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ID PMID Title PublicationDate abstract
19078491 Arthritis in leukemia. 2000 Dec The clinical features of 19 patients who had arthritis and leukemia were reviewed and compared with those without arthritis. There was leukemic arthritis (characterized by absence of other evident causes and response to chemotherapy) in 14, septic arthritis in 4, and hemarthrosis in 1. Among those with leukemic arthritis, there was acute leukemia in 11 and chronic leukemia in 3. The arthritis presented before, simultaneously with, and after the diagnosis of leukemia in 5, 4, and 5 cases, respectively. Acute symmetrical polyarthritis mimicking rheumatoid was the most common presentation. The knee, wrist, and ankle were most commonly involved. Adegree of pain, which was out of proportion to the degree of inflammation, could be seen occasionally. Synovial fluid blast cells were identified in 33% of cases. The leukemic arthritis responded well to chemotherapy. Septic arthritis was an initial presentation of leukemia in 2 patients. There was no significant difference in hematologic parameters or in mortality rate between those with and without arthritis. In conclusion, symmetric polyarthritis mimicking rheumatoid and septic arthritis could be an initial presentation of leukemic arthritis. One should have a high index of suspicion in patients with acute arthritis, especially if there is already anemia and pain is out of proportion to swelling.
11680402 Osseointegrated silicone implants for joint reconstruction after septic arthritis of the m 2001 Sep Seven men mean age 37 years (range 24-45) who had destroyed metacarpophalangeal joints as a result of pyogenic infection underwent reconstruction of the joint with osseointegrated silicone implants. The interval from infection to reconstruction varied from 5 months to 13 years. At mean follow-up 10 (8-12) years postoperatively the titanium fixtures were all well osseointegrated. The silicone spacer had fractured in one case. The extension lag was 16 (10-20) degrees and the range of movement (ROM) 34 (30-40) degrees. Our data confirm previous observations about the successful long-term osseointegration of titanium fixtures used for the attachment of joint prostheses in the hands of patients with rheumatoid arthritis or osteoarthritis. We conclude that titanium fixtures can be used successfully to fix joint mechanisms for reconstruction of small joints after pyogenous arthritis of the hand and can be used for fixation of joint mechanisms in such cases.
10337932 Gene therapy of arthritis. 1999 Mar Genes encoding anti-arthritic products can be transferred to intra-or extraarticular sites where their expression suppresses various aspects of the pathophysiology of arthritis. A variety of viral and non-viral vectors can be used for the in vivo or ex vivo delivery of such genes. Promising pre-clinical data have resulted from the application of these strategies in several animal models of disease. Genes showing efficacy in this way include these encoding interleukin (IL) -1Ra, IL-1sR, TNFsR, transforming growth factor beta (TGF-beta), IL-13, Fas L, IL- 10 and vIL-10. Two human arthritis gene therapy protocols are underway in the USA and Germany. Both studies involve the ex vivo transfer of an IL-1Ra cDNA to the metacarpophalangeal joints of patients with rheumatoid arthritis. Progress in developing gene treatments for arthritis has been rapid, and permits optimism about their ability eventually to improve the treatment of this group of diseases.
9316728 Cervical spine fusion: not protective of cervical spine injury and tetraplegia. 1997 Sep The occurrence of tetraplegic spinal cord injuries in two patients who had preexisting cervical spine fusions highlights the vulnerable nature of such patients. One patient sustained a C5-6 fracture-dislocation after a motor vehicle accident, despite a spontaneously fused cervical spine as a consequence of his juvenile rheumatoid arthritis. A second patient sustained a C3-4 distraction injury in a sporting injury 2 years after he had undergone a posterior cervical spine fusion because of an aneurysmal bone cyst. Prior to their spinal cord injuries, both patients had no apparent neurologic deficits, were relatively active, and followed no specific precautions for their spines.
11324795 Clinical utility of diagnostic tests for rheumatoid factor. 2001 OBJECTIVE: To investigate and compare the accuracy and usefulness of diagnostic tests for rheumatoid factor (RF). METHODS: In a cross-sectional study sera derived from patients admitted to the Section of Rheumatology were tested for presence of RF using either nephelometry or the Waaler test. Diagnostic sensitivity and predictive values of the tests were calculated and compared. The accuracy of the tests was compared using receiver-operating characteristics (ROC) methodology. RESULTS: Good agreement was found between the tests (kappa approximately 0.7). At cut-off 19 IU/mL nephelometry showed the highest sensitivity (82.4%) and specificity (95.9%) for rheumatoid arthritis (RA). In comparison, the Waaler test had a sensitivity of 60.3% and specificity of 95.9% at cut-off titer 128. The tests showed nearly equal performance characteristics when predicting SS. CONCLUSION: Although both tests exhibit good performance characteristics, nephelometry has a higher accuracy when predicting RA and SS. The common practice of using both tests for detection of RF is not recommended.
11798600 [Clinical and pathologic analysis of Sjögren's syndrome with renal impairment: a report o 2001 Jun OBJECTIVE: To further study the renal damage of Sjögren's syndrome (SS) and its clinical and pathologic characteristics with biopsy and recent technology. METHODS: 84 patients of SS with renal impairment from 1993 to 1999 were analyzed by routine, immunoassay, tubular function and biopsy examination. RESULTS: 55 of the 84 cases presented with renal tubular acidosis (RTA), 5 with diabetes insipidus and 3 with hypokalemic paralysis. Glomerulopathy occurred in 22 cases (nephrotic syndrome 12, glomerulonephritis 10) and mild renal failure (RF) was found in 14. 69.1% of the patients had hypergamma-globulinemia. 64.3% and 44.1% of the patients showed positive anti SS-A and anti SS-B. In 37 renal biopsy specimens 21 showed chronic interstitial nephritis (CIN) with extensive lymphoplasmic cell infiltration and tubular atrophy. 10 of the 37 specimens revealed lupus nephritis (LN, type III and IV) and 5 mesangial proliferative glomerulonephritis (MsPGN). With immunofluorescence tests, no positive findings were seen in most of the specimens, but deposition of IgA, IgM or C(3) was seen in some patients. IgG deposits in the interstitial lymphoplasmic cells were found in 1 patient. 25 patients were treated with prednisone combined with cytoxan (CTX). In 14 patients with renal failure, serum creatinine level returned to normal after treatment. CONCLUSION: Renal impairment may be the presenting or predominant feature in SS. The major clinical manifestations are RTA and GN. Treatment with prednisone may decrease the infiltration of lymphoplasmic cells in the interstitium, improve the renal function and correct RTA.
11685093 Lung manifestation in asymptomatic patients with primary Sjögren syndrome: assessment wit 2001 Oct The authors studied 37 consecutive patients with primary Sjögren syndrome and normal chest radiographs. Thin-section CT images were analyzed using a semiquantitative grading system. The presence, distribution, and severity of 9 morphologic parameters were assessed. In 34 patients, CT findings were correlated to pulmonary function tests (PFTs). Abnormal high resolution CT (HRCT) findings were seen in 24 of 37 patients (65%): interlobular septal thickening, n = 9; micronodules, n = 9; ground glass attenuation n = 4; parenchymal cysts, n = 5. Intralobular opacities, honey combing, bronchial wall thickening, bronchiectasis, and pleural irregularities were less frequent. Both HRCT and PFTs were normal in 10 patients. Computed tomography was normal in four patients with PFTs that indicated the presence of small airway disease. High resolution CT abnormalities were found in seven patients with normal PFT. The overall correlation between HRCT and PFTs was poor. High resolution CT and PFTs appear to be sensitive for both the early detection of parenchymal abnormalities and a decreases in lung function in asymptomatic patients with primary Sjögren syndrome. However, abnormal HRCT findings do not necessarily indicate a substantial alteration in PFTs.
9884552 [Peripheral neuropathy and dysautonomia in a patient with primary Sjögren's syndrome]. 1998 Feb A 46 year-old female presented with dry eyes and a dry mouth which she had been experiencing for about 15 years. She also began to notice dizziness (orthostatic hypotension) during the last 5 years. The symptoms gradually increased whereupon she began to have polyarthralgia, facial flashing, hyper and hyposweating in some areas on the face and trunk. Her sialography showed a damaged parotid gland. Minor salivary gland biopsy revealed chronic sialoadenitis. The Sirmer test was low, and the Rosebengal test indicated keratoconjunctivitis sicca. Her serological tests showed hypocomplementemia and were positive for antinuclear antibody and SS-A antibody. The diagnosis of primary Sjögren's syndrome (SjS) was made based on these findings. Prednisolone, at a dose of 15 mg per day, was given orally. As a result of this therapy, arthralgia disappeared immediately, although it had no effect on the neuropathy found in this patient. This is a rare case of SjS associated with peripheral neuropathy and severe dysautonomia.
9506885 Sjögren's syndrome and pernicious anaemia. 1998 We describe seven patients (five females and two males) with pernicious anaemia and Sjögren's syndrome. The diagnosis of pernicious anaemia was confirmed by bone marrow aspiration and a Schilling's test. Sjögren's syndrome was diagnosed by the Schirmer-I tear test, salivary gland scintigraphy, minor salivary gland biopsy and determination of lacrimal lysozyme. We have reviewed the literature and found few cases reported of the association of these two diseases.
9200940 [Potential role of Epstein-Barr virus in Sjögren's syndrome]. 1997 Jun In this review, I discuss recent progress in our understanding of the role of Epstein-Barr virus on pathological conditions in Sjögren's syndrome (SS). In order to clarify the association of EBV in SS pathogenesis and further analyze the precise transcriptional mechanism of EBV reactivation, we assayed transcription of ZEBRA which is the fast transcribed EBV-encoded immediate early gene product and was an indispensable role in EBV reactivation. The ZEBRA expression was observed in the ductal epithelial cells and infiltrating B cells, and SS peripheral blood lymphocytes (PBL). On the other hand, to identify the inducer of EBV reactivation, we have used in vitro EBV reactivation model cell line. Screening of cDNA by mRNA differential display subtraction method, one clone, named "AK-1", was highly expressed in SS PBL. Nucleotide sequence analysis revealed that AK-1 was complete match with p300/CBP-associated factor (P/CAF). Furthermore, as another approach, we have partially cloned cDNA with a novel sequence containing a region homologous to the basic leucin zipper domain of CREB/ATF family by degenerate PCR. Our results together with recent reports, suggest the possibility that CREB family protein-p300-P/CAF ternary complex on ZEBRA promoter might be involved in ZEBRA transcriptional activation.
9587591 [Bilateral optic neuropathy disclosing primary Gougerot-Sjögren syndrome]. 1997 A case of optic neuropathy and central nervous system disease developed in association with primary Sjögren's syndrome is described. Initial visual loss was partially reversible with intra veinous steroid therapy but four months later she underwent a controlateral visual loss and showed for the first time clinical manifestation of sicca syndrome. Fluorescein angiography showed signs of ischemic choroidopathy. Laboratory studies and histological findings on minor salivary gland biopsy was suggestive of primary Sjögren's syndrome. Attempts to taper oral steroid therapy resulted in worsening neurological disease and immunosuppressive therapy by azathioprine was successfully added to her regimen. Because of therapeutics and prognosis consequences, primary Sjögren's syndrome should be considered in differential diagnosis of optic neuropathy.
9890100 Eye involvement in children's rheumatic diseases. 1998 May Rheumatoid paediatric diseases are a leading cause of uveitis in childhood. Juvenile chronic arthritis (JCA), juvenile onset spondyloarthropathies as well as sarcoidosis and other systemic diseases with arthritis may include ocular manifestations that can threaten vision, and especially so in juvenile chronic arthritis. Special risk factors concerning the eye have to be considered for JCA. The diagnosis, detection, follow-up studies and treatment in children may differ significantly from adult rheumatoid diseases because of the young age of the patients and the specific features and signs of ocular involvement. Medical and surgical treatment of such ocular manifestations may be challenging. Special attention to children's ophthalmic complications must be undertaken by paediatricians and ophthalmologists.
11469515 The treatment of Sjögren's syndrome patients with pilocarpine-tablets. 2001 Therapeutic progress in Sjögren's syndrome has lagged behind our recognition and understanding of clinical manifestations and disease pathogenesis. Past treatment of sicca symptoms with immunosuppressives has been unsuccessful. The use of secretagogues (e.g. pilocarpine-HCl) to stimulate flow from exocrine glands represents a novel approach that has dramatically altered our management strategy for Sjögren's syndrome. Clinical studies with pilocarpine (Salagen) tablets in the United States have demonstrated significant subjective and objective benefit for xerostomia and related oral symptoms at doses of 20 mg/day or more. Symptomatic relief of xerophthalmia and other xeroses due to Sjögren's syndrome may also occur. Data from human and animal models suggest that regular use of pilocarpine may not only improve patient quality of life but potentially prevent complications as well. Better methods of patient selection for therapy are needed since clinical response to pilocarpine cannot be predicted based on the duration of patient symptoms.
11403269 Pinguecula and Sjögren's syndrome: two cases. 2001 A pinguecula is a small, raised conjunctival nodule at the temporal or nasal limbus classically associated with conjunctival microtrauma from exposure to sunlight and/or to dust. We report two cases occurring during the course of Sjögren's syndrome. To our knowledge this association has never been described.
11268409 Neuroendocrine manifestations in Sjögren's syndrome. Relation to the neurobiology of stre 2000 Evidence suggests that autoimmune rheumatic diseases are associated with neuroendocrine dysfunction. Sjögren's syndrome (SS) is proposed as an ideal model to study perturbations in the neuroimmune axis, since patients tend to be medication free and studies are not confounded by the effects of chronic immunosuppressive therapy. The functional integrity of the adrenal, gonadal, and thyroid axes was assessed in SS. Pituitary function of the HPA axis was evaluated directly by determining the ACTH released during oCRH stimulation, while adrenal function was assessed indirectly by endogenous ACTH released during oCRH stimulation. Low basal activity of the HPA axis was associated with pituitary hyporesponsiveness to exogenous CRH, as well as hyporesponsiveness of the adrenal glands to endogenous ACTH. These findings are compatible with a central deficiency of the adrenal axis. An overall attenuated and delayed LH and FSH response to LHRH stimulation was also indicative of central dysfunction of the gonadal axis in SS. SS patients demonstrated elevated basal TSH levels and evidence of mild hypothyroidism. Basal prolactin concentrations were also elevated in SS, and both TSH and PRL showed relatively increased responses to TRH stimulation. The data suggest a central deficiency in all three neuroendocrine axes: adrenal, gonadal, and thyroid. It is not clear if any one system plays a primary role in the expression of the disease. Rather, it is likely that the net effect involves the synergistic and antagonistic effects of multiple hormones. Taken together, adrenal and gondadal steroid hormone deficiency, plus elevated PRL levels, probably greatly affect immune function in SS patients.
10813292 Salivary gland ultrasonography in the evaluation of primary Sjögren's syndrome. Compariso 2000 May OBJECTIVE: To evaluate the diagnostic accuracy of ultrasonography (US) of the salivary glands versus histologic examination in detection of salivary gland involvement in primary Sjögren's syndrome (SS). METHODS: Thirty female patients with primary SS diagnosed according to the European criteria and 30 female subjects of similar age with dry mouth (symptomatic controls) due to other selected disorders were studied. Evaluation of salivary involvement was by 2 independent observers for both the parotid and submandibular glands using US score (range 0 to 4) that assigns points to the different degree of glandular inhomogeneity. Lip salivary gland (LSG) biopsies were graded according to the Chisholm and Mason classification system and by morphometric analysis. The area under receiver operating characteristic (ROC) curve was employed to evaluate the screening method's performance. RESULTS: Through ROC curves salivary gland US arose as the best performer, followed by the volume percentage of inflammatory cells and by the semiquantitative focus score. The difference between area under ROC curve of salivary gland US and lymphocytic focus score was significant (p = 0.044), reflecting the accuracy of the imaging diagnostic assessment. No differences arise between areas under the ROC curve of salivary gland US and volume percentage of inflammatory cells (p = 0.610) and between volume percentage of inflammatory cells and semiquantitative focus score (p = 0.081). Attempts to assess the independent contribution of the fractional composition of the LSG biopsy as the predictor of US score showed a significant contribution of both the fractional composition of inflammatory infiltrate (p = 0.01) and intralobular ducts (p = 0.02). These findings indirectly suggest that the US abnormalities we found were strongly related to SS. CONCLUSION: These results conclusively indicate that quantitative assessment of salivary gland ultrasonography is a very useful method fdr evaluating salivary gland involvement in SS and can replace other diagnostic techniques, such as sialography or salivary scintigraphy.
9410615 [Oral manifestations of autoimmune diseases with sicca symptoms. Comparative studies of pa 1997 Mar Sjögren's syndrome is a chronic autoimmune disease which mainly affects exocrine glands. We present a study where 123 patients with Sjögren's syndrome were examined according to their oral symptoms. A high percentage of patients showed mild or moderate inflammation of the oral mucosa. Remarkable is the high number of extracted teeth in older patients with Sjögren's syndrome. Oral ulcerations and erythema were found only in the group with secondary Sjögren's syndrome and systemic lupus erythematosus.
9812558 [Renal involvement in primary Sjögren's syndrome: a clinicopathological study of 26 cases 1997 Previous study suggested that renal involvement in primary sjögren's syndrome (SS) was rare and renal damage in SS cases has not been reported in Chinese literature. In this study the clinical and pathological features in 26 primary SS patients (23 female and 3 male, with an average age of 40.9 years) were analyzed. The spectrum of renal disorders in these patients was as follows: renal tubular acidosis (20/26), hypokelemia (18/26), hypocalcemia (14/26), mild proteinuria (less than 1.5 g/d, 12/26), massive proteinuria (more than 1.5 g/d, 9/26, including 5 nephrotic syndrome), and renal insufficiency (5/26). Light microscopy (LM) might show acute or chronic interstitial nephritis (IN, 10/26), or IN accompanied with focal glomerulonephritis (FGN, 4/26), focal segmental glomerulosclerosis (FSGS, 4/26), membranous nephropathy (MN, 3/26), diffuse mesangial proliferative glomerulonephritis (MePGN, 1/26) and membranoproliferative glomerunephritis (MPGN, 1/26) in these SS patients. A variable degree of interstitial lesion was also noted in all patients with glomerular damage. Immunofluorescent examination revealed deposits of IgG (9/24), IgA (8/24), IgM (7/24), C3 (10/24) and C1q (2/24) in granular distribution on the glomerular basement membrane (GBM) or focal masangium and focal deposits of IgG and C3 (7/24) on the tubular basement membrane (TBM). These results suggested that renal tubular acidosis is the most common finding in Chinese SS patients with renal involvement, but glomerular damage is not uncommon. Although IN was the major pathological manifestation in SS patients, FGN, FSGS, MN, MePGN and MPGN might also be found. The exact incidence of secondary glomerular disease in primary SS needs to be studied further.
9423337 [Disseminated intravascular coagulation in a case of adult onset Still's disease]. 1997 Nov We report a 82-year-old woman with adult onset Still's disease (AOSD), who presented with high fever, skin rash, swollen axillary lymph nodes, accelerated erythrocyte sedimentation rate, leukocytosis, abnormal liver function tests, hypoalbuminemia, negative antinuclear antibody and rheumatoid factor, and lack of renal involvement. Disseminated intravascular coagulation (DIC) was also diagnosed on admission. An antipyretic relieved high fever and DIC soon improved. Three years later, AOSD relapsed accompanied by hypercoagulation and hyperfibrinolysis. The patient developed subdural hematoma and DIC due to a brain contusion. High titers of serum soluble adhesion molecules and soluble thrombomodulin were noted on the first episode of DIC. These findings indicated that endothelial cells were damaged in AOSD complicated by DIC.
11434476 Ultrasonography and colour doppler sonography of salivary glands in primary Sjögren's syn 2001 To examine either the ultrasonographic (US) features of the parotids and submandibular glands or the blood flow alterations that may occur in the salivary glands of patients with primary Sjögren's syndrome (pSS) we studied 30 female patients with pSS and 30 controls suffering from dry mouth not due to pSS. All measurements were taken by the same examiner, who used the same equipment to avoid interobserver variability. The US parameters recorded (parenchymal homogeneity, echogenicity, size of the glands and posterior glandular border) were scored according to a previously described scoring system. For each waveform, peak systolic velocity (PSV) and resistive index (RI) were measured at the external carotid artery in the examination of the parotids and at the facial artery within the submandibular glands before and during lemon juice stimulation. On the basis of the degree of chronic inflammatory changes at minor salivary gland (MSG) biopsy, chronic sialadenitis (CS) was defined as mild in 10 and severe in 20 pSS patients. Abnormal US scores were obtained in 26/30 (86.6%) pSS patients and in 9/30 (30%) controls. Moreover, in pSS patients the US scores were sigificantly higher than in the control group (p=-0.0001). The mean (+/- SD) difference between the PSV values taken from parotids and submandibular glands before and during lemon juice stimulation was statistically significant (p=0.003 and p=0.01, respectively) in the controls. On the other hand, no significant changes in the PSV values were found in the whole group of pSS patients. However, the changes in PSV values before and during lemon juice stimulation were statistically significant in both parotids (p=0.019) and submandibular glands (p=0.012), and not significantly different from those in the controls in pSS patients with mild CS. The variability of RI taken from the salivary glands before and during lemon juice stimulation was not statistically significant in either pSS patients or controls. US abnormalities were detected in the majority of pSS patients and their severity was significantly greater than those recorded in the controls. Of the colour Doppler sonographic (CDS) parameters only PSV was influenced by the degree of chronic inflammation, as shown at the MSG biopsy, suggesting that PSV may reflect the vascular changes occurring in the salivary glands during the course of an autoimmune disease such as pSS.