Cases reported "Sjogren's Syndrome"

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1/92. Primary sialoangiectasia--a diagnostic pitfall in sjogren's syndrome: case report.

    A case of primary sialoangiectasia, which in this case was initially misdiagnosed as sjogren's syndrome, is described. Other diseases, including hiv infection, psoriatic arthritis, and acute parotitis, may cause glandular changes similar to the changes found in the syndrome. Therefore, sialography must be combined with other methods of assessment of the oral cavity when suspicion is high for sjogren's syndrome. Properly applied, sialography provides essential information regarding the severity of glandular damage and the progression of the disease.
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2/92. Mucosa-associated lymphoid tissue lymphomas in two patients with rheumatoid arthritis on second-line agents, and secondary sjogren's syndrome.

    We report two patients with rheumatoid arthritis and secondary sjogren's syndrome whose disease was complicated by a mucosa-associated lymphoid tissue (MALT) lymphoma. Although this particular type of lymphoma is associated with primary sjogren's syndrome, it has not been described, to our knowledge, in the context of rheumatoid arthritis and sjogren's syndrome. The potential causative factors are discussed.
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3/92. trochlear nerve palsy in sjogren's syndrome.

    sjogren's syndrome (SS) is a systemic lymphoproliferative, autoimmune disease, which is characterized by dryness of the eyes, mouth, and other mucous membranes. The nervous system may be affected in up to 20% of the cases of primary or secondary SS. We present a case of a 54-year-old woman with trochlear nerve palsy complicating sjogren's syndrome secondary to rheumatoid arthritis. We suggest that all patients with multiple cranial neuropathies, especially when associated with rheumatoid arthritis, should be carefully examined for the possible presence of secondary SS.
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4/92. sjogren's syndrome associated with multiple myeloma.

    sjogren's syndrome (SS) is a chronic autoimmune disease of unknown etiology characterized by lymphocytic infiltration of the exocrine glands and a polyclonal B-cell activation; it is demonstrated by the presence of multiple autoantibodies against organ- and non-organ-specific autoantigens. SS is associated with malignant lymphomas, Waldenstrom's macroglobulinemia and benign monoclonal gammopathy, while its relationship with multiple myeloma is extremely rare. The association between multiple myeloma and rheumatoid arthritis and other autoimmune diseases has been established, but it is not clear why a B-cell proliferation like myeloma occurs more rarely than other B-cell disorders in patients with SS. We describe a patient who presented with multiple myeloma and SS that might have existed for at least 2 years prior to the appearance of myeloma.
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5/92. Interstitial pneumonia complicated by sjogren's syndrome, Hashimoto's disease, rheumatoid arthritis and primary biliary cirrhosis.

    A 66-year-old woman diagnosed as having Hashimoto's disease and rheumatoid arthritis manifested interstitial pneumonia. We diagnosed sjogren's syndrome and primary biliary cirrhosis as complications in this case. Steroid therapy was relatively effective for the interstitial pneumonia which was in an active state; however, during tapering of the steroid, there was a relapse and also severe dry throat. cyclophosphamide was added and was effective in the prevention of recurrence. Even after discontinuation of steroid therapy, her general condition is stabilized. It is very important to carefully investigate other organ involvement as a prognostic factor in cases in which there are multiple autoimmune diseases.
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6/92. Rapid improvement of osteomalacia by treatment in a case with sjogren's syndrome, rheumatoid arthritis and renal tubular acidosis type 1.

    We present here a case of sjogren's syndrome (SjS) with osteomalacia based on renal tubular acidosis type 1 (RTA-1). A 53-year-old woman, diagnosed as having rheumatoid arthritis (RA) at the age of 33, was admitted to our hospital because of sicca complex, fatigability and worsening general aching. The activity of RA had been low, but it was complicated by SjS, RTA-1 and remarkable osteomalacia. acidosis was corrected by alkali supplement therapy. By treatment with a regimen consisting of alfacalcidol, calcium L-aspartate, elcatonin and ipriflavone, her bone mineral density (BMD) was remarkably improved within months and the generalized aching gradually diminished.
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7/92. Tuberculous arthritis mimic arthritis of the sjogren's syndrome: findings from sonography, computed tomography and magnetic resonance images.

    A patient with a history of sjogren's syndrome developed chronic arthritis of left ankle. It was diagnosed as arthritis of the sjogren's syndrome initially. However, joint pain persisted despite corticosteroid therapy. Sonography disclosed a multiloculated cystic lesion with peripheral hyperechoic enhancement around left ankle and extended to achilles tendon and subcutaneous region. Computed tomography (CT) confirmed the findings. magnetic resonance imaging (MRI) revealed increased signal intensity of the lesion after gadonillium enhancement on T1-weighted images. These abnormalities showed inhomogenous high signal intensities on T2-weighted images. Tuberculous arthritis was diagnosed by positive synovial tuberculous culture. Sonography is a valuable tool that offers significant advantages for the initial evaluation of arthritis of the sjogren's syndrome and help early suspicious of tuberculous arthritis, because of its cost-effectiveness, superior differentiation between the cyst and solid lesions, convenience for guiding biopsy and drainage.
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8/92. The occurrence of various collagen diseases in one family: a sister with ISSc, PBC, APS, and SS and a brother with systemic lupus erythematosus.

    We encountered siblings who had collagen diseases and related symptoms. Case 1 was a 53-year-old woman who had limited cutaneous systemic sclerosis (ISSc) associated with primary biliary cirrhosis (PBC), antiphospholipid antibody syndrome (APS), and subclinical sjogren's syndrome (SS). Case 2 was a 48-year-old man, her younger brother, with systemic lupus erythematosus (SLE) that developed at 32 years of age. Investigation of their family revealed that their mother had Raynaud's phenomenon, arthritis, and subclinical sjogren's syndrome, and that another younger brother of Cases 1 and 2 had Raynaud's phenomenon and general fatigue. HLA analysis revealed that the sister and brother had some identical hla antigens in common, including A2, A33 (19), B67, B44 (12), Cw7, DR2, DR6, DR52, and DQ1. The sister, brother and their mother had common hla antigens including A2, B67, Cw7, DR2, and DQ1. Although Cases 1 and 2 shared the same HLA system, they presented different phenotypes of collagen disease.
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9/92. An autopsy case of primary biliary cirrhosis with severe interstitial pneumonia.

    A 74-year-old woman was admitted to our hospital because of interstitial pneumonia. She had a 14-year history of primary biliary cirrhosis (PBC) diagnosed histologically, with a positive test for anti-mitochondrial antibodies and elevated biliary enzyme activity. She also had a 7-year history of rheumatoid arthritis and a 26-year history of sjogren's syndrome. Though the symptoms of these complications improved, the interstitial pneumonia deteriorated very quickly and the patient died of respiratory failure due to acute exacerbation of interstitial pneumonia when the activity of PBC decreased. We report this case because it is relatively rare for PBC to be complicated by severe interstitial pneumonia, and it may offer insight into the etiology of these diseases.
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10/92. Co-occurrence of spondyloarthropathy and connective tissue disease: development of sjogren's syndrome and mixed connective tissue disease (MCTD) in a patient with ankylosing spondylitis.

    spondylarthropathies (SpA) and connective tissue diseases (CTD) are clinically distinct entities which, at first glance, seem to have little in common. However, a link between SpA and CTD has recently been suggested by a study in which a higher prevalence of sjogren's syndrome (SS) and sicca symptoms was reported in patients with ankylosing spondylitis (AS) and undifferentiated SpA (1). Another link between SpA and CTD is a possible side effect of a DMARD widely used to treat SpA: sulfasalazine (SAS). SAS was reported to induce antinuclear antibodies (ANA) and systemic lupus erythematosus (SLE)-like syndromes such as drug-induced lupus. This report describes a 54-year-old white male, HLA B27-positive AS patient with some syndesmophytes who, after 15 years of disease, developed SS with salivary gland involvement, Raynaud's syndrome and anti-Ro antibodies. Then, 20 years after the onset of AS, he became acutely ill, suffering severe myositis and myocarditis along with swollen hands and highly elevated autoantibody titers recognizing UIRNP; his condition was interpreted as mixed connective tissue disease (MCTD). The patient had been treated with SAS and azathioprine (AZA) alone several times during the last years because he had not tolerated other DMARDs. A combination of both drugs had been prescribed 3 weeks before a severe flair because of progredient high disease activity with painful peripheral arthritis of the MCP and PIP joints which, however, had not shown radiographic erosions. We describe the rare development of MCTD in an AS patient and report, for the first time, the onset of MCTD potentially triggered by sulfasalazine.
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