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)

Sarcoidosis is a granulomatous multisystemic disorder, of unknown origin, that commonly affects young adults between 20-40 years of age. The disease usually manifests itself with changes in the chest which are radiologically visible in 90% of the patients in the form of bilateral hilar lymphadenopathy to interstitial infiltrates in the lungs and bronchi. Once the diagnosis of sarcoidosis has been established, the next step is to evaluate the activity and stadium of the disease. These activity markers include clinical, biochemical and immunological parameters. The clinical criteria include symptoms which indicate the clinical manifestation of sarcoidosis, as well as symptoms which are of prognostic importance for the further course of the disease: dry cough, dyspnea, erythema nodosum, posterior uveitis, polyarthralgia, myopathy, cardiac, renal or nervous system involvement, lymphadenopathy, skin lesions, splenomegaly, enlarged parotid and lacrimal glands, changes in chest x-ray and changes in pulmonary function tests. Biological criteria: biochemical markers in serum which are related to: macrophage and epithelioid cell activity, to lymphocyte activity, to granuloma activity and to collagen metabolism alterations; isotopic markers--67 gallium scan and cellular and soluble components in bronchoalveolar lavage fluid (BAL).
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PMID:[Sarcoidosis activity markers]. 180 91

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

A 24-year-old female had juvenile rheumatoid arthritis since the age of 3, with systemic manifestations such as fever, polyarthralgia, micrognathia, splenomegaly, hepatomegaly, lymphadenopathy, subcutaneous nodules, hidden rheumatoid factor of IgG type. Particularly noteworthy is selective IgA deficiency in this patient, with increases in concentration up to 50 mg/dl in the serum concurrently with the repeated febrile attacks in the clinical course.
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PMID:Juvenile rheumatoid arthritis with IgA deficiency and appearance with febrile episode. 697 20

The conditions of five adults were eventually diagnosed as juvenile rheumatoid arthritis (Still's disease). Prolonged hospitalization, repetitive roentogenographic examinations, biopsies, laparotomies, and therapeutic trials with toxic agents preceded the establishment of the final diagnosis. Common early findings in all cases were prolonged "septic fever," polyarthralgia, and an elevated ESR. Three patients had a rash, four had splenomegaly, three had a vague history of a similar disease, and four had leukocytosis. Some of the patients were older than others described in the literature. Two received immunosuppressive agents and did relatively well. In view of our experience and the few reports in the literature, we concluded that juvenile rheumatoid arthritis in adults has to be seriously considered in the presence of prolonged septic fever, polyarthralgia, rash, and splenomegaly, before harmful drugs are given or risky procedures are performed.
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PMID:An adult form of juvenile rheumatoid arthritis. 739 11

A twelve-years-old boy developed fever, pharyngitis and acute migrant polyarthralgia. An increasing ASO titre was observed, so that rheumatic fever was firstly diagnosed. Finally splenomegaly and positive IgG and IgM against Epstein Barr virus led to the diagnosis of acute EBV infection with polyarthritis. EBV infection should be considered into the differential diagnosis of migrant polyarthritis.
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PMID:[Migrant polyarthritis and EBV infection]. 931 50

The authors report 5 cases of Still's disease in adults whose symptoms were mainly characterised by high fever, transient exanthema, polyarthralgia and/or polyarthritis, lymphoadenomegaly, splenomegaly and neutrophil leukocytosis. Assays for leukocytosis were positive, as were those for inflammatory markers and serum ferritin was also high in all 3 patients in which it assayed. On the contrary, serum ferritin latex test, Waaler-Rose reaction and all other tests commonly used to diagnose long-term fevers were all negative. All the subjects examined recovered after prolonged steroid therapy. Only one patient reported severe sequelae in the hip joints and subsequently underwent bilateral hip replacement surgery.
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PMID:[Adult-onset Still's disease. Report of 5 cases]. 1051 58

We describe a case of nodular sclerosing Hodgkin's disease (NSHD) developing in a 61-year-old woman with seropositive rheumatoid arthritis treated with oral methotrexate (MTX) 5 to 15 mg/week for 5 years. Computed tomography (CT) of the abdomen revealed splenomegaly and marked abdominal and retroperitoneal lymphadenopathy. MTX was discontinued; several weeks later prednisone 10 mg/day was added to control worsening polyarthralgia. Repeat CT at 3 months showed almost complete regression of the splenomegaly and lymphadenopathy. However, CT studies at 10 months showed asymptomatic progression of lymphadenopathy, which on biopsy revealed NSHD. Patients with apparently reversible MTX associated lymphoproliferative disorder require periodic monitoring for asymptomatic development of malignant lymphoma.
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PMID:Reversible methotrexate associated lymphoproliferative disease evolving into Hodgkin's disease. 1074 30

A 23-year-old man, admitted because of high fever, polyarthralgia, butterfly rash and chest pain, was diagnosed as systemic lupus erythematosus (SLE) from the findings of positive antinuclear antibody and anti-DNA antibody. He was treated with 60 mg prednisolone daily, but as reducing the dose, white blood cell counts and platelet counts were decreased and fever, polyarthralgia, decrease of complements, increase of ferritin, hepato-splenomegaly and liver dysfunction were observed. Bone marrow specimen revealed phagocytosis of blood cells by histiocytes and he was diagnosed as hemophagocytic syndrome(HPS) due to active SLE. Methylprednisolone pulse therapy was effective temporarily, HPS recurred while reducing steroid, and cyclosporin was added. After a temporary remission, marked extensive swelling in the face appeared suddenly. Facial skin biopsy showed necrosis of fat cells and hemophagocytosis by histiocytes. Accordingly, he was diagnosed as panniculitis due to HPS and was treated successfully with intravenous cyclophosphamide pulse therapy and high dose of gammaglobulin. Several cases of HPS due to SLE have been reported recently, but this is a rare case of cytophagic histiocytic panniculitis (CHP) due to SLE.
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PMID:[A case of systemic lupus erythematosus with hemophagocytic syndrome and cytophagic histiocytic panniculitis]. 1129 53

Adult-onset Still disease (AOSD) is a systemic inflammatory disorder characterized clinically by high spiking fever, polyarthralgia/arthritis, a salmon-pink evanescent rash, predominantly neutrophilic leucocytosis, lymphadenopathy, liver dysfunction, and splenomegaly. Recently, a nonclassic, nonevanescent skin rash has been reported. We report a 27-year-old woman with AOSD showing persistent pruritic papular lesions. Histologically, dyskeratotic keratinocytes were seen in the upper epidermis. We describe this case in detail and review the previous literature. Nonclassic pruritic eruptions with characteristic dyskeratotic keratinocytes might provide an important clue for the diagnosis of AOSD.
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PMID:Adult-onset Still disease with peculiar persistent plaques and papules. 2477 96


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