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ID PMID Title PublicationDate abstract
12687042 Immunosuppressive Therapy with Cyclosporin A of Severe Treatment Refractory Juvenile Rheum 1996 Dec Rheumatoid arthritis is severe destructive inflammatory joint disease which usually leads to invalidisation of patient in spite of current therapies. The aim of the trial was to assess the effect of Cyclosporin A (Sandimmun (SIM), Sandos Pharma) in prevention of disability, reduction of inflammatory disease activity, tolerability, safety and suitability for a long treatment in severe treatment refractory juvenile rheumatoid arthritis (JRA). Fifteen patients of 7-15 years old (12 girls and 3 boys), of whom 6 had systemic seronegative and 9 had poliarticular seropositive severe JRA with anatomical stage 2-4 and functional capacities 2-3, were treated with Cyclosporin A in open self-controlled trial of 6 months duration for 15 patients and of 12 months duration for 7, All of them received non-steroid anti-inflammatory drugs and were regularly intra-articularly injected with corticosteroids and 6-prednisolone at a mean dose of 8.0 mg/day. Cyclosporin A was administrated in a dose of 3.5-4.5 mg/kg/day. Efficacy control was carried out according to dynamics of clinical and laboratory criteria of the disease activity, functional capacities and radiographic data. The results of the trial have shown that Cyclosporin A therapy led to statistically significant improvement in almost all clinical outcome variables (the number of swollen joints, p < 0.01, the number of painful joints, p < 0.001; pain score, p < 0.01; duration of morning stiffness, p < 0.001; volume of proximal interphalangeal joints, p < 0.001) and several laboratory parameters (ESR, p < 0.001; IgG level, p < 0.05; 6 seropositive patients became seronegative). All the patients exhibited significant increase in functional abilities and stabilization of joint destruction process. Response was considered as very good in 70% of patients and good in 20%, Prednisolone dose and the number of corticosteroid injections were reduced (p < 0.05). Adverse effects of Cyclosporin A included hypertrichosis, gingivitis, increase in appetite and weight, transitory increase in creatinin serum level of 30% over baseline, relapse of bacterial viral infection. These patients required temporal withdrawal of Cyclosporin A. It is concluded that Cyclosporin A therapy is effective in severe JRA, is well tolerated by patients, is suitable for a long-term treatment and has probable disease modifying effect in JRA.
7488828 Sulphasalazine desensitization in a paediatric patient with juvenile chronic arthritis. 1995 Aug Sulphasalazine is an effective drug for the treatment of rheumatoid arthritis in adults. In paediatric patients, the drug has been used to treat inflammatory bowel disease and is currently under investigation for the treatment of juvenile chronic arthritis. Although sulphasalazine has a rather low incidence of serious side effects, one of the most common is skin rash, thought to be an allergic reaction. In adults, sulphasalazine desensitization programmes have proven to be effective for the treatment of this side effect. We present the case of a 7-year-old boy suffering from HLA-B 27 positive juvenile chronic arthritis. After initiation of treatment with sulphasalazine he developed an allergic skin rash, but tolerated the drug well after completion of a desensitization programme. To our knowledge, this is the first report of a paediatric patient with juvenile chronic arthritis successfully desensitized with sulphasalazine.
1284417 [Angiography in the differential diagnosis of mono-oligo-arthritides in children and adult 1992 The results of the use of angiography in the diagnosis of chronic mono-oligoarthritides are summarized. The author provides the data on 113 patients aged 2 to 50 years, in whom the diagnosis was confirmed histologically. Describes the angiographic appearance of synovial sarcoma, pigmented villonodular synovitis, juvenile chronic arthritis, juvenile rheumatoid arthritis, tuberculous gonitis, chondromatosis, chondroblastomas, post-traumatic synovitis, hemangiomas, and reactive synovitis in the articular form of osteoid osteoma. The use of angiography improved the radiodiagnosis of mono-oligoarthritides by 45.8%. The conclusion is made that in accordance with indications, angiography may be used as a modality in the system of all-round examination of patients suffering from chronic mono-oligoarthritides.
8293808 1 alpha-hydroxyvitamin D3 inhibits type II collagen-induced arthritis in rats. 1994 Jan 17 The effects of 1 alpha-hydroxyvitamin D3 and 24,25-dihydroxyvitamin D3 on Type II collagen-induced arthritis in rats, an experimental model of rheumatoid arthritis, were examined. Oral administration of 1 alpha-hydroxyvitamin D3 significantly suppressed the incidence of arthritis and inhibited hind paw swelling. The level of anti-Type II collagen antibodies was decreased in the 1 alpha-hydroxyvitamin D3 treated-group. In contrast, 24,25-dihydroxyvitamin D3, indomethacin, and gold had no effect on either the incidence of arthritis or the antibody levels. These findings demonstrate a beneficial effect of 1 alpha-hydroxyvitamin D3 on Type II collagen-induced arthritis in rats and indicate that it has an antirheumatic effect.
7595817 Brief report: adherence-facilitating behaviors of a multidisciplinary pediatric rheumatolo 1995 Jun Investigated the behaviors of pediatric rheumatology health care providers that were expected to be related to patient or parent adherence. Medical charts of 108 patients ages 1 to 20 years diagnosed with Juvenile Rheumatoid Arthritis were examined. The 473 outpatient visits over 15 months yielded a total of 2,578 treatment recommendations, but only 1,390 adherence-facilitating behaviors by medical staff were documented. Providing information about how often to perform the recommendation was the most common staff behavior. In contrast, care providers rarely indicated that they addressed their patients' concerns and barriers to implementing the recommendations, or employed behavior modification strategies to increase adherence. Implications of these findings for development of programs designed to increase treatment adherence in children with chronic diseases requiring time-consuming, intrusive medical regimens are discussed.
1533960 Lymphocyte subsets and mitogen induced proliferation in adjuvant arthritis. Part I. Migrat 1992 Mar Adjuvant arthritis (AA) was used as an animal model of rheumatoid arthritis in this study. Seventy 10-week-old inbred female rats of both Long-Evans rats (LE, high responder of adjuvant arthritis) and Sprague-Dawley rats (SD, low responder of adjuvant arthritis) were inoculated with adjuvant. Using monoclonal antibodies, we demonstrated that redistribution, migration, or an imbalance of T lymphocytes rather than the change of total T cells are involved in the pathogenesis of AA. After the adjuvant injection, the lymphocytic proliferative responses of LE and SD rats to phytohemagglutin (PHA) had no significant difference, whereas the response of LE rats to concanavalin A (ConA) was significantly lower than that of SD rats. In addition, a highly significant positive correlation was found between the absolute numbers of helper T cells and the lymphocytic proliferative response to PHA, and between absolute numbers of suppressor T cells and the lymphocytic proliferative response to Con A. We postulated high responder strain LE would have a defect in suppressor T lymphocytes leading to severe arthritis, while in low responder strain SD, adjuvant-activated suppressor T lymphocytes might reduce the severity of arthritis.
8934918 The use of whole saliva in the differential diagnosis of Sjögren's syndrome. 1996 Apr Sjögren's Syndrome (SS) is a chronic, multisystem, autoimmune disorder. It is characterized by (1) generalized exocrine gland dysfunction, (2) serologic abnormalities, and (3) organ-system changes. Oral changes are a prominent feature of this disease. Among these are xerostomia and hypofunction of the salivary glands. Given the intimate relationship between SS and the salivary glands, it is reasonable to postulate that whole saliva (WS) contains the stigmata associated with the presence of this disease. But few studies have been conducted on this secretion. Indeed, WS has largely been neglected and ignored by physicians, dentists, and scientists. Objections to its use have included the fact that it is "impure", that it does not adequately represent what is present in the salivary glands, that no standards have been established for its rate of flow, and that findings based on it lack specificity. Yet, it is this secretion which coats and protects the hard and soft oral tissues, enables us to prepare our food for digestion, and assists our speech. This review will demonstrate that there is a uniqueness and constancy to whole saliva and that it may be used to diagnose the presence of SS. "Screening tests", which include several simple-to-perform sialometric, chemical, and microbiologic procedures, may be conducted in doctors' offices to establish the "profile" of an SS patient. Electrophoretic studies may be used to study the nature of the salivary proteins, and an immunologic test, which is performed on WS and utilizes Western Blot Autoantibody Strips (ImmunoVision, Springdale, AR), may be used to establish the definitive diagnosis of SS.
8531349 [Autoantibodies in patients with Sjögren's syndrome and their clinical significance]. 1995 Oct A variety of antinuclear antibodies were examined in 136 patients with primary Sjögren's syndrome, 131 patients with secondary Sjögren's syndrome and 139 patients with various connective tissue diseases without Sjögren's syndrome. Among these, anti SS-A/Ro antibodies were found most frequently in secondary Sjögren's syndrome, whereas the incidence of anti SS-B/La antibodies, associated with anti SS-A/Ro antibodies, was highest in primary Sjögren's syndrome. In addition, anti RNP or anti centromere antibodies were also found in some patients with primary Sjögren's syndrome. Clinical characteristics related to individual antinuclear antibodies are also described.
7635878 Non-neoplastic tumors of the hand and upper extremity. 1995 May A variety of non-neoplastic, tumor-like conditions exist in the upper extremity. Some are common, others rare. Careful history, physical diagnosis, and a variety of imaging modalities can be helpful in arriving at an accurate diagnosis and developing a successful treatment plan for this group of lesions.
8299277 [A case of progressive systemic sclerosis with Sjögren's syndrome presenting with coma, c 1993 Sep A 35-year-old female with progressive systemic sclerosis (PSS) and Sjögren's syndrome developed consciousness disturbance and generalized seizure after the episode of fever and erythema lasting for 3 weeks. Neurological examination disclosed deep coma and spastic tetraplegia with pathological reflexes. Laboratory data showed mild anemia, severe hypoproteinemia, hypoalbuminemia and increase of protein content in the cerebrospinal fluid. Cranial CT scans obtained after convulsion revealed diffuse brain swelling and bilateral symmetrical hypodensity involving the thalami and posterior limbs of the internal capsule. High-dose corticosteroid therapy and osmotherapy with correction of hypovolemia were started, because severe dehydration, hypoalbuminemia and cerebral vasculitis were suspected to change the vascular permeabilities. Neurological symptoms and CT findings were rapidly improved. Cerebral angiography 4 weeks after convulsion showed definite angitis. The patient was discharged 9 weeks after convulsion. In our case, a possible cause of the characteristic CT findings may be the disturbance of cerebral venous return due to hypovolemia and vasculitis. High-dose corticosteroid therapy may be recommended in patients with collagen disease, who show the bilateral symmetrical thalamic hypodensity on CT scans.
8269779 [Epidemiological studies on primary Sjogren's syndrome]. 1993 Aug 2,066 adult people of a Beijing suburb village were surveyed for primary Sjogren's syndrome, using questionnaire and serological tests in each case as the primary screening tool. 16 cases were discovered by Copenhagen criteria indicating a prevalence rate of 0.77% and 7 cases with a prevalence rate of 0.33% by Fox criteria. Similar study was done in 100 non-connective tissue diseases in-patients. 4 cases were discovered by Copenhagen criteria and one case by Fox criteria. In 126 definite cases of primary Sjogren's syndrome, the longest time interval between first onset of symptoms and firm establishment of diagnosis was 20 years with an average of 7.8 years indicating most cases were wrongly diagnosed or highly neglected.
8350331 Sjögren's syndrome in patients with interstitial cystitis. 1993 Jun OBJECTIVE: Patients with interstitial cystitis, a chronic nonbacterial inflammation of the bladder, were investigated for the presence of systemic autoimmune diseases, in particular for Sjögren's syndrome (SS). METHODS: Ten patients were included in the study on the basis of a diagnosis of interstitial cystitis according to usual criteria. They underwent clinical and laboratory investigations including those for keratoconjunctivitis (KCS) and focal lymphocytic sialoadenitis (FLS). RESULTS: In 2 patients both KCS and FLS were present allowing the diagnosis of primary SS according to classification criteria. Additionally, in 6 patients one of the 2 hallmarks of primary SS was present, KCS in 3 and FLS in 3. CONCLUSION: Interstitial cystitis is a new example of a disease that occurs in association with SS, supporting the concept that interstitial cystitis is an autoimmune cystitis. The clinical relevance of the finding is that a high index of suspicion for SS is indicated in patients with interstitial cystitis.
8645490 [Lip biopsy of minor salivary glands in Sjogren's syndrome: histological findings and clin 1996 Jan Thirty-seven lip biopsies of minor salivary glands from patients with suspected Sjögren syndrome (SS) and no other known disease were studied. Two cases were excluded for not meeting disease criteria (one had histologic findings of sarcoidosis). Of 35 SS cases, 4 were considered secondary to associated disease. In 24 cases the Daniels technique was used and in 11, an elliptical excision. In 3 elliptical excisions (27.3%) the biopsy did not contain glands (p = 0.002 compared with the Daniels technique). The mean number of glands per biopsy was 7.7 (2-16) with the Daniels technique and 5 (0-15) by elliptical excision (no significant difference). Focal lymphoid sialoadenitis was present in all 32 gland-bearing specimens. Multiple inflammatory foci were found in 28 (87.5%), 8(25%) of which had a diffuse infiltrate. SS presented more frequently as xerostomy in patients with diffuse infiltrate (p = 0.09). In 11 lip biopsies from controls with other diseases of the oral cavity, 7 (63.6%) showed changes suggestive of SS.
1496168 Diffuse infiltrative lymphocytosis syndrome: a disorder occurring in human immunodeficienc 1992 Aug Certain adults and children infected with the human immunodeficiency virus(HIV)-1 developed a disorder termed diffuse infiltrative lymphocytosis syndrome (DILS) that, although having certain similarities to classic Sjögren's syndrome, manifests distinctive clinical, serologic, immunologic, and immunogenetic characteristics. DILS is characterized by circulating CD8 lymphocytosis and apparently antigen-driven CD8 T-cell infiltration of salivary and lacrimal gland, pulmonary, renal, gastrointestinal, and breast tissues. The disproportionately greater degree of salivary gland enlargement and extraglandular disease, as well as the low frequency of autoantibodies and differing HLA associations, serve to distinguish DILS from classic Sjögren's syndrome.
7495349 Increased serum IgE level and interleukin-4 release from cultured lymphocytes from a patie 1995 Sep OBJECTIVE: To evaluate the relationship between high serum levels of IgE and the release of interleukin-4 (IL-4) from cultured lymphocytes of a patient with adult onset Still's disease. METHODS: IL-4 concentrations in plasma and culture from inactivated peripheral blood mononuclear cells were assessed by enzyme immunoassay during febrile episodes and remission. RESULTS: A high level of IL-4 was detected by enzyme immunoassay in the peripheral blood mononuclear cells cultured from the patient. These seemed to correspond with a febrile episode and a high serum IgE concentration. CONCLUSION: Increased serum IgE concentrations during a febrile episode are rare in patients with adult onset Still's disease, but the relationship between the high levels of serum IgE and IL-4 in cultured lymphocytes may provide clues to pathogenesis of the condition.
8358981 Echocardiographic findings in patients with primary Sjögren's syndrome. 1993 Jun Cardiac involvement in 27 consecutive patients fulfilling the criteria for primary Sjögren's syndrome was evaluated using echo-doppler-cardiography. A clinical physical examination showed that the heart was normal in all patients. Nine (33%) had signs of present or previous pericarditis with effusion and/or fibrin deposition, four of them had chest symptoms. The pericarditis patients were slightly older, had significantly shorter disease duration, had significantly increased levels of orosomucoid and haptoglobin, and were significantly more often antinuclear-antibody positive than those without pericarditis. Of the echocardiographic measurements, the left ventricular systolic dimension was significantly smaller and the fractional shortening of the left ventricle significantly higher in the pericarditis patients. However, four of the nine pericarditis patients had localized hypokinesia of the left ventricle, all with unspecific ECG changes, while only one without pericarditis showed this symptom. No patient had low voltage, ST-T elevation or conduction abnormalities. Mitral valvular thickening was found in one patient and age-related sclerosis of the aortic cusps appeared in some.
1405090 [A case of systemic lupus erythematosus associated with Sjögren's syndrome diagnosed by l 1992 Apr A 30-year-old female, diagnosed as having SLE and followed at our hospital while receiving oral prednisolone (5 mg/day) for about 6 months, was admitted because of fever and productive cough. Chest X-ray and CT scan showed a tumor-like homogeneous shadow in the right middle lobe. Antibiotic treatment was not effective. Specimens obtained by TBLB revealed massive infiltration of mature plasma cells and lymphocytes into the alveolar septa and interstitial tissues. These findings were considered compatible with lymphoid interstitial pneumonia. Associated conditions such as Sjögren's syndrome were suggested, but the diagnostic criteria of Sjögren's syndrome were not satisfied. Both the abnormal shadow on chest X-ray and the clinical symptoms markedly improved by increasing the dose of prednisolone (20 mg/day). After 3 months, however, bilateral BAL findings showed alveolar lymphocytosis, providing evidence that subclinical diffuse pulmonary involvement had continued. This case indicates that radiological appearance may vary depending on the extent of lung involvement, even if the histological features are identical. It is concluded that the present diagnostic criteria of Sjögren's syndrome must be reconsidered so as not to overlook subclinical and asymptomatic cases of Sjögren's syndrome.
1606727 A somatically mutated V kappa IV gene encoding a human rheumatoid factor light chain. 1992 Jun The light chain of an IgA kappa rheumatoid factor (RF) produced by a hybridoma derived from a patient with rheumatoid arthritis (RA) has been shown to belong to the V kappa IV family. This RF light chain has 31 nucleotide differences compared with the single V kappa IV germline gene reported for the human genome. The patient's V kappa IV germline gene was sequenced, using the polymerase chain reaction (PCR), and shown to be identical to that previously reported. This demonstrates that the RF light chain is the product of a somatically mutated gene. A comparison with other known V kappa IV sequences shows that the RF light chain has more replacement mutations than most of the known V kappa IV light chains.
8926981 [The imaging diagnosis of Sjögren's syndrome: echography, sialography and scintigraphy co 1996 Apr The aim of the present study was to evaluate the sensitivity and specificity of the ultrasonography, in comparison with other methods of investigation (scintigraphy, sialography, and biopsy), in scanning morphostructural changes in the parotid gland in patient with Sjögren's syndrome. During the period June-October 1994, 34 patients (5 males and 29 females, age ranged between 20 and 88 years) with "sicca syndrome" underwent to echography, scintigraphy, sialography and biopsy. The diagnosis was confirmed or excluded using the European Community Epidemiologic Committee criteria for Sjögren's syndrome. Twenty-two patients out of 34 were affected by Sjögren's syndrome, while the others resulted as control subjects. The ultrasonographic investigation has shown 76.19% of sensitivity and 30.43% of specificity. Even if echography is a non-invasive method, which could be used as preliminary approach for studying the diffused involvement of the parotid gland, at the status of the art, it is not completely reliable for the global evaluation of the morphostructural changes in patients with Sjögren's syndrome, in comparison with the other techniques. Because of the double nature of the gland injury, it appears to be essential the diagnostic integration between echography and sialography.
8777844 Tear and saliva ferning tests in Sjögren's syndrome (SS). 1996 Mar The purpose of this study was to evaluate the tear ferning test (TFT) and the saliva ferning test (SFT) as diagnostic tests for xerophthalmia and xerostomia respectively in patients with SS. Dried samples of freshly produced tears and saliva from: (A) 36 healthy controls without sicca symptoms, (B) 61 patients with primary SS, (C) 53 patients with secondary SS and (D) 22 patients with psychiatric disorders receiving antidepressants, were examined by polarizing light microscopy. All individuals included in the study were postmenopausal women. The crystallization was classified into 4 types according to the ferning phenomenon: uniformity, branching, spreading and integrity (type I normal and II, III, IV abnormal). Abnormal TFT was found in tear samples of: (A) 8/72 normal control eyes, (B) 110/122 prim SS eyes, (C) 86/106 sec SS eyes and (D) 30/44 psychiatric patients' eyes. Abnormal SFT was found in: (A) 6/36 of normal controls, (B) 59/61 of prim SS, (C) 51/53 of sec SS patients and (D) 16/22 of psychiatric patients. The differences of both TFT and SFT of patients with prim SS and sec SS versus controls were significant (p < 0.001). The sensitivity of TFT was found to be high both in prim SS (90%) and in sec SS (81%). In addition the specificities of TFT and SFT were high 89% and 83% respectively. On the other hand the differences of TFT and SFT in psychiatric patients receiving antidepressants versus normal controls were also significant (p < 0.05 and p < 0.025 respectively). According to our data TFT and SFT are simple, sensitive and specific tests to evaluate xerophthalmia and xerostomia in SS and probably in other conditions characterized by mucous membrane dryness.