Cases reported "Sjogren's Syndrome"

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1/3. sjogren's syndrome presenting as remitting seronegative symmetric synovitis with pitting edema (RS3PE).

    Remitting seronegative symmetric synovitis with pitting edema (RS3PE) syndrome is characterized by symmetrical and acute synovitis, pitting edema, the absence of rheumatoid factor, increased acute phase reactants, lack of bony erosions on radiography, and benign and short clinical course. Half of all patients with sjogren's syndrome experience arthritis during the disease course. We here describe the first case of sjogren's syndrome presenting as RS3PE. She had swelling in knees, ankles, and wrists. After then the swelling spread to her lower legs, feet, face, and both hands. She was admitted to another hospital and was suspected of lupus or rheumatoid arthritis. Three months later, she had dry mouth and had lower lip biopsy. She was admitted to this hospital due to development of swelling in face and lower legs for 3 days. On physical examination, she had pitting edema in both hands and feet dorsum. Laboratory test showed elevated erythrocyte sedimentation rate, positivity of rheumatoid factor, anti-nuclear antibody, and anti-Ro antibody. There was no erosion in the hands radiography. Schirmer's test and lip biopsy was compatible with sjogren's syndrome. She was diagnosed RS3PE and sjogren's syndrome. She was begun with prednisolone and her symptoms improved gradually.
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2/3. Benign hypergammaglobulinemic purpura of Waldenstrom associated with sjogren's syndrome. Case report and review of immunologic aspects.

    Since its first description, fewer than 150 cases of benign hypergammaglobulinemic purpura of Waldestrom have been reported. There is a preponderance of females with this disorder, which is characterized by long-standing purpuric vasculitic lesions usually in the lower extremities, increased sedimentation rate, anemia, leukopenia, and hyperglobulinemia with a normal clotting process. Numerous associations have been shown between this and other systemic disorders such as sjogren's syndrome, systemic lupus erythematosus, a rheumatoid arthritis. A 40-year-old woman is described who had an 18-year history of recurrent purpura and increased IgG levels. skin biopsy showed acute vasculitis, and immunofluorescent study revealed fibrinogen precipitation and C3 deposition. Serologic evaluation demonstrated the presence of rheumatoid factor and antinuclear antibodies (1:80). Raji assay showed increased circulating immune complexes, and the T cell subsets were normal. The purpura was associated with symptoms and physical findings of sjogren's syndrome. On the basis of serologic and skin biopsy findings, an autoimmune origin of the disease is postulated.
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3/3. Severe, chronic anorexia and extensive leg ulcerations as presenting signs of primary sjogren's syndrome.

    We report the case of a 75-year-old woman with a 15-year history of inappetance resulting in weight loss of approximately 40 kg. On physical examination, the skin of the lower extremities was markedly hyperpigmented with a brown-greyish hue. In addition, the skin of the legs was infiltrated, erythematous, riddled with erosions and necrotic ulcers. Clinical and laboratory evaluation revealed sicca syndrome, a pronounced polyclonal hypergammaglobulinemia (60 g/l), high levels of antinuclear, anti-SSA and anti-SSB antibodies. Histological examination of involved skin demonstrated a leukocytoclastic vasculitis.
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