrespective of the disease pattern (monocyclic or polycyclic). However, severe deforming arthritis with marked functional limitation occurred only in JOSD, particularly with polyarthritis at disease onset (more than five affected joints). In contrast, AOSD patients with chronic arthritis had a favourable functional outcome at the end of the follow-up. Our study suggested different articular outcomes of Still's disease in Chinese children and adults.
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PMID:Different articular outcomes of Still's disease in Chinese children and adults. 1079 24

A fifteen-year-old boy was admitted to our hospital because of lower abdominal pain, watery diarrhea and mucobloody stool. Two years before admission, he was diagnosed to have Still's disease presenting with polyarthritis, sore throat, remittent fever and typical skin rash. He had been treated with non-steroidal anti-inflammatory agents, oral prednisolone and low-dose methotrexate. Although he was almost free of symptoms during the next two years, serum C-reactive protein (CRP) levels continued to be elevated moderately. He began to complain of lower abdominal pain and loose stool in May 1997 and came down with mucous-bloody diarrhea in June. Laboratory data on admission showed an elevated level of serum CRP (13.9 mg/dl). The biopsy of the stomach, ileum, sigmoid colon and rectum revealed the deposition of amyloid protein of AA type, which confirmed the diagnosis of secondary amyloidosis. The dose of prednisolone was increased and dimethyl sulfoxide per os or rectum was instituted, which improved his gastro-intestinal symptoms to some extent. However, fever, arthritis and diarrhea recurred along with tapered prednisolone dosage. In addition to gastro-intestinal symptoms, arrhythmia and proteinuria appeared. These symptoms were considered to reflect general deposition of amyloid in his body. He is now on immunosuppressive agent and high-dose prednisolone. Several studies report the higher frequency of gamma-allele of SAA 1 gene in the cases of rheumatoid arthritis with AA-amyloidosis than in those without. In the patient presented here, molecular biological analysis revealed that his SAA 1 gene was composed of beta- and gamma-allele. The presence of gamma-allele in his SAA 1 gene might be one of the factors that predisposed him for generalized deposition of amyloid protein in such a short period of time.
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PMID:[Rapidly progressed secondary amyloidosis in a patient with Still's disease with gamma-allele in his SAA 1 gene]. 1092 Jun 89

Empyemas are usually of infectious origin. Noninfectious causes of empyema may be a considerable diagnostic challenge, as exemplified in the present case report. A 21-year-old male presented with high fever, sore throat and myalgias of 1 week duration. In the following days, bilateral pleural effusions developed, with cellular counts in the pleural fluid up to 117 x 10(9)/liter (98% neutrophils). Despite institution of empiric antibiotic therapy, spiking fever continued. All culture studies resulted in being negative. Following the report of a serum ferritin concentration of 6,975 microg/l, adult-onset Still's disease was diagnosed and successfully treated with anti-inflammatory drugs. This case adds adult-onset Still's disease to the list of noninfectious causes of empyema and underlies the value of measuring serum ferritin as a diagnostic tool.
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PMID:Bilateral empyema caused by adult-onset Still's disease. 1122 37

In this study we evaluated the efficacy of Infliximab in the treatment of adult Still's disease (ASD) refractory to conventional therapy. Three patients with chronic and active ASD unresponsive to corticosteroids and methotrexate were given intravenous Infliximab infusions at a dosage of 3 mg/kg at weeks 0, 2, 6 and then once every 8 weeks. Methotrexate was maintained in all cases at a dosage of 15 mg/week, whereas the prednisone dose was modified according to disease activity. The follow-up lasted 50 weeks and disease activity improved in all cases during Infliximab therapy. Two patients presented arthralgias and sore throat at 20 and 28 weeks, that was rapidly controlled by Infliximab reinfusion every 4 weeks. One patient relapsed at 18 weeks and dropped out at 22 weeks due to an urticarioid rash after the beginning of the fifth infusion. Infliximab may be effective in the treatment of relapse of ASD refractory to conventional therapy and requiring continuous high dose corticosteroid medication. Further studies are needed to evaluate the long-term safety, efficacy and the optimal schedule of infusion.
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PMID:Infliximab in the treatment of adult Still's disease refractory to conventional therapy. 1189 94

A 43-year-old woman was admitted for examination of fever, an elevated transaminase level, LDH, skin eruption, sore throat and bicytopenia. As bone marrow examination revealed an increased proportion of histiocytes and active phagocytosis, hemophagocytic syndrome (HPS) was diagnosed. After admission, the peripheral blood counts recovered spontaneously and the HPS subsided, but other symptoms persisted and the neutrophil count increased. At this time, we diagnosed the patient as having adult Still's disease. All the symptoms disappeared after administration of prednisolone. The markedly increased concentrations of TNF-alpha and IFN-gamma in the peripheral blood at the time of HPS declined gradually, and the IL-6 concentration increased at the time of diagnosis of Still's disease. However, all of these concentrations normalized after administration of prednisolone. As HPS and Still's disease have a common etiology, and each shows high concentrations of IFN-gamma, IL 6 and TNF-alpha, the symptoms are similar in both diseases. In particular, a relationship between HPS and high concentrations of TNF-alpha and IFN-gamma is suspected.
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PMID:[Adult-onset Still's disease accompanied by hemophagocytic syndrome at onset]. 1192 84

A 19-year-old woman was admitted because of high fever, rash, arthralgia and sore throat. On physical examination a diffuse skin rash was observed, leaving a facial mask unaffected. C-reactive protein and erythrocyte sedimentation rate were raised (114 mg/l and 26 mm in the first hour, respectively); white blood cell count was normal (6.2 x 10(9)/l) with an increased count of immature forms. An infective, metabolic or haematological cause was excluded. Serum ferritin turned out to be extremely elevated (4318 micrograms/l), so adult-onset Still's disease was diagnosed. The patient fulfilled the criteria of Cush et al. for adult-onset Still's disease. She was first treated with non-steroidal anti-inflammatory drugs (NSAIDs) and, at a later stage in the disease, with corticosteroids. All symptoms disappeared and blood test results normalised.
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PMID:[Clinical thinking and decision making in practice. A young woman with fever, arthralgia and exanthema]. 1235 83


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