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ID PMID Title PublicationDate abstract
9334481 [Distal renal tubular acidosis and nephrolithiasis in 3 cases of primary Sjögren syndrome 1996 Dec Tubulo interstitial nephritis, the main manifestation of renal involvement in Sjögren syndrome, may lead to a tubular dysfunction that is usually subclinical. We report three women, aged 32, 35 and 35 years old, with a primary Sjögren syndrome and symptomatic type I or distal tubular acidosis. Two patients had nephrolithiasis and one a nephrocalcinosis. Two had a basal hyperchloremic metabolic acidosis. The ammonium chloride acidification test was abnormal in all, demonstrating a distal tubular defect. None had proximal tubular dysfunction. All had an urinary pH over 6.5 and hypocitraturia and none had hypercalciuria. Renal calculi were composed of calcium oxalate and calcium phosphate in two patients and calcium phosphate and ammonium phosphate in the other. All women had positive antinuclear antibodies with mottled pattern, two had anti Ro antibodies and positive rheumatoid factor and one had hypergammaglobulinemia. None had anti La antibodies, crioglobulinemia or monoclonal proteins.
1418023 Signal transduction in Sjögren's syndrome T cells. Abnormalities associated with a newly 1992 Sep OBJECTIVE: To study the effects of a novel A-type retrovirus, detected in cocultures of lip biopsy specimens from Sjögren's syndrome (SS) patients and a human T cell line, on the infected T cells. METHODS: Interleukin-2 (IL-2) and IL-6 secretion were measured by bioassay and enzyme-linked immunosorbent assay, respectively, in the infected and noninfected cell lines. Surface antigen expression was determined by flow cytometry, using monoclonal antibodies. Protein kinase C (PKC) activity was measured using an enzyme assay kit, and calcium mobilization was assessed with a fluorescent probe. RESULTS: Infected cells expressed less CD4 and IL-6 receptor, but more HLA-DR, compared with noninfected cells. Infected cells also produced less IL-2 and displayed reduced PKC activation and calcium mobilization. A similar defect in calcium mobilization was detected in T cells from SS patients. CONCLUSION: These data suggest a possible involvement of the newly described retrovirus in T cell abnormalities.
8331119 Long-term results of Charnley low-friction arthroplasty in young patients. 1993 Jul We report the long-term outcome of 218 Charnley low-friction arthroplasties in 141 patients who were 40 years old or younger at the time of surgery. The minimum follow-up was ten years with a mean of 16 years. The probability of the femoral component surviving 20 years was 86% and of the acetabular component, 84%. The chance that both components would survive for this period was 75%. The pathological diagnosis significantly influenced implant survival. In rheumatoid patients the probability of both components surviving at 20 years was 96% compared with 51% in patients with osteoarthritis. Clinical assessment of 103 patients (166 hips) in whom the arthroplasty was still functioning showed that 94% of hips had minimal pain or none. We conclude that in young patients cemented total hip replacement is a good procedure for those with rheumatoid arthritis but that the results are much less reliable in those with osteoarthritis.
8339127 Serological and immunogenetic markers of extraglandular primary Sjögren's syndrome. 1993 Jul The clinical course of 48 patients with primary Sjögren's syndrome (primary SS) was reviewed. Forty-three north European patients were typed for HLA class I and class II alloantigens. In this population with primary SS HLA B8, DR3 and DRw52 all occurred more frequently than in the control population (P < 0.009, P < 0.0035, P < 0.02 respectively). The subgroup of primary SS patients with antibodies to Ro and/or La antigen had the greatest prevalence of DR3 (relative risk 33.4). The primary SS patients fall into two distinct groups: those with extraglandular disease in whom lymphopaenia, hypergammaglobulinaemia, antibodies to Ro and/or La and HLA DR3 were all more frequent and those patients with either glandular disease alone or only one extraglandular feature. There was no difference in disease duration between the two groups, although on average the latter group were 10 years older.
9138387 [Acute methotrexate poisoning: apropos of 16 cases reported to the Paris Poison Control Ce 1996 Sep 16 cases of acute methotrexate (MTX) poisoning were reported to the Paris Poison Control Centre and 62 others were published between 1974 and 1995. Until 1992, MTX was mainly prescribed for neoplastic diseases. Clinical features involve acute renal failure, pancytopenia, and cutaneous or mucous injury. These cases emphasise major risk factors responsible for misuse and overdosage. Since 1992, MTX has been increasingly used in rheumatological or pulmonary diseases (rheumatoid arthritis, steroid-dependent asthma). Most of the overdosages were due to a medical misuse (in the dosage regimen or a misunderstanding of the prescription by nurse, pharmacist or patient). All patients had a bone marrow suppression. The treatment is based especially on early administration of folinic acid rescue.
8146721 [Bronchial hypersensitivity in Sjögren's syndrome]. 1994 Feb It has been reported that Sjögren's syndrome has various pulmonary involvement including lymphoid interstitial pneumonia, lung fibrosis and various pulmonary manifestation. And some abnormalities in respiratory function have been pointed out. Bronchial hypersensitivity has been indicated in patients with bronchial asthma. It has been revealed that it plays an important role in pathogenesis of bronchial asthma. Recently, some authors reported bronchial hypersensitivity in patients with Sjögren's syndrome. We studied bronchial responsiveness to methacholine by using astograph in patients with Sjögren's syndrome. The patients are 25 subjects with Sjögren's syndrome. Bronchial hypersensitivity was seen in 15 patients (60%). And it's pattern was more slight than it of bronchial asthma patients. Our results show bronchial hypersensitivity in Sjögren's syndrome which has been pointed out recently. It may be due to an alteration of bronchial epithelium secondary to lymphocytic inflammation and damage of bronchial glands.
8293606 [Autonomic dysfunction in sensory ataxic neuropathy with Sjögren's syndrome]. 1993 Oct Sjögren's syndrome (SjS) is occasionally associated with chronic progressive sensory neuropathy, and its major pathology is suggested to be dorsal root ganglionitis with the T-lymphocyte invasion. Autonomic dysfunction is frequently accompanied by SjS-associated sensory neuropathy but its underlying pathology is uncertain. We reported four patients with SjS-associated sensory ataxic neuropathy who satisfied the diagnostic criteria of Sjögren's syndrome and also showed a wide variety of autonomic disturbances. The sensory involvement was initially those of multiple mononeuritis in the distal extremities, but in the advanced stage it involved the area distributed along the spinal segmental dermatomes particularly in the trunk. Complete anhidrosis with discrete segmental distribution, as well as absence of response to cholinergic agents, was observed in three cases. Surface skin temperature recorded by a thermoviewer also showed a segmental involvement along the spinal dermatomes in two cases. Adie's pupils were present in two cases. These features suggested that the segmental involvement of the postganglionic sympathetic ganglion cells could be present in the underlying pathological condition. As for the other autonomic dysfunction, two cases showed severe postural hypotension and three cases had an abnormal heart rate variation (R-R interval). Our cases indicated that SjS-associated sensory ataxic neuropathy also manifest variable autonomic disturbances and suggested that similar pathological process to that involving the dorsal root ganglia would be present in the pathology of sympathetic and ciliary ganglion cells.
8168217 [Age-related changes of human lip salivary glands and their values in the diagnosis of Sjà 1993 Oct The lip salivary glands (LSG) from 30 non-autoimmune disease postmortem subjects and 21 biopsies of Sjögren syndrome patients were examined histopathologically. Acinar atrophy, duct dilation, fat tissue hyperplasia, fibrosis and lymphocytic infiltration (including foci) were found to be age-related changes of LSG, and indicate that attention should be paid to distinguishing such aging changes from Sjögren syndrome's histopathological changes, especially in patients over 60 years old. The immunohistological study showed some differences in IgG, IgM and IgA synthesis in the LSG between SS and age-related subjects.
1399434 Fractal dimensions of ductal patterns in the parotid glands of normal subjects and patient 1992 Oct RATIONALE AND OBJECTIVES: The sialographic ductal patterns of the parotid glands in patients with Sjögren syndrome were compared with those of normal patients by measuring the fractal dimensions. METHODS: Fractal dimensions were estimated using the modified pixel dilation method. RESULTS: The mean fractal dimension was 1.64 +/- 0.06 for the normal glands and 1.39 +/- 0.10 for the glands with Sjögren syndrome (P < .005). No correlation between the age or sex and fractal dimension was observed for both groups. In Sjögren syndrome, a significant difference in the fractal dimension was observed between the subgroup having punctuate fillings with a diameter less than 1 mm and the subgroup from 1 to 2 mm (P < .001). CONCLUSION: The fractal dimension is useful as a numeric grading of the complexity of the ductal pattern and the progression of parotid disease.
8023647 Acquired factor VIII:C inhibitor in a patient with Sjögren's syndrome: successful treatme 1994 A 57-year-old woman affected with Sjögren's syndrome without bleeding history developed spontaneous hematomas at the arms, the left foot and the thigh, cutaneous hemorrhages and hematuria. Routine coagulation tests showed a prolongation of activated partial thromboplastin time associated with a marked reduction of factor VIII activity (VIII: C 5%). Other deficiencies of blood coagulation factors, especially von Willebrand factor, were excluded. Measurement of factor VIII inhibitor revealed an activity of 26.4 Bethesda units/ml. These findings were consistent with the diagnosis of acquired hemophilia A due to the presence of a factor VIII inhibitor. The patient was treated with a combination of prednisone and azathioprine. The therapy led, in a few months, to a significant reduction of factor VIII: C inhibitor and she did not require replacement therapy. Furthermore, there was a complete remission of the bleeding tendency. Long-term therapy for about 3 years induced the complete disappearance of the inhibitor and a full normalization of coagulation tests.
9137688 [Sjögren's syndrome and HTLV-I myelopathy]. 1996 The objective of this study is to describe the clinical and immunological aspects observed in patients with both "tropical spastic paraparesis/HTLV-I associated myelopathy" (TSP/HAM) and ocular dryness. In 10 such patients clinical and biological examinations completed with a biopsy of secondary salivary glands were performed to assess the etiology of the ocular dryness. According to the Chisholm's scale, 70% of the patients had a biopsy grade 3 or grade 4. Polyclonal hypergammaglobulinemia was found in 90% of patients and lymphocytic alveolitis in 80%. Three patients had past medical history of chronic uveltis. All the findings were compatible with Sjögren's syndrome, however no characteristic immunological disorders were found. Antinuclear antibodies and rheumatoid factor proved negative in all cases.
7581782 [Deep granuloma annulare in children]. 1995 Sep BACKGROUND: The granuloma annulare is a common benign disorder in childhood which may be difficult to recognize in its atypical forms, like deep granuloma annulare. CASES REPORT: Case 1: A 5-year-old girl suffered from subcutaneous nodules occurring on her soles. Open biopsy at the age of 6 years showed typical features of granuloma annulare. These nodules were still present one year later. Case 2: A 2-year-old girl suffered from nodular lesions on the anterior aspect of the lower legs similar to erythema nodosum with spontaneous outbreaks over 6 months. Histological examination showed typical features of granuloma annulare. CONCLUSIONS: Diagnosis of the deep granuloma annulare is often difficult. Its relation with rheumatic nodules which have similar histological patterns is discussed.
8023070 [Gougerot-Sjögren syndrome. Central neurological involvement with recurrent development]. 1993 A 64-year-old woman was repeatedly hospitalized for various recurrent clinical signs of central nervous system involvement. The diagnosis of primary Sjögren's syndrome was established 3 years 6 months after the onset of the disease. Sicca symptoms, as well as inflammatory biological abnormalities were absent. Moreover, both lacrymal and salivary gland secretions were affected. A high level of antinuclear antibodies to SSA and SSB was associated with inflammatory lesions in minor salivary glands biopsy samples consistent with the diagnosis of Sjögren's syndrome.
8970040 Risk factors for adult Still's disease. 1996 Dec OBJECTIVE: To assess risk factors for adult Still's disease (ASD). METHODS: A matched case-control study of 60 patients with ASD and 60 same sex siblings closest in age was conducted. Subjects were recruited from cohorts in Eastern Canada, Pittsburgh, and the Arthritis, Rheumatism, and Aging, Medical Information Systems (ARAMIS). A questionnaire was used to obtain data on demographic characteristics, education, income, occupation, exposure to toxic substances, stress, and medical history. RESULTS: 116 patients with ASD were identified, of which 104 participated. 86 identified same sex siblings, of which 60 replied. When compared to same sex siblings, ASD patients were similar with respect to education and occupation but had a trend to higher median income. There were no significant associations of ASD with smoking, alcohol consumption, individual toxic substances, vaccination, blood transfusion, minor or major surgery, pregnancy, or diet in the year preceding disease onset. There were no significant associations with tonsillectomy or adenoidectomy, appendectomy, asthma, hay fever, allergy shots, or pregnancy at any time preceding the onset of disease. There was a statistically nonsignificant increase in a history of exposure to coal dust [odds ratio (OR) 3.0; 95% confidence interval (CI) 0.30 to 28.84], in allergy preceding the onset of disease (OR 2.67; 95% CI 0.71 to 10.05), and in oral contraceptive use in the year preceding onset (OR 2.00; 95% CI 0.18 to 22.06). Stressful life events (OR 2.56; 95% CI 1.18 to 5.52) in the year preceding onset was significantly associated with increased risk for ASD. This positive association should be treated with caution unless confirmed by a separate study. CONCLUSION: This exploratory study of risk factors for ASD draws attention to stress as a potentially important risk factor, while likely excluding a considerable number of others.
9171038 Sjögren's syndrome: diagnosis and management of oral complications. 1996 Sep Sjogren's syndrome, a common autoimmune disease process, is characterized by destruction of exocrine (including the lacrimal and salivary) glands. Patients with this disorder may initially complain of a dry mouth or have rapid onset of new carious lesions. Here, a case of Sjogren's syndrome is presented that was initially diagnosed because of dental complaints, and long-term treatment of Sjogren's patients is discussed.
7964153 A severe form of Sjogren's syndrome. 1994 Sep Sjogren's syndrome is an autoimmune disease characterized by exocrine gland destruction and manifested by parotid, submandibular and lacrimal gland infection. We report a case with recurrent severe parotid gland infections. The sialographic and CT findings are presented.
1616370 Primary Sjögren's syndrome preceding the presentation of systemic lupus erythematosus as 1992 Jun The case is reported of a 41 year old white woman who developed systemic lupus erythematosus (SLE) seven years after primary Sjögren's syndrome and four years after the association of Sjögren's syndrome with Jaccoud's arthritis. The SLE was detected by a benign intracranial hypertension, which is a rare neuro-ophthalmic presentation of lupus. No associated conditions linked to benign intracranial hypertension syndrome were seen in this patient and the hypertension syndrome recurred one year later.
9045493 [The cytological findings in decreased tear production in patients with Sjögren's disease 1996 Apr Scrapings off the inferior conjunctival plica were studied in 15 patients with Sjogren's disease and 15 ones with Sjogren's syndrome. The cytology of the conjunctiva in patients with Sjogren's disease or syndrome reliably differed from that in normal subjects. Irrespective of the form and stage of the underlying disease, changes in the conjunctival epithelium of patients with dry kerato-conjunctivitis reflected the processes of squamous-cell metaplasia with loss of goblet cells. Five variants of the cytologic picture of involved conjunctival epithelium are distinguished. The authors come to a conclusion about the efficacy of cytologic studies in a complex of examinations of patients with Sjogren's disease and syndrome.
7971236 [Subacute sensory neuropathies]. 1994 A 52 year old woman, previously healthy apart from a mild chronic persisting hepatitis, developed a sensory ataxia one week after an acute febrile illness. Clinically she had a severe impairment of proprioception and kinesthesia in the arms. Neurophysiological examinations demonstrated absent sensory potentials in the hands. No malignancy was found, and the patient had no sicca symptoms. Some tests for Sjögren's syndrome were positive, but not conclusive. A slight improvement followed after the acute phase. The diagnosis is compatible with an idiopathic subacute sensory neuronopathy.
1475463 [Dermatomyositis, Sjögren's syndrome and vasculitis: an infrequent overlapping connective 1992 Nov A dermatomyositis case with Sjögren's syndrome, Leucocytoclastic vasculitis, and sclerodermiform features is presented. This is a peculiar over lapping connectivepathy in which clinical features of three multisystemic diseases are together. The association of dermatomyositis with Sjögren's syndrome is very rare.