Cases reported "Sjogren's Syndrome"

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1/109. Parkinsonism associated with sjogren's syndrome: three cases and a review of the literature.

    sjogren's syndrome (SS) is a common multisystem autoimmune disorder. As with other autoimmune disorders such as systemic lupus erythematosus (SLE), SS has been associated with a wide range of neurologic abnormalities. Parkinsonism has been reported previously in five SS patients. We present three additional cases of SS with parkinsonism.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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2/109. Relevance of complement fixing antinuclear antibodies.

    BACKGROUND: connective tissue diseases (CTDs) are a heterogeneous group of disorders defined by the association of a variety of clinical manifestations with immunologic and other laboratory findings. Overlap of syndromes and aberrant findings appear rather frequently. methods: Sera of eight antinuclear antibody (ANA) negative, cases of subacute cutaneous lupus erythematosus (SCLE) with antibodies to Ro (SS-A) and a ninth case with clinical and laboratory signs of sjogren's syndrome and systemic lupus erythematosus (SLE) were tested for complement (C') fixing antinuclear antibodies (C-ANAs). The ninth case was examined in depth by direct immunofluorescence (DIF) and a two-step "C DIF" test of biopsies for C' fixation to in vivo bound ANAs, as well as serum tests for C-ANA, ANA, and SCLE markers. RESULTS: Sera of five of the eight ANA negative, Ro(SS-A) positive SCLE cases had C-ANAs. The ninth case, a 50-year-old woman with clinical and laboratory signs of sjogren's syndrome and SLE, gave a strong positive C DIF reaction in the skin biopsy for in vivo bound ANAs that fix C', but negative ANAs and C-ANAs in routine serum tests; they revealed antimitochondrial antibodies. serum tests on normal skin, however, revealed weak ANA and strong C-ANA reactions with in vitro fixed C'. CONCLUSIONS: ANA negative cases of SCLE or sjogren's syndrome may have C-ANAs. A case with sjogren's syndrome and signs of SLE had both in vivo and in vitro C' fixing ANAs. C-ANA tests can aid in the identification of such cases.
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ranking = 1.8965119080448
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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3/109. The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women.

    A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of hla-dr antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjogren syndrome (SjS), Sjogren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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4/109. A complex case of hepatitis in a patient with systemic lupus erythematosus.

    liver involvement in patients with systemic lupus erythematosus (SLE) is considered rare. Previous treatment with potentially hepatotoxic drugs or viral hepatitis have usually been implicated as the main causes of liver disease in SLE patients. On the other hand, even after careful exclusion of these aetiologies, the problem remains whether to classify the patient as having a primary liver disease with associated autoimmune clinical and laboratory features resembling SLE, such as autoimmune hepatitis, or as having liver disease as a manifestation of SLE. We report the case of an elderly woman who presented with acute hepatitis, who had been diagnosed with SLE 14 years ago and who also had sjogren's syndrome and anti-phospholipid's syndrome for several years. The histology depicted chronic active hepatitis and, after drug-induced hepatitis and viral hepatitis were excluded, the serological and clinical features were shown to be typical of liver damage caused by SLE. The patient was treated with azathioprine 100 mg/d and prednisone 30 mg/d. The clinical symptoms resolved in 10 days and the laboratory values were normal at the end of the first month of therapy. prednisone was progressively reduced, during a period of 4 months, to 10 mg/d but azathioprine was kept to the same dose. One year after the diagnoses the patient is still in remission. Although uncommon, hepatic involvement is well recognised in SLE. The interest of this case lies in the differential diagnosis and recognition of this condition, which deserves an aggressive treatment.
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ranking = 5
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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5/109. Diminished expression of CD59 on activated CD8 T cells undergoing apoptosis in systemic lupus erythematosus and sjogren's syndrome.

    The present study was undertaken to examine the phenotype of T cells undergoing in vitro apoptosis in patients with autoimmune diseases such as systemic lupus erythematosus (SLE) and primary sjogren's syndrome (SS). Compared with normal controls, we found diminished expression of CD59 antigen (one of the cell-surface complement-regulatory proteins) on CD8 T cells, but not on CD4 T cells, from patients with SLE and SS. Three-colour immunofluorescence analysis revealed that these CD59dim CD8 T cells were activated T cells, expressing both human leucocyte antigen (HLA)-DR and CD45RO antigens. In addition, these CD59dim CD8 T cells were more susceptible to in vitro apoptosis than CD59bright CD8 T cells. In two patients with active lupus, the percentage of CD59dim CD8 T cells was significantly decreased after steroid therapy. These findings suggest that decreased expression of CD59 antigen on in vivo-activated CD8 T cells may be correlated with disease activity and may be involved in activation-induced apoptosis in patients with SLE and SS.
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ranking = 5.0447271897036
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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6/109. Clinical constellation of annular erythema associated with anti-Ro/La autoantibodies.

    Annular erythema (AE) associated with anti-Ro (SSA) and/or La (SSB) autoantibody in patients with Sjogren syndrome (SS) or with SS/systemic lupus erythematosus overlap syndrome (SS/SLE), has recently been described in Orientals, and it may be a counterpart of annular skin lesion of the subacute cutaneous LE seen mostly in Caucasians. The author examined five Korean AE patients in respect to dinical diversity. In this small-sample study, subtle differences appeared between individual cases regarding the serologic features and the diagnoses of the disease. Among the five cases, four had circulating anti-Ro and anti-La antibodies, and one had only anti-La. Regarding the diagnosis, one was SS/SLE, two were primary SS, and the remaining two were only "AE associated with anti-Ro/La antibody". There seem to be a wide clinical spectrum in the disease expression of AE associated with anti-Ro/La autoantibody than previously thought.
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ranking = 1
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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7/109. Annular erythema of sjogren's syndrome in a white woman.

    Annular erythema of sjogren's syndrome (AE-SS) is believed to be the Asian counterpart of subacute cutaneous lupus erythematosus (SCLE) in white persons. We report the second case of a white person with AE-SS, diagnosed by clinical and serologic findings, as well as the absence of histologic criteria for SCLE. The diagnosis of AE-SS was established by symptoms of xerophthalmia and xerostomia, as well as by examination of skin and salivary gland biopsy specimens. Evaluation showed the presence of anti-Ro(SS-A) and anti-La(SS-B) autoantibody, with the presence of anti-Ro(SS-A) antibody against the 60-kd, but not the 52-kd, epitope, a pattern frequently seen in both the Asians with AE-SS and in white patients with SCLE. Both skin and sicca symptoms were alleviated with combination antimalarial therapy, which included hydroxychloroquine and quinacrine. This case demonstrates that AE-SS can occur in white patients and that the autoantibody profile is similar to that described in Asians with this disease.
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ranking = 0.89651190804476
keywords = lupus erythematosus, erythematosus, lupus
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8/109. Immunoblastic sarcoma with features of sjogren's syndrome and systemic lupus erythematosus in a patient with immunoblastic lymphadenopathy.

    A patient with immunoblastic lymphadenopathy and features of sjogren's syndrome and systemic lupus erythematosus is presented. Clinical features included generalized lymphadenopathy, rash, alopecia, and synovitis, with associated laboratory abnormalities of a positive antinuclear factor and double-stranded dna antibodies, positive lupus band test, and hemolytic anemia. Symptoms of sjogren's syndrome included dry eyes and mouth and swollen parotid gland; biopsy results of the accessory salivary glands were positive. At autopsy immunoblastic sarcoma was found that involved the myocardium, which stained for both kappa and lambda light chains by immunoperoxidase techniques.
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ranking = 5.0447271897036
keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus
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9/109. Subacute cutaneous lupus erythematosus and life-threatening hypokalaemic tetraparesis: a rare complication.

    We describe a patient with lesions of subacute cutaneous lupus erythematosus associated with high titres of anti-Ro and anti-La antibodies who developed a rapidly progressive flaccid tetraparesis due to profound hypokalaemia. After investigation she was found to have distal renal tubular acidosis (dRTA) without pathological evidence of lupus nephritis. It is likely that her dRTA was a manifestation of associated sjogren's syndrome, which had been otherwise asymptomatic. This is the first report of such a complication in the dermatological literature.
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ranking = 4.5272867299273
keywords = lupus erythematosus, erythematosus, lupus
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10/109. Bamboo node: primary vocal fold lesion as evidence of autoimmune disease.

    Descriptions of vocal fold lesions related to autoimmune diseases are rare in the literature, and focus mainly on rheumatoid nodules. This is the first report in which autoimmune diseases were promptly suspected by the observation of a unique white transverse submucosal lesion in the vocal fold during clinical examination. This lesion, reported only in autoimmune disease, has been called the bamboo node and its features are different from those of rheumatoid nodules. We report here on two patients who did not have a diagnosis of systemic disease before investigation of their main complaint of hoarseness. At the patients' first visit, vocal fold bamboo nodes were seen in the vocal fold and the otolaryngologist suspected the presence of an autoimmune disease. We requested clinical investigation to clarify our suspicion that there was an underlying systemic disease. After the investigation, both patients were shown to have autoimmune disease, sjogren's syndrome and systemic lupus erythematous, respectively. This paper emphasizes the important role of the otolaryngologist in the detection of these unique lesions in the vocal folds through the conventional laryngeal methods. These methods consisted of direct observation with a rigid laryngeal endoscope and investigation of the patient's distinctive vibratory pattern by means of laryngeal stroboscopy. The method of treatment we used to obtain the best outcome in terms of voice improvement is also discussed.
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ranking = 0.065549835952805
keywords = lupus, systemic lupus
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