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ID PMID Title PublicationDate abstract
1582057 Hypokalemic quadriplegia and respiratory arrest revealing primary Sjögren's syndrome. 1992 Apr We report a case of hypokalemic flaccid quadriplegia with sudden respiratory arrest in a 38-year-old woman discovered to have distal renal tubular acidosis which lead to the diagnosis of primary Sjögren's syndrome. This case is compared to 8 similar cases previously described in the literature.
8315114 Giant mucocele of oral cavity as a mucocutaneous manifestation of Sjögren syndrome. 1993 Apr Two cases of Sjögren syndrome who developed a giant mucocele on the floor of the oral cavity are reported. Histological analysis revealed the dense infiltration of lymph-plasmacytic infiltration around the dilated salivary duct. Similar findings were observed in the biopsied specimens of the labial salivary gland which were consistent with the grade 4 score proposed by Chisholm and Mason's criteria. Mucocele is a common mucocutaneous disease of unknown etiology except for traumatic origin and Sjögren syndrome might be one of the important underlying disease which causes giant mucocele, especially in elderly female patients.
8452579 Preliminary criteria for the classification of Sjögren's syndrome. Results of a prospecti 1993 Mar OBJECTIVE: Different sets of diagnostic criteria have been proposed for Sjögren's syndrome (SS), but none have been validated with a large series of patients or in a multicenter study. We conducted the present study involving 26 centers from 12 countries (11 in Europe, plus Israel), with the goals of reaching a consensus on the diagnostic procedures for SS and defining classification criteria to be used in epidemiologic surveys and adopted by the scientific community. METHODS: The study protocol was subdivided into two parts. For part I, questionnaires regarding both ocular and oral involvement were developed; they included 13 questions and 7 questions, respectively. For part II a limited set of diagnostic tests was selected, and the exact procedure to be followed in performing these tests was defined. Part I of the study included 240 patients with primary SS and 240 age- and sex-matched controls. Two hundred forty-six patients with primary SS, 201 with secondary SS, 113 with connective tissue diseases but without associated SS, and 133 control patients were studied in part II. RESULTS: The study resulted in (a) the validation of a simple 6-item questionnaire for determination of dry eyes and dry mouth, which showed good discriminant power between patients and controls, to be used in the initial screening for sicca syndrome; and (b) the definition of a new set of criteria for the classification of SS. The sensitivity and specificity of the criteria in correctly identifying patients with either the primary or the secondary variant of SS were also determined. CONCLUSION: Using the findings of this prospective multicenter European study, general agreement can be reached on the diagnostic procedures to be used for patients with SS. Final validation of the preliminary classification criteria for SS is underway.
8009004 [Sicca syndrome and low-grade chronic viral hepatitis C. Apropos of a series of 50 cases]. 1993 Systemic manifestations are described in chronic hepatitis C. Haddad et al have showed the prevalence of sicca syndrome with lymphocytic sialadenitis. We analysed prevalence in 50 patients suffering from chronic hepatitis C with histologic low rank. 50% of them have pronounced abnormality of lachrymal secretion without sialadenitis. It seems that sicca syndrome is present in low rank hepatitis. In that case xerophtalmia preceded xerostomia.
8568184 [Association of Takayasu's arteritis, pregnancy and Still's disease]. 1995 Two pregnant patients presented with Takayasu's disease predominating in the major branches of the aorta. There was no extention below the diaphragm and no complications. One of the patients had Still's disease which is a predominantly cutaneous form without chronic arthritis. Both pregnancies were uneventful excepting dysgravidia in one case. Two eutrophic infants were born at term. The risk of Takayasu's arteritis associated with pregnancy, as reported in the literature, is mainly due to the consequences of arterial hypertension with pre-eclampsia (60%), heart failure and cerebral vascular events (5%). The major fetal risk is in utero death (2 to 5%), but intra-uterine growth retardation is more frequent (18%). The risk is greatest during the third trimester and during the perinatal period. Fetal involvement is greatest in sever cases and in those treated late. Prevention is based on the initial work-up to identify the disease and possible complications, programming pregnancies and increasing surveillance during periods of risk, defining the delivery route with cesarean section reserved for complications of arteritis (30%), and planned labour with instrumental extraction and epidural anaesthesia with control of the blood pressure. Still's disease has no particular consequence on pregnancy, although sequellae of chronic arthritis of the pelvis may have an impact on obstetrical technique. An association with Takayasu's disease is rare with only one case being reported in the literature; aetiopathology remains unknown.
8141212 Gynecologic manifestations of Sjögren's syndrome. 1994 Mar OBJECTIVE: Sjögren's syndrome is a chronic, inflammatory autoimmune disease in which the salivary and lacrimal glands are progressively destroyed by lymphocytes and plasma cells. Because women are affected 10 times more often than men, we studied gynecologic manifestations of Sjögren's syndrome. STUDY DESIGN: One thousand questionnaires were sent to women with Sjögren's syndrome in New York, New Jersey, Connecticut, and Pennsylvania. Five hundred thirty-nine women responded. RESULTS: Women with Sjögren's syndrome reported significant vaginal dryness. There was no relationship of Sjögren's syndrome to either the incidence of infertility or miscarriage, although the 4% incidence of congenital anomalies in offspring was relatively high. Of the congenital anomalies, nine of 19 (47%) were cardiac. A long menstrual cycle (> 35 days) was associated with infertility and neuropathy. CONCLUSIONS: The vaginal dryness in women with Sjögren's syndrome is not surprising, because the nasal and esophageal mucosae are also dry in this disorder. The relationship of infertility to a long menstrual cycle may simply indicate the presence of ovulatory dysfunction or inadequate luteal phase unrelated to Sjögren's syndrome. The relationship of neuropathy to a long menstrual cycle may be related to repeated, prolonged estrogen or progesterone exposure during the long cycles or to involvement of hypothalamic-pituitary-ovarian function.
8978969 Juvenile onset of primary Sjögren's syndrome: report of 10 cases. 1996 Nov OBJECTIVE: Ten new cases with primary Sjögren's syndrome (pSS) whose disease began before age 16 are described. Special attention is paid both to the follow-up and treatment of this condition. METHODS: Cases with juvenile pSS were retrospectively identified from our series of 180 pSS patients. Ocular, salivary, and extraglandular manifestations as well as a full laboratory evaluation including HLA-DR typing were retrieved. RESULTS: A disease prevalence of 5.5% (10 cases, 8 female and 2 male) was found in our series. The mean age at onset was 11.0 years, but the disease started at the age of 4 in 2 patients. At onset, parotid swelling was found in 6 cases and extraglandular manifestations in 3. Throughout the follow-up period (mean 48.6 months from the time of diagnosis), the clinical picture was similar to that of pSS in adults, but oral involvement was generally milder. Extraglandular manifestations were always present but never severe. Pertinent laboratory abnormalities (e.g. rheumatoid factor, polyclonal hypergammaglobulinemia, leukopenia, increased ESR, ANA and anti-SSA/SSB antibodies) were found in all patients. Specifically, ANA and anti-SSA were always positive. Moreover, in our cases histocompatibility antigens HLA-DR3 and DR 52 were closely associated with the disease. Clinical outcome was difficult to predict; however, no serious complications have been observed so far. We obtained good results with low-dose steroids and/or hydroxychloroquine, especially with regard to the extraglandular manifestations and laboratory abnormalities. CONCLUSION: We confirm that juvenile pSS is not a rare condition. It closely resembles pSS in adults except for the extremely high prevalence of recurrent parotitis and immunological findings.
8810621 Interstitial lung disease in primary Sjögren's syndrome. Clinical-pathological evaluation 1996 Sep Interstitial disease is a recognized form of lung involvement in primary Sjögren's syndrome in which frequency and severity have not yet been established. We studied 20 patients 61.2 +/- 36.4 mo after the first symptoms of the syndrome that fulfilled criteria for both xerostomia and xerophtalmia. Eighteen patients exhibited pulmonary symptoms; nine presented a restrictive functional pattern. The chest roentgenogram disclosed interstitial involvement in nine patients. Gallium-67 lung scan presented hypercaptation in 15 of 19 patients studied. Thirteen patients who submitted to bronchoalveolar lavage presented higher cell counts with increases of lymphocytes and/or polymorphonuclear cells. All patients had abnormal results in at least one of the above. Lung biopsy, undertaken in 12 patients, showed a whole spectrum of interstitial disease, from a follicular bronchiolitis to a lymphoid interstitial pneumonia and finally fibrosis with honeycombing. One patient presented with associated sarcoid granulomas. We treated 11 patients with an azathioprine-based regimen and found a significant improvement in the forced vital capacity (p < 0.05) after at least 6 mo when compared with nontreated patients. We conclude that the frequency of interstitial lung disease is high in Sjögren's syndrome and that an azathioprine-based treatment can favorably change the outcome in this population.
8646439 'Seronegative' Sjögren's syndrome manifested as a subset of chronic fatigue syndrome. 1996 May We determined the extent to which chronic fatigue syndrome (CFS) patients with sicca symptoms fulfil the diagnostic criteria for Sjögren's syndrome (SS). Three sets of diagnostic criteria for SS, formulated by the Japanese, Europeans and Fox, were used. One-third of the CFS patients with sicca symptoms fulfilled the diagnostic criteria for SS. However, they were 'seronegative', differing from the ordinary primary SS.
8209965 [Magnetic resonance applied to the diagnosis of Sjogren disease]. 1994 In order to characterize typical MRI-findings in patients with Sjögren's syndrome who present recurrent parotid swelling 15 patients with clinical diagnosis of this disease were examined in a prospectives study. Most of the patients showed bilateral enlarged glands with an inhomogeneous, nodular pattern. The signal intensity was very high, especially in the T2 weighted sequences.
8286835 Primary Sjögren's syndrome with subcortical dementia. 1993 Jul A 48-year-old man with primary Sjögren's syndrome (P-SS) developed subcortical dementia, characterized by forgetfulness, poor attention and concentration, slowness of thought process, difficulty to manipulate acquired knowledge, introversive and hostile personality change and inactivity. These were improved by corticosteroid treatment. Magnetic resonance imaging (MRI) revealed small lesions in the subcortical and periventricular white matter. An electroencephalogram (EEG) revealed short runs of theta activities in bilateral parietal areas. Latency of P300 shortened in accordance with clinical improvement. These findings suggest that the cognitive and neuropsychiatric manifestations in P-SS are included in a spectrum of organic brain dysfunction and are treatable.
8310206 The clinical significance of the detection of anti-Ro/SS-A and anti-La/SS-B autoantibodies 1993 Recently, an enzyme-linked immunosorbent assay (ELISA), using purified recombinant non-fusion proteins, has been introduced to detect and quantify Ro/SS-A and La/SS-B autoantibodies. We compared this method with anti-Ro/SS-A and anti-La/SS-B detection by means of counterimmunoelectrophoresis and immunoblotting in patients with primary Sjögren's syndrome (SS), patients suspected of the syndrome and controls. The sensitivity and specificity of the newly developed ELISA for anti-Ro/SS-A were 53% and 100%, respectively, and for anti-La/SS-B, 40% and 98%, respectively. No significant difference was found between these results and those obtained from both other assays. Titres of Ro/SS-A and La/SS-B autoantibodies correlated with the presence of an abnormal parotid gland sialogram and hypergammaglobulinaemia. We concluded that the new ELISA did not enhance the diagnostic yield in cases of suspicion of primary Sjögren's syndrome. Longitudinal studies of large groups of patients with primary Sjögren's syndrome are necessary to demonstrate whether following the course of the titres of these autoantibodies would be of value for prediction of disease exacerbations.
1575789 Acute iritis associated with primary Sjögren's syndrome and high-titer anti-SS-A/Ro and a 1992 May OBJECTIVE: We describe a patient with primary Sjögren's syndrome who developed severe, acute, anterior uveitis (iritis), an uncommon complication in this setting. METHODS: We present the case report of the clinical findings, course, treatment, and resolution of the acute uveitis. Titers of anti-SS-A/Ro and anti-SS-B/La antibodies were assessed (by immunodiffusion), as were fluorescent antinuclear antibodies (on HEp-2 cells) and cryoglobulins. RESULTS: Initial treatment with topical steroids, oral prednisone (20 mg/day), and oral methotrexate was unsuccessful. The iritis resolved after combined treatment with intravenous cyclophosphamide (1,500 mg/month), high-dose prednisone (60 mg/day), and cyclosporine (5 mg/kg/day). CONCLUSION: An uncommon, severe complication of primary Sjögren's syndrome is acute uveitis. Combination immunosuppressive therapy may be needed to control this condition.
1565240 Primary Sjögren's syndrome in patients with multiple sclerosis. 1992 Apr The relationship between multiple sclerosis (MS) and primary Sjögren's syndrome (PSS) is ambiguous; it was suggested that some patients diagnosed with MS may instead have PSS. In a recent epidemiologic study, the prevalence of PSS was 2.7% in southern Sweden. We randomly selected 30 patients with definite MS from our patient population and investigated them for evidence of PSS according to the Copenhagen criteria. One patient had clinical symptoms compatible with PSS and also fulfilled the Copenhagen criteria. In addition, five patients had keratoconjunctivitis sicca, one had xerostomia, and three had histopathologic evidence of sialadenitis in the lower lip salivary glands. However, one of these findings alone is not sufficient to support the diagnosis of PSS. We conclude that MS and PSS may coexist in the same individual, but PSS is not more common among MS patients than expected in the general population.
8531339 [Epidemiology and pathogenesis of Sjögren's syndrome]. 1995 Oct A nationwide epidemiological survey of Japanese Sjögren's syndrome (SS) patients was conducted in 1994 by the Research Committee on Epidemiology of Intractable Diseases and the Research Committee of Autoimmune diseases, Ministry of Health and Welfare, Japan. The total number of patients treated for SS in 1993, in Japan, was estimated as 17,000 (95% confidence interval 15,000-20,000). The estimated crude prevalence rates were 1.9 and 25.6 per 100,000 population in males and females, respectively. The ratio of female to male patients was 13.7. The peak of age distribution was in their fifties. New research trends to explore the pathogenesis of SS are also discussed here.
8039975 Senile dry skin type Sjögren's syndrome. 1994 May BACKGROUND: Elderly patients may show asteatotic dermatitis complicating Sjögren's syndrome (sjs) that we have named "senile dry skin type sjs." To define the condition further, we have examined a group of patients with sjs and have recorded clinical and laboratory findings in these patients. PATIENTS AND MATERIALS: Of the 36 patients, 11 were men and 25 were women, with a mean age of 72 years. In addition to the subjective sensation of dryness of the eyes and oral cavity and objective signs of dryness revealed by the Schirmer test, the rose bengal test, minor labial gland biopsy, and sialography were carried out, as well as other hematologic and immunologic tests. RESULTS: The following abnormal laboratory tests were found: elevated erythrocyte sedimentation rate (85%), mild hepatic dysfunction (50%), and anemia (45%), leukopenia (42%), and thrombocytopenia (16%). Immunologic abnormalities were also observed, such as: positive antinuclear antibody (55%), an increase in gamma globulin (28%), and a positive rheumatoid factor (20%). In 11 of the 36 patients (30%), the subjective sensation of dryness was absent and only objective signs of dryness were present. CONCLUSIONS: The present study suggests that dry-skin-dermatitis associated with sjs is rather common in the elderly.
1491657 [The role of biopsy of the minor salivary glands in the diagnosis of Sjögren's syndrome]. 1992 Sep After a short critical review of the latest literature, the authors evaluate the role of minor salivary gland biopsy in the diagnosis of Sjögren's syndrome. The histological factors typical of this syndrome are examined in this context.
1556425 The pathogenesis of nodular regenerative hyperplasia of the liver associated with rheumato 1992 Mar Nodular regenerative hyperplasia (NRH) is an uncommon liver disease that typically develops in the setting of a chronic illness such as Felty's syndrome. Although the pathogenesis of NRH has not been defined, vasculitis has been postulated to play an important pathogenetic role in some cases of NRH, even though the association of NRH and vasculitis has been reported rarely. We describe two unusual cases of NRH in patients with Felty's syndrome complicated by rheumatoid vasculitis. Morphometric analysis demonstrated evidence of injury to the hepatic vasculature in the form of loss of small intrahepatic arteries and portal spaces, findings that support the hypothesis that hepatic arteritis plays a role in the development of NRH in cases associated with systemic vasculitis.
7920524 [Diagnosis of arthritis in black Africa. Apropos of 473 cases in Congo]. 1994 Apr A cross-sectional study of arthritis was conducted in the Rheumatology Department of the Brazzaville Teaching Hospital, Congo. A total of 473 patients with arthritis seen between 1989 and 1991 were subjected to the limited tests available. Gout was the leading diagnosis (n = 83). Septic arthritis (n = 82) and infectious discitis (n = 55) were the most common reasons for admission. Tests often failed to identify the causative organism; Staphylococcus was the most commonly recovered organism. Tuberculous discitis was less common than discitis due to pyogenic bacteria. HIV-related arthritis (n = 57) usually manifested as severe, febrile, asymmetrical, nonerosive, polyarthritis. Cases of rheumatoid arthritis (n = 29) fit the classical description of the disease. In 83 patients with monoarthritis, oligoarthritis, or polyarthritis, no etiology could be identified.
7504436 Occurrence of antiperinuclear, antikeratin, and anti-RA 33 antibodies in juvenile chronic 1993 Nov OBJECTIVES: Antiperinuclear factor (APF), antikeratin antibodies (AKA), and anti-RA 33 antibodies are currently considered to be good markers for the diagnosis of adult rheumatoid arthritis with or without rheumatoid factor (RF). The prevalence of these markers was retrospectively reviewed in children with juvenile chronic arthritis (JCA) to determine whether they were associated with specific features. METHODS: One hundred and twenty-four patients with JCA participated in this study. Controls included 28 patients with juvenile systemic lupus erythematosus and 21 healthy children. Antiperinuclear factor and AKA were determined by indirect immunofluorescence on buccal mucosal cells and oesophagus sections respectively. Anti-RA 33 antibodies were detected using a Western blot technique on HeLa cell nuclear extract. RESULTS: Antiperinuclear factor was virtually absent in all the tested subgroups and anti-RA 33 antibodies were detected only in a subset of patients with RF positive polyarticular onset. Antikeratin antibodies were found in 27% of all children with JCA and in 42% of those with RF negative polyarticular onset. These results were statistically significant compared with healthy controls, but the presence of AKA was not specific to any patient subgroup. Moreover, in contrast with previous studies in adult RA, no relation was found between the presence of AKA and disease severity or activity. CONCLUSION: These data suggest that APF, AKA, and anti-RA 33 antibodies are not useful for the diagnosis or classification of JCA.