Cases reported "Sjogren's Syndrome"

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1/4. sarcoidosis or Sjogren syndrome? Clues to defining mimicry or coexistence in 59 cases.

    We present 5 new cases of coexisting sarcoidosis and Sjogren syndrome (SS) and review the literature for additional cases in order to analyze the clinical, immunologic, and histologic characteristics that may help physicians differentiate the mimicry of SS by sarcoidosis from a true coexistence of both autoimmune diseases. We considered the coexistence of sarcoidosis with SS to be when patients presented specific histologic patterns of both diseases, simultaneously or at different times.Fifty-nine patients were included in the analysis (54 identified in the literature search plus our 5 unpublished cases): 49 (83%) patients were female and 10 (17%) were male, with a mean age at diagnosis of 50 years. According to the histopathologic examination of the exocrine glands performed in 53 cases, we defined coexistence of sarcoidosis and SS in 28 cases, while in the remaining 25 patients, sarcoidosis mimicked SS. Clues to identifying when sarcoidosis coexists with SS were a higher prevalence of systemic manifestations (arthritis and uveitis) and positive immunologic parameters (antinuclear antibodies, rheumatoid factor, and anti-Ro/SS-A), as well as the existence of a focal sialadenitis (Chisholm-Mason score grades III-IV, with a CD4 lymphocytic infiltration) in the salivary gland biopsy. In patients first diagnosed with primary SS, the appearance of some clinical features such as hilar adenopathies, uveitis, or hypercalcemia leads to the diagnosis of coexisting sarcoidosis. A careful application of the new American-European consensus criteria had a sensitivity of 93% and a specificity of 92% in identifying when SS coexists with sarcoidosis.In conclusion, the association of sarcoidosis with SS leads to a true coexistence of both diseases in more than half the patients described in the literature, while in the remaining patients, sarcoidosis mimics SS. In light of these results, sarcoidosis should not be considered as an exclusion criterion for the diagnosis of SS, and in patients with a suspected overlap of the two diseases, application of the new American-European consensus criteria for diagnosis of SS should be mandatory.
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2/4. pilocarpine toxicity and the treatment of xerostomia.

    pilocarpine has been used as an ophthalmologic agent for decades; however, toxicity rarely has been reported in the medical literature. Oral pilocarpine tablets, as well as another muscarinic agent (cevimeline), have recently been approved for the treatment of dry mouth (xerostomia). We report a case of unintentional overdose of oral pilocarpine tablets that resulted in bradycardia, mild hypotension, and muscarinic symptoms in a patient with sjogren's syndrome. The patient's symptoms were relieved with 0.5 mg intravenous atropine and she recovered uneventfully. The case is unique in that it is the first reported ingestion of oral pilocarpine tablets and the first dosing error reported for this indication. In addition, this case underscores the importance of communication between physician and patient and the resulting toxicity related to miscommunication.
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3/4. autoantibodies and the spectrum of sjogren's syndrome.

    In studies reported recently, the sera of patients with sjogren's syndrome were found to contain precipitating antibodies to nuclear antigens that can be identified by immunodiffusion analysis. These precipitating autoantibodies have been termed SS-A and SS-B antibodies. We show that identification of these autoantibodies helped in establishing the diagnosis of sjogren's syndrome in 12 of 30 patients in whom the diagnosis had not been considered at the time of the physician's initial examination. The reasons for this were related to lack of spontaneous complaints of keratoconjunctivitis sicca and xerostomia and prominence of symptoms associated with arthritis, myalgia, pulmonary fibrosis, and cardiac disease. This study re-emphasizes the importance of multisystem disease in sjogren's syndrome and shows that specific serologic assays for autoantibodies aided in diagnosis.
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4/4. sjogren's syndrome: implications for the primary care physician.

    sjogren's syndrome (SS) is a common immunologically-mediated disorder that will afflict a great proportion of our population in future years. The primary care physician undoubtedly will be confronted with such patients, many of whom will have complaints and findings not generally believed to be due to SS. In this communication, case reports of four patients with SS as well as a literature review are presented. Two patients had presentations that were classic for SS; however, the other two were diagnostic challenges. Emphasis is placed on the atypical and unusual way SS can present so that the primary care physician can more easily make the diagnosis and initiate therapy.
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