Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four adults with an illness similar to the systemic variant of juvenile rheumatoid arthritis seen in children (Still's disease) are described. All four had fever, an erythematous maculopapular rash, splenomegaly and arthritis. The arthritis was asymmetric and involved only a few joints simultaneously. Erosive arthritis developed in one patient. Three patients had a sore throat, two had pleurisy and pericarditis, and one had transient abnormalities of liver function. The laboratory features included anemia, leukocytosis and high leukocyte counts in the synovial fluid. High titres of rubella hemagglutination-inhibiting antibody were detected in two patients, one of whom was found to have rubella virus in the urine. Only one patient responded well to therapy with acetylsalicylic acid; the other three were given prednisone therapy, and two continue to require it.
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PMID:Adult-onset Still's disease. 45 6

Sixteen patients with adult onset Still's disease are reported and compared to 42 previously reported cases. The onset of this illness is sudden and is characterized by quotidian fever, evanescent rash, arthritis, leukocytosis and with variable frequency abnormalities of the liver function tests, adenopathy, splenomegaly and loss of weight. The response to anti-inflammatory therapy is satisfactory and the majority of patients will have a good functional prognosis even though they may require corticosteroids to suppress the signs and symptoms of the disease. It is stressed that Still's disease should be considered one of the diagnostic possibilities in cases of fever of unknown etiology and in cases of seronegative arthritis in adult patients. The pertinent clinical, laboratory, radiological and histological features of this illness are reviewed.
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PMID:[Adult-onset Still's disease]. 55 11

We have attempted to design classification criteria for adult Still's disease by analyzing the data obtained through a multicenter survey of 90 Japanese patients with this disease and of 267 control patients. The proposed criteria consisted of fever, arthralgia, typical rash, and leukocytosis as major, and sore throat, lymphadenopathy and/or splenomegaly, liver dysfunction, and the absence of rheumatoid factor and antinuclear antibody as minor criteria. Requiring 5 or more criteria including 2 or more major criteria yielded 96.2% sensitivity and 92.1% specificity. However, an exclusion process will be needed for an accurate classification, since this disease is relatively rare.
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PMID:Preliminary criteria for classification of adult Still's disease. 838 64

Adult onset Still's disease seems to be the adult form of Still's disease in children. The key symptoms of the disease are high spiking fever, arthritis and a macular or maculopapular, salmon-pink evanescent rash, almost always accompanied by neutrophilic leukocytosis and frequently by sore throat, intense myalgias, lymphadenopathy, splenomegaly and signs of serositis. Tests for IgM rheumatoid factor and antinuclear antibody are characteristically negative. With respect to haematologic abnormalities, the disease may give rise to several problems. First, there is a neutrophilic leukocytosis, which currently is unexplained, and often a normocytic normochromic anaemia, that may be profound. The anaemia has the characteristics of anaemia of chronic inflammatory disease. Both abnormalities disappear after effective treatment of the disease or at spontaneous remission. Secondly, there might be a problem to differentiate AOSD from malignant haematological disorders, including malignant lymphoma and leukaemia, especially when the picture is dominated by lymphadenopathy, splenomegaly, fever and leukocytosis. Although in rare cases the differential diagnosis is extremely difficult, diagnosis can mostly be made or excluded by peripheral blood smear staining, bone marrow biopsies and occasionally lymph node biopsy. Finally, like the juvenile counterpart, AOSD is occasionally complicated by sometimes life-threatening diffuse intravascular coagulation. Factors that might be important in the development of this complication include severe disease activity, liver abnormalities and particular drugs including salicylates, other NSAIDs and some slow-acting antirheumatic drugs. Prompt therapy, including withdrawal of the drug, corticosteroids and sometimes anticoagulant therapy have been successfully applied to most patients.
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PMID:Adult-onset Still's disease. 175 84

Adult-onset Still's disease is characterized by high spiking fever, evanescent maculopapular rash and arthritis. Several recent reports have associated its development with a variety of infectious agents. We describe the case of a 25-year old woman presenting high fever, maculopapular rash and seronegative polyarthritis associated with lymphoadenopathy, splenomegaly and neutrophil leucocytosis together with acute acquired toxoplasmosis. Other causes of systemic illness were excluded by appropriate laboratory, radiological and histological investigations. Clinical, radiological and laboratory findings as well as possible etiopathogenetic correlations among both pathological conditions are discussed. Toxoplasma gondii should be considered as a further possible triggering agent associated with the development of adult-onset Still's disease.
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PMID:Adult-onset Still's disease associated to toxoplasma gondii infection. 179 Jun 46

Still's disease is a seronegative arthritis of children which, in a limited number of cases, can affect adults. The diagnosis of adult-onset Still's disease is characterized by high fever, arthritis and negative serologic tests for rheumatoid factor and antinuclear antibodies and by at least two minor symptoms (leukocytosis, evanescent rash, serositis, hepato- or splenomegaly, and lympho-adenopathy). Since many diseases present analogous manifestations and the adult-onset Still's disease is generally diagnosed by exclusion, we report two patients, aged 26 and 39, with Still's disease, the former with a classic clinical feature, the latter with a clinical feature characterized by severe hepatic abnormalities. The determination of histocompatibility antigens can be useful because some of them (HLA-DR4 in case 1 and HLA-DRw6 in case 2) are frequently associated with the adult-onset Still's disease. The role of anti-inflammatory therapy (acetylsalicylic acid, indomethacin, steroids) must be emphasized, whose efficacy can constitute the pathognomonic element on which the diagnosis of adult-onset Still's disease can be based in a proper clinical pattern.
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PMID:[Still's disease in adults: diagnostic problems]. 224 94

The clinical and laboratory features, treatment, prognosis, complications and disability of 45 patients with adult-onset Still's disease were studied. Sixty per cent of the patients were female. Median age at onset was 25 years and median observation period after onset was 41 months. For 28 patients detailed data were available of the first month of illness. In only 43 per cent of these did the disease present with the classical triad of fever, arthritis and rash, although in the whole group eventually, fever with temperatures of 40 degrees C or more occurred in 84 per cent, arthritis in 98 per cent, and the typical rash in 82 per cent. In 32 per cent of the patients with rash, this was pruritic. Other features seen frequently were: lymphadenopathy (71 per cent), splenomegaly (36 per cent), pleuritis and/or pneumonitis (31 per cent), pericarditis (22 per cent), leucocytosis (98 per cent) and hepatic abnormalities (84 per cent). The high spiking fever was reduced to normal in six of 21 cases (29 per cent) by aspirin, in 19 of 27 cases (70 per cent) by indomethacin and in six of seven cases (86 per cent) by naproxen. In 16 of 21 cases (76 per cent) glucocorticoids reduced the systemic and/or joint symptoms. In three patients who remained febrile on glucocorticoids, indomethacin reduced temperature to normal. Eight patients whose joint disease improved on steroids later developed severe joint destructions. Thirteen patients received one or several slow-acting antirheumatic drugs. Only in eight of 18 trials (44 per cent) with one of these drugs did symptoms and signs improve. Fifty-one per cent of the 45 patients had self-limiting disease and 49 per cent had persistent disease with continuous activity for at least one year. At the time of evaluation 47 per cent of the 45 patients were in remission without medication, 33 per cent were in remission while on medication and 20 per cent had active disease. Three life-threatening complications occurred: two patients developed signs of cardiac tamponade and one almost died from diffuse intravascular coagulation. Disability was determined primarily by the course of the arthritis. At the time of review 43 per cent of the 45 patients had no joint destruction (Group 1), 24 per cent had destruction of at least one joint, but had no evidence of disease of the root joints (Group 2) and 33 per cent had destruction in at least one root joint (Group 3).(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Adult-onset Still's disease; clinical and laboratory features, treatment and progress of 45 cases. 365 48

Two patients with the first symptoms of adult onset Still's disease in their 7th decade are reported. In both cases their advanced age was a main reason for a prolonged delay in diagnosis. Besides the characteristic fever pattern, rash and arthritis, our patients showed other typical features of Still's disease such as splenomegaly, pleuritis, pneumonitis, carpal ankylosis and leukocytosis. In addition both showed marked hepatic abnormalities which seemed to be due to the disease itself in one case and at least in part related to exposure to phenylbutazone and diclofenac in the other.
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PMID:Adult onset Still's disease in the elderly: a report of two cases. 405 3

The study of 36 published cases of adult Still's disease shows the following features: the onset takes place usually during the third decade of life, most often as a polyarthralgia characterized by sometimes starting fever, evanescent rash, neutrophil leukocytosis, negativity of the serological tests for rheumatoid arthritis and systemic lupus; lymphadenopathy and splenomegaly are frequent but not constant; serous cavities, particularly pericardium, are rather frequently involved; evolution is characterized by a succession of relapses and remissions, the far prognosis being rather good (1/3 of cures, 1/3 of mild articular relapses, 1/3 of articular sequelae, involving chiefly neck and hips); corticosteroids and heavy doses of aspirin seem to give the best therapeutic results, the usefulness of a maintenance treatment is debatable.
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PMID:[Adult Still's disease (author's transl)]. 625 65

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


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