Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adult-onset Still' s disease (AOSD) is an uncommon rheumatic disease characterized by high spiking fever, arthritis, an evanescent skin rash and variety of systemic symptoms, though initial onset of pleuropulmonary manifestation is relatively rare and could be responsible for a delay in diagnosis. We report a case of AOSD presenting with pleuritis with concomitant pericardial effusion. A 42-year-old Japanese man was admitted with a spiking fever of 40 degrees C, hyperleucocytosis (21.6 x 10(9)/l), and a high titer of C-reactive protein (16.84mg/dl). Chest X-ray film and computed tomography showed bilateral pleural effusion and massive pericardial effusion which both required tube drainage. Analyses of fluids revealed that both were exudative and sterile, and pleural biopsy showed nonspecific inflammation with mild fibrosis. Neither antibiotics nor antituberculosis drugs were effective. Rash, hepatosplenomegaly, polyarthritis, pharyngitis and right hypochondralgia were accompanied by serum hyperferritinemia. After exclusion of the possibility of infection, other connective tissue disease and malignancy, a diagnosis of AOSD was made. Improvement was not observed with nonsteroidal anti-inflammatory drug and corticosteroid therapy. Double filtration plasmapheresis (DFPP) following steroid pulse therapy alleviated the symptoms and the laboratory data immediately and corticosteroids could be tapered. DFPP is a safe therapeutic procedure and can be an alternative for refractory AOSD.
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PMID:[A case of severe adult-onset Still' s disease presenting with pleuropericarditis]. 1678 97

Adult Still disease is characterized by high fever, arthritis, leukocytosis, hepatosplenomegaly, rash, and lymphadenopathy. It affects primarily young adults and is often disabling or even fatal. Many patients achieve long-term remissions, while others may develop a chronic illness refractory to multiple therapies. Polyarthritis or root joint involvement has been identified as a marker of chronic disease. No controlled studies on second-line agents have been published, but anecdotal studies suggest efficacy of a variety of agents including hydroxychloroquine, sulfasalazine, penicillamine, and methotrexate. Although these therapies have been used, optimal treatment of patients with resistant disease remains problematic. High levels of tumor necrosis factor have been noted, and recent report indicates etanercept may be an effective therapy. We report 2 cases of adult-onset Still disease resistant to NSAIDs, steroids, and methotrexate that have been successfully maintained on 5 mg/kg infliximab for 30 and 29 months, respectively, with improvement of signs and symptoms despite a reduction of steroid dosage. In patients with resistant disease, infliximab may be a safe, effective agent. Its use in this disease merits further study.
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PMID:Treatment of resistant adult still disease with infliximab: a report of 2 cases. 1704 13

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and outcome of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/microl. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values (>500 ng/ml). The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria.
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PMID:Adult-onset Still's disease: a report of 28 cases and review of the literature. 1875 76

Adult onset still's disease usually presents with high grade intermittent fever, polyarthritis, salmon pink evanescent rash and hepatosplenomegaly. Pulmonary involvement in the form of pneumonitis, as a presenting feature is very rare. We report a case of a young lady who presented with fever, cough and respiratory distress. Chest X-ray revealed patchy infiltration in left upper lung zone. She was subsequently diagnosed as Adult onset Still's disease. There was no improvement in clinical condition despite five days of antibiotics. On trans-bronchial lung biopsy (TBLB) proved she had interstitial pneumonitis and responded dramatically to steroid treatment.
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PMID:An Unusual Presentation of Adult Onset Still's Disease. 2377 Aug 76

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of peripheral T-cell lymphoma (PTCL). AITL typically presents with lymphadenopathy, fever, rash, hepatosplenomegaly, and rarely polyarthritis. We report the case of a 50-year-old female who presented with lymphadenopathy, rash, and symmetric polyarthritis. She was later diagnosed with AITL and was treated with chemotherapy with resolution of arthritis. AITL should be suspected in paitents presenting with rheumatoid-like arthritis and diffuse lymphadenopathy.
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PMID:Angioimmunoblastic T-Cell Lymphoma with Polyarthritis Resembling Rheumatoid Arthritis. 2818 40

Extranodal involvement in Kikuchi's disease is uncommon. A 31-year-old previously healthy Indian woman was admitted with high grade fever, multiple joint pain and skin rash for 3 weeks. She had negative anti-nuclear antibodies and had features of Kikuchi's disease on lymph node biopsy. She also had multiple extranodal manifestations including erythematous maculopapular rash, symmetric polyarthritis and hepatosplenomegaly. Kikuchi's disease with extranodal involvement can clinically mimic diseases like hematological malignancies, connective tissue disorders and certain infections. A lymph node biopsy plays a crucial role in making an accurate diagnosis by excluding other diseases. A discussion on the importance of differentiating Kikuchi's disease from systemic lupus erythematosus is included.
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PMID:Kikuchi-Fujimoto disease with multiple extra-nodal features - A clinical mimic. 3130 84


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