Objectives We propose new classification criteria for Sjögren’s Syndrome (SS), which are needed considering the emergence of biological agents as potential treatments and their associated co-morbidity. These criteria target individuals with signs/symptoms suggestive of SS. Methods Criteria are based on expert opinion elicited using the Nominal Group Technique, and analyses of data from the Sjögren’s International Collaborative Clinical Alliance. Preliminary criteria validation included comparisons with classifications based on the American-European-Consensus-Group (AECG) criteria, a model-based “gold standard” obtained from Latent Class Analysis (LCA) of data from a range of diagnostic tests, and a comparison with cases and controls collected from sources external to the population used for criteria development Results Validation results indicate high levels of sensitivity and specificity for the criteria. Case definition requires at least 2 out of the following 3: Positive serum anti-SSA and/or anti-SSB or [positive rheumatoid factor and ANA ≥ 1:320]; Ocular staining score ≥ 3; Presence of focal lymphocytic sialadenitis with focus score ≥ 1 focus/4mm2 in labial salivary gland biopsies. Observed agreement with the AECG criteria is high when these are applied using all objective tests. However, AECG classification based on allowable substitutions of symptoms for objective tests results in poor agreement with the proposed and LCA-derived classifications. Conclusion These classification criteria developed from registry data collected using standardized measures are based on objective tests. Validation indicates improved classification performance relative to existing alternatives, making them more suitable for application in situations where misclassification may present health risks.
Objective To study the prevalence of extra-glandular manifestations (EGM) in primary Sjögren’s Syndrome (pSS) among participants enrolled in the Sjögren’s International Collaborative Clinical Alliance (SICCA) registry. Methods 1927 participants in the SICCA registry were studied, including 886 participants who met the 2002 American-European consensus group (AECG) criteria for pSS, 830 “intermediate” cases who had some objective findings of pSS but did not meet AECG criteria, and 211 control individuals. We studied the prevalence of immunologic and hematologic laboratory abnormalities; specific rheumatologic examination findings; and physician confirmed thyroid, liver, kidney disease and lymphoma among SICCA participants. Results Laboratory abnormalities, including hematologic abnormalities, hypergammaglobulinemia and hypocomplementemia, frequently occurred among pSS cases, and were more common among the intermediate cases than among control participants. Cutaneous vasculitis and lymphadenopathy were also more common among pSS cases. In contrast, the frequency of physician confirmed diagnoses of thyroid, liver and kidney disease, and lymphoma was low and only primary biliary cirrhosis was associated with pSS cases status. Rheumatologic and neurologic symptoms were common among all SICCA participants, regardless of case status. Conclusions Data from the international SICCA registry support the systemic nature of pSS, manifest primarily in terms of specific immunologic and hematologic abnormalities. The occurrence of other systemic disorders among this cohort is relatively uncommon. Previously reported associations may be more specific to select patient subgroups, such as those referred for evaluation of certain neurologic, rheumatologic or other systemic manifestations.
The conventional treatment of patients with ureteric obstruction due to retroperitoneal fibrosis (RF) is surgery in combination with long-term corticosteroids. This report describes 11 cases of RF with ureteric obstruction treated with methyl-prednisolon pulse therapy (MPPT) in combination with azathioprine or penicillamine following initial insertion of ureteral stents. The medial treatment suggested was successful in 7 patients, but only moderately effective in the last 4 patients. This principle of non-operative management of RF has not been previously reported.
Local hypoxia in the arterial wall may be a factor of pathogenetic importance in the development of human arteriosclerosis.1 Experimental studies of the reactions of the arterial wall to hypoxia are, however, few. Thorner and Lewy,2 in a study of the effects of anoxia on the brain of guinea pigs, observed calcification and fibrosis in the media of the small arteries of the cortex.In a preliminary investigation3 the authors were able to report the development of severe gross arteriosclerosis in the rabbit aorta as a result of daily exposure of the animals to repeated periods of systemic hypoxia for 3 weeks. In view of these findings, this study was designed to analyze biochemical and microscopic changes in the vascular connective tissue of the aorta in this type of experimental arteriosclerosis and to elucidate the pathogenesis. METHODSMale albino rabbits of the Danish country species, 5 months old and weighting about 3 kg, were used. The animals were fed an adequate uniform laboratory diet. The study included two groups. One group of animals was exposed to daily periods of systemic hypoxia according to the following procedure. Each rabbit was placed in a plastic box (8 by 16 by 28 in) covered with plastic foil. The oxygen content in the box was registered by a Beckmann oxygen electrode, placed in the side wall of the box. Through a valve in the side, pure nitrogen was substituted until the oxygen content was reduced to 5 per cent (after about 35 sec.). At that time the animals became unconscious and had convulsions. Atmospheric air was immediately supplied, and after a further 20 sec. oxygen content increased to 17 per cent. Simultaneously, the animals reat UNIV OF MICHIGAN on June 22, 2015 ang.sagepub.com Downloaded from 2 gained consciousness and were able to move. Now and then, however, artificial respiration had to be given. Immediately thereupon, nitrogen was added again and the above mentioned procedure repeated. Thus, each animal was exposed to two hypoxic periods in one session. The entire procedure was performed twice daily for 14 days. The control rabbits of the other group were exposed to exactly the same procedure as the experimental animals with the exception that atmospheric air was given instead of nitrogen.On the 15th day, all the rabbits were given i.p. injections of 1 me of 35S-labeled sodium sulfate (carrier-free 35S-sulfate diluted to a suitable volume with a 10 mg/100 ml solution of sodium sulfate). At the same time, 1 ml of a 2 per cent solution of human serum-albumin-~1, containing 40 lie, was injected i.v. into each animal. After 48 hr., the animals were killed by i.v. injections of 300 mg of sodium Pentobarbital. The entire aorta and the heart were removed. The hearts were weighed and specimens for microscopic studies were removed from the anterior wall of the left ventricle. Blood samples and the right 10th rib were taken for isotope studies.Macroscopic Studies The aortae were cut longitudinally, placed on a millimeter paper and photographed. The inner surface area of the aorta and the e...
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