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ID PMID Title PublicationDate abstract
10227096 [Constrictive bronchiolitis obliterans. Apropos of a case]. 1999 Feb INTRODUCTION: Constrictive bronchiolitis obliterans is defined histologically as obliteration of the lumina of bronchioles by inflammatory tissue elements and progressive destruction of bronchioles, eventually replaced by fibrotic tissue. Most of the cases, which are not associated with either solid organ or bone marrow transplantation, occur in the development of rheumatoid arthritis. EXEGESIS: We present the case of a 73-year-old woman who developed severe constrictive bronchiolitis obliterans without readily identifiable cause, rapidly fatal despite corticosteroid therapy. The patient had antinuclear antibodies (titer 1/1024) and antiphospholipid antibodies, but there was no anti-DNA, anti-ENA or anti-beta 2GPI antibodies. Tests for rheumatoid factor were negative. Cryoglobulinemia was present (monoclonal IgM Kappa). CONCLUSION: In this case, constrictive bronchiolitis obliterans was associated with serologic abnormalities and cryoglobulinemia. However, were was no clinical evidence of connective tissue disease.
28246738 Intercarpal and radiocarpal resection arthroplasty and arthrodesis. 1999 Oct Indications for intercarpal and radiocarpal resectionarthroplasty and fusions are osteoarthritis, KIEHNBÖCK'S disease, rheumatoid arthritis and several posttraumatic disorders of the wrist joint. The resection of carpal bones leads to severe instability patterns of the wrist. In conclusion we recommend resection-arthroplasty just for treatment of the thumb carpo-metacarpal osteoarthritis. Implant resection arthroplasty of the lunate and scaphoid or total wrist implants are still causing multiple problems regarding heavy load. Therefore this implants should be confined to rheumatoid patients. Of the limited carpal arthrodeses the scaphotrapezium-trapezoid arthrodeses is the most frequent performed procedure. It can be indicated for STT-osteoarthritis, KIEHNBÖCK's disease, scapho-lunate instability and scaphoid pseudarthrosis if other surgical procedures had failed presuming there are no signs of arthrosis in the radiocarpal joint. Persisting pain especially in heavy work is quite frequent after limited arthrodesis but can be greatly releaved by simultaneous wrist denervation. In advanced cases of osteoarthritis total wrist arthrodesis is still the best choice for the patient.
11726216 Use of muscarinic agonists in the treatment of Sjögren's syndrome. 2001 Dec Two muscarinic agonists (pilocarpine and cevimeline) have recently been approved for the treatment of symptoms of xerostomia in Sjögren's syndrome (SS). These agents stimulate the M1 and M3 receptors present on salivary glands, leading to increased secretory function. The use of these agents emphasizes the importance of neuroendocrine mechanisms in SS, which is considered an autoimmune disorder. We review recent studies on the release of cytokines and metalloproteinases in SS-affected glands and their influence on the release of and response to neurotransmitters. Also, we review the structure and function of muscarinic receptors as they may relate to SS and the potential use of novel muscarinic agonists in SS.
11575639 Bamboo node: primary vocal fold lesion as evidence of autoimmune disease. 2001 Sep Descriptions of vocal fold lesions related to autoimmune diseases are rare in the literature, and focus mainly on rheumatoid nodules. This is the first report in which autoimmune diseases were promptly suspected by the observation of a unique white transverse submucosal lesion in the vocal fold during clinical examination. This lesion, reported only in autoimmune disease, has been called the bamboo node and its features are different from those of rheumatoid nodules. We report here on two patients who did not have a diagnosis of systemic disease before investigation of their main complaint of hoarseness. At the patients' first visit, vocal fold bamboo nodes were seen in the vocal fold and the otolaryngologist suspected the presence of an autoimmune disease. We requested clinical investigation to clarify our suspicion that there was an underlying systemic disease. After the investigation, both patients were shown to have autoimmune disease, Sjögren's syndrome and systemic lupus erythematous, respectively. This paper emphasizes the important role of the otolaryngologist in the detection of these unique lesions in the vocal folds through the conventional laryngeal methods. These methods consisted of direct observation with a rigid laryngeal endoscope and investigation of the patient's distinctive vibratory pattern by means of laryngeal stroboscopy. The method of treatment we used to obtain the best outcome in terms of voice improvement is also discussed.
11408709 Mucosa-associated lymphoid tissue lymphoma of the salivary glands occurring in patients af 2001 OBJECTIVE: Mucosa-associated lymphoid tissue (MALT) lymphoma of the salivary glands occurring in 6 patients affected by primary Sjögren's syndrome is reported. METHODS: Clinical findings, histologic type, stage, treatment and outcome of the 6 patients have been revised. RESULTS: In all 6 cases the lymphoma was of the MALT type. Four patients had stage IE disease, 1 patient had stage IIE disease and 1 patient had stage IV disease. The patients received different treatments resulting in all cases in prolonged remission. After 7 years of complete remission 1 patient developed a diffuse large B-cell lymphoma. CONCLUSION: MALT lymphoma of the salivary glands is an indolent disease. Though the best therapy of this lymphoproliferative disorder remains to be established, prolonged remission has been obtained in our cases with different therapeutic approaches. We review the literature regarding the relationship between Sjögren's syndrome and MALT lymphomas and study the mechanisms which may be involved in the transformation from a lymphoepithelial lesion into a neoplastic disorder.
11302875 Reversibility of histological and immunohistological abnormalities in sublabial salivary g 2001 May Sjögren's syndrome (SS) is a chronic autoimmune disease characterised by specific lesions in exocrine glands, so sublabial minor salivary gland biopsy (SLGB) plays an important part in its diagnosis. The extent and composition of the lymphocytic infiltrate in SLGB specimens can be considered as target organ specific parameters. They are quantified after histological and immunohistological examination by a focus score (describing the extent of the infiltrate) and IgA% score (describing the composition of the infiltrate), respectively. However, little is known about the factors that contribute to the extent and composition of the infiltrate and whether these features are reversible as repeated SLGBs are rarely performed. A patient with SS is described who underwent SLGBs before and after treatment with high dose corticosteroids. After treatment there was not only clinical improvement, but also improvement in the histological and immunohistological parameters. Although these findings need to be confirmed in further studies, this suggests that histopathological changes may be reversible in SS. Furthermore, it shows that the potential effects of corticosteroid use should be taken into account when interpreting SLGB specimens. When clinical changes do parallel histological changes, repeated SLGBs might offer a marker for disease activity in patients with SS.
11229473 "Lymphoid" chemokine messenger RNA expression by epithelial cells in the chronic inflammat 2001 Feb OBJECTIVE: Many studies have shown that the microanatomic organization of infiltrating leukocytes in the salivary gland lesions of patients with Sjögren's syndrome (SS) resembles the structure of lymphoid organs. A newly defined set of chemokines referred to as "lymphoid," which orchestrate leukocyte microenvironmental homing and contribute to the formation of lymphoid structures, provides directional clues. The aim of this study was to investigate the possible existence of "lymphoid" chemokines in the chronic inflammatory lesions of SS patients and thus validate their potential involvement in the disease process. METHODS: Twelve patients with primary SS, 3 patients with secondary SS, 4 patients with other autoimmune disorders, and 4 control individuals were the subjects of this study. Reverse transcriptase-polymerase chain reaction analysis was performed in order to examine the messenger RNA (mRNA) expression of "lymphoid" chemokines. Furthermore, in situ hybridization studies revealed chemokine mRNA localization. Immunohistochemistry was also applied in order to identify the cell types that expressed the chemokine mRNA. RESULTS: STCP-1/monocyte-derived chemokine and TARC mRNA were expressed in the majority of patients with primary and secondary SS, in 2 of 4 patients with other autoimmune disorders, and in 2 of 4 controls. BCA-1, ELC, and PARC mRNA were only detected in patients with primary and secondary SS. SLC mRNA was also detected in 1 non-SS patient. The main cellular sources of chemokine mRNA were ductal epithelial cells and infiltrating mononuclear leukocytes. CONCLUSION: The expression pattern of "lymphoid" chemokine mRNA points further to the role of epithelial cells in the pathogenesis of SS and offers new insight into the potential mechanisms that could be involved in leukocyte attraction and in the in situ formation of secondary lymphoid tissue structures.
10813291 Abnormal blood flow to the submandibular glands of patients with Sjögren's syndrome: Dopp 2000 May OBJECTIVE: To assess abnormalities in blood flow to the submandibular glands of patients with Sjögren's syndrome (SS). METHODS: Doppler waveform analysis was performed on the facial artery to assess blood inflow to the submandibular gland of 21 patients with primary SS and 69 healthy subjects. Blood flows were compared before and after secretory stimulation with lemon extracts. RESULTS: The facial artery of healthy subjects in the resting state exhibited a biphasic waveform with a high systolic peak and a prominent second peak of compliance followed by a low diastolic flow. In contrast, the waveform of patients with SS was more uniform compared with the healthy subjects, substantiated by decreased resistive and pulsatility indices, suggesting a hyperemic state of the downstream vascular bed. After stimulation of salivary secretion, the facial artery of healthy subjects responded by decreasing resistive and pulsatility indices, waveform changes indicative of increased blood inflow to the submandibular gland. In contrast, the facial artery of patients with SS responded insufficiently to the stimulation, with the magnitude of changes in the resistive and pulsatility indices being significantly lower than those of the controls. Doppler waveform abnormalities were correlated with the severity of gland damage, supporting a close connection between abnormal blood inflow to the salivary gland and impaired secretory function in SS. CONCLUSION: Our findings suggest that blood inflow responses to secretory stimulation may be defective in the salivary glands of patients with SS.
10078005 [Sjögren's syndrome]. 1999 Feb Sjögren's syndrome(SS) is a chronic inflammatory disease characterized by keratoconjunctivitis sicca and xerostomia. According to the epidemiological survey done by the Japan Ministry of Health and Welfare(MHW) in 1994, about 17,000 SS patients visit hospitals and the prevalence rate is approximately 0.06% for females. SS is diagnosed mainly by the criteria of MHW in 1977, and the revised ones will be published in the near future. Several candidates for the corresponding antigens for SS autoimmunity have discovered, such as alpha-fodrin and 203-211 amino acid residues of Ro/SSA-52 kDa. The sicca complex is treated with fluid replacement, but corticosteroids or immunosuppressive drugs are sometimes indicated in the treatment of extraglandular involvement. Several new drugs are now under investigation.
9380944 [Nonsteroidal anti-inflammatory agents: their topical use in the treatment of xerophthalmi 1997 Sep The authors, in a case-control study, analyzed the topical application of nonsteroidal anti-inflammatory drug (flurbiprofene drops) in patients affected by keratoconjunctivitis sicca in Sjögren's syndrome. The variation of break-up-time values, and xerophthalmia grade were analyzed. A mild increase of mean values of break-up-time (two seconds in the treated group), and a mild decrease of xerophthalmia grade were found.
9009125 Esophageal motor function in primary Sjögren's syndrome: correlation with dysphagia and x 1997 Jan The incidence of dysphagia in patients with primary Sjogren's syndrome (pSS) has been underestimated and all too often ascribed to xerostomia, without considering the possible presence of esophageal motor abnormalities affecting other nonscleroderma connective tissue diseases. Esophageal and salivary functions were prospectively evaluated in 27 females who met the four criteria proposed by Fox for the diagnosis of pSS, using esophageal manometry after wet swallows and Saxon's test, respectively. Dysphagia was graded using a standard symptoms questionnaire and results were compared with those obtained in a group of 21 healthy controls. Seven patients with pSS (26%) had no swallowing discomfort, 2 (7.4%) had mild dysphagia, 7 (26%) had moderate dysphagia, and 11 (40.6%) had severe dysphagia. Saxon's test revealed an overall decrease in the salivary flow rate compared to controls, with no difference between patients with or without dysphagia. Esophageal manometry demonstrated the absence of any lower or upper esophageal sphincter function abnormalities in all patients. In the patients with pSS as a whole, manometric study of the esophageal body showed a motor pattern comparable with that of controls, with no difference between patients with and without dysphagia. Defective peristalsis, ie, the presence of simultaneous contractions in more than 30% of wet swallows was detected, however, in the distal tract of the esophagus of six patients (22.2%) and in the proximal tract of three (11.1%). All these patients had severe dysphagia and the modified Saxon's test revealed a salivary secretion comparable with that of patients with a normal peristalsis. Dysphagia is a very common complaint in patients with pSS and does not seem to correlate with xerostomia, which is a constant and typical finding of the disease. About one third of patients with pSS have an abnormal esophageal peristalsis that is responsible for severe dysphagia, whereas decreased salivary outflow exacerbates the swallowing discomfort. This has to be taken into account and justifies the routine use of esophageal manometry in patients with pSS. The cause of dysphagia in pSS patients without peristaltic disorders of the esophagus has to be investigated.
9830882 Cross-cultural adaptation and validation of an Argentine Spanish Version of the Stanford C 1998 Oct OBJECTIVES: To translate into Argentine Spanish and cross-culturally adapt the Childhood Health Assessment Questionnaire (CHAQ) and validate the adapted instrument in Argentine patients with juvenile rheumatoid arthritis (JRA). METHODS: Five bilingual Argentine pediatric rheumatologists translated into Argentine Spanish and cross-culturally adapted the United States English CHAQ. Pretesting was done in a sample of 23 parents using a probe question technique. Parents of 70 patients with JRA and 21 healthy children (controls) participated in the validation phase. All were from Argentina. RESULTS: The mean disability index (DI) scores for patients with systemic, polyarticular, or pauciarticular onset JRA were 0.64, 0.32, and 0.1, respectively. Healthy controls averaged 0.2. Intercomponent correlations were between 0.4 and 0.9, suggesting internal consistency, but also some redundancy. Test-retest reliability, studied at a 1-week interval, was moderate (mean DI 0.44 [in clinic] and 0.29 [one week later], Pearson's correlation = 0.82). We compared CHAQ scores from 15 parents with those of their children > 10 years of age. Significantly higher DI scores were given by patients than their respective parents (P > 0.019), but the pairwise scores (parent-patient) were highly correlated (r = 0.986). CONCLUSIONS: Cross-cultural adaptation of the US CHAQ to Argentina required few changes. Although DI scores for all patient subgroups were higher than for controls subjects, the scores were low, particularly for those with pauciarticular disease. Prospective studies designed to examine the sensitivity to change and predictive validity will help to assess further the usefulness of the adapted CHAQ in the Argentine population.
9884441 Arthroscopic ankle arthrodesis: a preliminary report. 1998 Dec BACKGROUND: Over the past decade, arthroscopy has become a well-accepted technique for treating complex ankle problems. Recently, technical refinement with improvements in instrumentation has allowed more extensive arthroscopic debridement. With these improvements, arthroscopy can be used to perform successful arthrodesis of the ankle. METHODS: Five arthroscopic ankle arthrodesis procedures were performed. Three patients had post-traumatic osteoarthritis, one had rheumatoid arthritis and one had gouty arthritis combined with pyogenic arthritis. Following arthroscopic resection of the synovial membrane, cartilage and subchondral bone from the tibial, fibular and talar articular surfaces, an autogenous iliac bone graft was performed using arthroscopic guidance. Two percutaneous screws were used for internal fixation in three cases. Postoperatively, patients wore a short leg walking cast for eight weeks. The average follow-up was 18.8 months (range, 12-24 months). RESULTS: Clinical ankylosis was achieved in all cases and there was radiologic evidence of bone fusion in four cases. In one case, delayed union occurred, but it was achieved at the 12th postoperative month of follow-up. CONCLUSIONS: Arthroscopic ankle arthrodesis is less invasive than open techniques and less painful after surgery; it also allows for a better cosmetic effect. There is a learning curve associated with arthroscopic ankle fusion, and it is time-consuming. Severe deformity is a contraindication for this method and such cases should only be treated using the open method.
11685094 Pulmonary involvement in primary Sjögren's syndrome: spectrum of pulmonary abnormalities 2001 Oct The purpose of this study was to describe the high-resolution computed tomography (HRCT) findings of pulmonary involvement in primary Sjögren's syndrome. The study included 60 patients who met the diagnostic criteria for primary Sjögren's syndrome. The authors retrospectively reviewed the presence, extent, and distribution of various HRCT findings. Results showed that the most common HRCT findings were areas with ground-glass attenuation (92%), followed by subpleural small nodules (78%), non-septal linear opacity (75%), interlobular septal thickening (55%), bronchiectasis (38%), and cysts (30%).
9818879 D-penicillamine treatment for chronic sensory ataxic neuropathy associated with Sjögren's 1998 Nov Beneficial treatment has not been established for chronic sensory ataxic neuropathy associated with Sjögren's syndrome (CSAN-SS). We describe two patients with CSAN-SS who clinically improved in response to D-penicillamine treatment. Their neuropathic symptoms were lessened after D-penicillamine, and the results of electrophysiologic studies support the clinical improvement. D-Penicillamine can be considered a potentially beneficial agent in the treatment of CSAN-SS.
9787114 Lacrimal gland innervation is not altered with the onset and progression of disease in a m 1998 Nov The lacrimal glands of patients with Sjögren's syndrome develop extensive lymphocytic infiltration, but also contain a large number of seemingly healthy looking acinar and ductal cells. Despite this, the secretory function of this tissue is impaired, leading to aqueous tear-deficient dry eye. This raises the possibility that there is a defect in the neural innervation of the remaining portion of the lacrimal gland. To test for this possibility, we used antibodies specific to various markers of the parasympathetic, sympathetic, and sensory nerves and performed immunohistochemical analyses of lacrimal glands from a murine model of Sjögren's syndrome, the MRL/Mp-Fas-lpr/lpr (MRL/lpr) and the control mice MRL/Mp-+/+ (MRL/+). Our results show that the MRL/lpr, but not the MRL/+, lacrimal glands become infiltrated with lymphocytes starting at 8 weeks of age which worsens by 12 and 18 weeks. The density and the pattern of parasympathetic, sympathetic, and sensory innervation of the noninflamed acinar tissue of MRL/lpr lacrimal glands, at 4, 8, 12, and 18 weeks, is indistinguishable from that of age-matched control MRL/+ lacrimal glands. We conclude that the loss of the secretory function in Sjögren's syndrome lacrimal glands is not due to a loss or decrease of its innervation.
9694067 Still's disease in children and adults: a distinct pattern of acute-phase proteins. 1998 Crossed affinoimmunoelectrophoresis with Con A as a ligand was used to examine the microheterogeneity of alpha1-acid glycoprotein (AGP) and alpha1-antichymotrypsin (ACT) in sera of patients with child-onset and adult-onset Still's disease. The reactivity of both proteins was increased in sera of adults and decreased in sera of children with active disease, when compared with normal values. We also found statistically significant differences in serum concentration of ACT and ferritin in both diseases. This result suggests different pathogenic mechanisms of Still's disease in children and adults. Serum concentration of ferritin and ACT could be of value as a combined marker for the adult, but not the juvenile form of Still's disease.
9462169 Prevalence and clinical relevance of 52-kDa and 60-kDa Ro/SS-A autoantibodies in Japanese 1997 Nov OBJECTIVE: To determine the prevalence of 52-Kda and 60-Kda Ro/SS-A autoantibodies in serum samples from Japanese patients with systemic sclerosis (SSc). METHODS: Serum samples from 263 Japanese patients with SSc were examined by double immunodiffusion and enzyme linked immunosorbent assay (ELISA). RESULTS: By double immunodiffusion, 29 serum samples from patients with SSc were found to possess anti-Ro/SS-A antibodies. By ELISA, 31 serum samples were positive for anti-Ro52 and/or anti-Ro60. Of 27 serum samples that were positive by both methods, 15 reacted with both Ro52 and Ro60, five with Ro52 alone, and seven with Ro60 alone. Eleven of 12 patients with both anti-Ro52 and anti-Ro60 and all five patients with anti-Ro52 alone had Sjögren's syndrome, while only one of six patients with anti-Ro60 alone had this disorder. CONCLUSIONS: Anti-Ro52 may be a serological marker for the presence of Sjögren's syndrome in anti-Ro/SS-A-positive patients with SSc.
9051869 Gastritis classified in accordance with the Sydney system in patients with primary Sjögre 1997 Feb BACKGROUND: According to some earlier reports, chronic atrophic gastritis is a common finding in Sjögren's syndrome. However, the findings are controversial. The aim of this study was to investigate the occurrence of gastritis classified in accordance with the Sydney System in patients with primary Sjögren's syndrome. METHODS: Thirty-two consecutive patients (27 women, 5 men) with primary Sjögren's syndrome, and 64 age- and sex-matched control subjects with dyspepsia underwent gastroscopic examination. Mucosal biopsy specimens were taken from the gastric antrum and corpus. RESULTS: Eight (25%) patients with Sjögren's syndrome and three (4.1%) control subjects had atrophic antral gastritis (P = 0.01). Atrophic corpus gastritis was more frequently found in control subjects, but the difference was not statistically significant. None of the subjects had severe (grade 3) atrophy. Gastric inflammation, in either the corpus or antrum, was found in 85% of patients with Sjögren's syndrome and in 61% of control subjects (P = 0.02). Helicobacter pylori was present in 31% of Sjögren's syndrome patients and in 39% of controls (NS). CONCLUSIONS: In patients with primary Sjögren's syndrome mild atrophic changes in the antrum were common, but severe mucosal atrophy was rare. Compared with control subjects, gastric inflammation was seen more often in patients with Sjögren's syndrome.
11490517 Changes in glycosylation of immunoglobulins in primary Sjögren's syndrome. 1998 Feb Serum IgG and IgA are glycoprotein and significant glycoform abnormalities have been established in primary Sjögren's syndrome. The proportion of asialylated IgG is abnormally high in the patients, whereas IgA1 and IgA2 appear to be over-sialylated. This peculiarity might explain the defective binding of IgA to asialoglycoreceptors. Furthermore, the activity of alpha 2,6 sialyl transferase is higher in the IgA-producing B cells from the patients than in the controls, whereas the alpha 2,3 sialyl transferase operates in the former cells but not in the latter. The mechanism of this enzyme dysregulation warrants elucidation.