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ID PMID Title PublicationDate abstract
11890655 Successful treatment of a small cohort of patients with adult onset of Still's disease wit 2001 Nov OBJECTIVE: To test the efficacy of infliximab in the treatment of patients with severe and active adult onset Still's syndrome (AOSD) despite conventional immunosuppressive therapy. PATIENTS AND METHODS: Six patients with the diagnosis of AOSD according to the Yamagushi criteria of 1992 were treated with infliximab. All patients had severe disease with high clinical and serological activity. Patients were treated initially with high dose steroids or more intensive immunosuppressive therapy. Two patients had a history of multiple disease modifying antirheumatic drug (DMARD) treatments. One patient had a history of three years of AOSD with fever, chills, pleural and pericardial effusions, and hepatosplenomegaly. Despite these treatments, he developed increasing serological signs of inflammation and arthritis of both hips and peripheral joints. Another patient had a history of five years of AOSD with oligoarthritis, myalgias, and recurrent fever despite multiple DMARD treatment, including cyclophosphamide pulse therapy. Our patients with AOSD presented with massive polyarthralgias, polyarthritis, splenomegaly or hepatomegaly, the typical rash, sore throat, weight loss, serositis, continuing fever, leucocytosis, and raised C reactive protein (CRP), erythrocyte sedimentation rate (ESR), and ferritin levels. Four patients with early onset of the disease, fulfilling the diagnostic criteria for AOSD and a clinical and serological high disease activity, were included in our pilot study without any further DMARD treatment apart from the initial steroid treatment. Reduction of established treatment, mainly with steroids, caused a relapse of the disease in all our patients. Patients then received 3-5 mg/kg infliximab on weeks 0, 2, and 6, continuing with intervals of 6-8 weeks depending on the patient's individual disease activity. RESULTS: In all patients, fever, arthralgias, myalgias, hepatosplenomegaly, and the rash resolved after the first courses of treatment with infliximab. All serological variables (CRP, ESR, hyperferritinaemia) returned to normal. After three courses of infliximab infusions, splenomegaly could not be detected in any of our patients. One caused by hip postarthritic osteoarthrosis, requiring hip replacement. After three courses of treatment with infliximab, splenomegaly could not be detected in any of our patients. Up to now, our patients have received infliximab infusion treatment for between five and 28 months. Throughout this period all patients have continued to benefit from this treatment, with improvement in their clinical symptoms, joint counts, and serological disease activity. One of our patients had a moderate infusion reaction during the second treatment. The infusion was discontinued for one hour and then was resumed with no further problems. CONCLUSION: The disease improved remarkably in all six patients with AOSD after treatment with infliximab, also in the early stage of AOSD. These preliminary data suggest the potential therapeutic benefit of anti-tumour necrosis factor alpha treatment in AOSD.
10492146 Severe hepatitis and pure red cell aplasia in adult Still's disease: good response to immu 1999 Aug Adult-onset Still's disease is a systemic inflammatory disorder with a highly variable clinical course. Mild hepatitis and anemia are common manifestations. We describe a patient with adult Still's disease who developed a severe hepatitis and a life-threatening pure red cell aplasia. The hepatitis developed after treatment with NSAIDs was started. The patient was successfully treated with a combination of prednisone, cyclosporin, and methotrexate. Physicians should be aware that severe hepatitis and pure red cell aplasia can occur in adult Still's disease. We recommend a careful monitoring of liver functions in patients with adult Still's disease who are being treated with NSAIDs.
9990376 Lymphocytic autoimmune hidradenitis, cutaneous leucocytoclastic vasculitis and primary Sjà 1998 Dec We describe a 60-year-old woman with primary Sjögren's syndrome, mixed cryoglobulinaemia and cutaneous leucocytoclastic vasculitis who developed generalized hypohidrosis with a markedly decreased sweating response to pilocarpine chloride. Skin biopsies demonstrated dense peri-eccrine lymphocytic infiltrates in the lower reticular dermis, with glandular atrophy. From previous studies it is evident that although patients with Sjögren's syndrome commonly have skin dryness, a lymphocytic hidradenitis has been documented only in a few cases. The histological findings in this case support the role of autoimmune hidradenitis in the development of hypohidrosis in Sjögren's syndrome.
9932974 Hypertrophic cranial pachymeningitis and lymphocytic hypophysitis in Sjögren's syndrome. 1999 Jan 15 The authors describe a patient with primary Sjögren's syndrome who developed pachymeningitis, hypopituitarism, and central diabetes insipidus. The patient improved with corticosteroid pulse therapy.
9619671 Salivary electrophoresis in the diagnosis of Sjögren's syndrome. 1998 May OBJECTIVE: The purpose of this study was to investigate the potential use of salivary electrophoresis for the diagnosis of Sjögren's syndrome. METHODS: Salivary protein profiles of 43 patients and 39 healthy control subjects were compared on three different gel electrophoresis systems: sodium dodecylsulfate-polyacrylamide gel electrophoresis, anionic polyacrylamide gel electrophoresis, and immobilized pH gradient gel electrophoresis (isoelectric point, 3.5-5.0). RESULTS: Most of the patients with Sjögren's syndrome exhibited an electrophoretic profile that was different from that of the healthy control subjects. Among the three gel electrophoresis systems examined, the immobilized pH gradient system appeared to be the most reliable for Sjögren's syndrome. Tests of accuracy revealed that the immobilized pH gradient system exhibits high specificity (97%), sensitivity (95%), positive predictive value (97%), and negative predictive value (95%) in the diagnosis of Sjögren's syndrome. CONCLUSIONS: Our results suggest that salivary electrophoresis is a potentially useful test for the diagnosis of Sjögren's syndrome.
9180879 [Gougerot Sjögren syndrome and hepatitis C virus: what relation?]. 1997 Apr 26 The incidence of Sjogrëns syndrome (SS) in patients with chronic liver disease and that of hepatitis C in mixed cryoglobulinemia strongly-suggest a link between the hepatitis C virus (HCV) and SS. A recent report has also demonstrated typical lymphocyte sialadenitis in HCV-positive subjects, raising a question concerning the classical definition of SS. Do patients with HCV and lymphocyte sialadenitis have SS? If the problem of variable diagnostic criteria can be overcome (for example by using the criteria established by the European study group) it can be concluded that the proportion of HCV+ patients with SS is related to female sex, age (perimenopause period in women), and liver histology rather than fibrosis, but not with duration of the liver disease, nor cirrhosis or viral genotype. The second question is to determine whether the observed focal lymphocyte infiltration of the salivary glands is typical of SS. As routine biopsy results lack specificity and sensitivity, immunohistochemistry is required to identify T8 predominance distinctive of SS. Results obtained to date suggest that the T8 sialadenitis might result from an autoimmune mechanism and consequently that the SS-HCV association might either be a coincidence between the two relatively frequent diseases or on the contrary that HCV plays a pathogenic role in SS. The major argument for the latter hypothesis would be the demonstration of HCV within the salivary gland epithelial cells. As HCV-positive immunohistochemistry tests on salivary biopsies may simply indicate presence of HCV in the blood stream at the time of biopsy, more sophisticated in situ PCR methods are currently being applied in an attempt to obtain objective evidence which could incriminate HCV infection of the salivary glands as a causal agent in Sjogrëns syndrome.
11865567 [Psychiatric manifestations of lupus erythematosus systemic and Sjogren's syndrome]. 2001 Nov We present one case of Sjögren's syndrome (SS) secondary to systemic lupus erythematosus (SLE) with predominant psychiatric manifestations, treated with success by cyclophosphamide. From this case, we review the psychiatric aspects of these two autoimmune diseases as described in the literature and we present the etiopathogenic hypothesis and treatment of the psychiatric disorders. Case report--In August 1996, a 38 year old man was admitted in our psychiatric department for agitation. Primary SS had been diagnosed in July 1996. He had previously attempted to suicide but was never hospitalized in a psychiatric department. During the hospitalization in our department, the patient had auditive hallucinations and felt persecuted. He received loxapine 400 mg/day and was remitted in a few days. He was discharged to a convalescent home with the diagnosis of brief psychotic disorder. In October 1996, he was readmitted to our department for agitation. He had shown agitated behavior and aggression in the convalescent home. There were no hallucinations and no affective disorders. He became calm rapidly and was discharged home a few days later. In November 1996, he was found in a coma by a neighbor. He was admitted to an intensive care unit. The lumbar punction revealed blood cells. Cerebral computer tomography showed subarachnoid hemorrhage. The diagnosis was meningeal hemorrhage due to vasculitis. After regaining consciousness, the patient complained of reduced visual acuity. This was believed to be due to retrobulbar neuritis and the patient's vision improved slightly with corticosteroids. The third hospitalization in our department occurred in February 1997 for depression. The patient had shut himself away for days in his apartment. He had suicidal ideas. His mood improved progressively under fluoxetine 40 mg/day. He was discharged to a convalescent home with the diagnosis of major depressive disorder. The fourth and last admission in our department occurred in June 1997. There were disturbances of memory and orientation. He felt sad and guilty about accusation of sexual abuse on his daughter. He presented typical histrionic symptoms: he had catatonic attitudes only in public areas such as the corridors. Cerebral computer tomography and electroencephalogram were normal. There was no biological abnormality. Signs of confusion rapidly disappeared. He felt better after reintroduction of fluoxetine 40 mg/day. Diagnosis was non-specified depressive disorder, but this episode could be retrospectively seen as delirium. After being hospitalized on these four occasions in one year in our psychiatric department, the diagnosis of his systemic disease was revised by rheumatologists. The patient was diagnosed as suffering from systemic lupus erythematosus associated with secondary Sjögren's syndrome. From September 1997, he received cyclophosphamide 2 g intraveinously per month during 6 months. His vision improved dramatically. His ocular dryness became milder. His mood is now stable. He has not suffered from hallucinations or delusion since. Psychiatric disorders in SLE--During the course of SLE, the occurrence of psychiatric manifestations varies widely from 5 to 83%. They include psychotic disorders, major depressive disorders, subtle cognitive disorders and personality disorders of histrionic type. Etiopathogenic hypothesis are: direct activity of the disease on the central nervous system by autoantibodies (antiphospholipide and antiribosome P autoantibodies) (18, 19) or cytokines (interleukin 2, interleukin 6, alpha interferon) (38, 59), side-effects of glucocorticosteroids and hydroxychloroquine (16) or anxious reaction to a chronic and potentially lethal illness (43, 54). Nevertheless, immunologic and cerebral imagery research suggests that psychiatric disorders are related to vasculitis and non-inflammatory vasculopathy of the small cerebral blood vessels. The management of the patients should include treatment of the disease itself and specific psychotropic treatment. Glucocorticosteroids and especially intravenous infusions of immunosuppressive agents, such as cyclophosphamide, are effective. Psychotropic drugs must be used, making sure to avoid SLE-inducing drugs, like chlorpromazine, carbamazepine and lithium carbonate (19, 20, 45). In addition, psychologic care is essential. Psychiatric disorders in SS--During the course of the primary SS, the occurrence of psychiatric disorders is large as well: from 20 to 70% (47, 61, 62). They are mainly major depressive disorders, anxiety disorders, cognitive disorders and dementia. Brief psychotic disorders and delirium are rare. Etiopathogenic hypotheses are similar as those in SLE, with some differences: antiphospholipide and antiribosome P autoantibodies are not usually found in SS and anti-Ro (SSA) autoantibodies in serum are associated with psychiatric disorders (3-11, 61). According to Drosos et al. (29, 30), psychiatric disorders are explained by psychological distress. This slowly progressive fluctuating disease creates constant discomfort from dysphagia, dyspareunia and functional disability. Some of these manifestations can be treated by corticosteroids and psychotropic drugs. Drugs with anticholinergic side-effects, like phenothiazines, tricyclic antidepressants and hydroxyzine which can enhance the oral dryness have to be avoided. Social and psychological support is important too. DISCUSSION: The diversity of psychiatric morbidity in SLE and SS may be due to differences in patient selection and a lack of uniform clinical criteria. Studies which use standardized diagnostic criteria and control groups don't allow one to come to a conclusion about the relative prevalence of the psychiatric disorders in these autoimmune diseases. This will probably be resolved thanks to the recently published "American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes" (1). Finally, we can ask ourselves if there is a significant number of undiagnosed SLE and SS in psychiatric departments. Two studies report systematic search for SLE in psychiatric patients. In 1992, Hopkinson et al. (39) searched for several autoantibodies in serum samples of nearly 300 hospitalized psychiatric patients. In 1993, Van Dam et al. (65) did the same with more than 2,000 patients admitted to a psychiatric hospital. Hopkinson et al. found 1% undiagnosed SLE, which is much higher than in general population, and recommended to search SLE in every patient with a high erythrocyte sedimentation rate in psychiatric services. Results of the Van Dam et al. study suggest on the contrary, that SLE is not a common cause of admission to psychiatric hospitals. There is no study which report systematic search of Sjögren's syndrome in a psychiatric department. This is probably because most of patients receive or have recently received psychotropics with anticholinergic side-effects which is an exclusion criteria of SS. CONCLUSION: Psychiatrists should keep in mind that SLE and primary SS are potential causes of psychiatric manifestations when examining patients with multiple unexplained somatic complaints and psychiatric symptoms. They should then search for autoantibodies in the serum after careful physical examination. Diagnosis of SLE or SS could lead to a better adapted prescription of corticosteroids and/or immunosuppressive drugs and specific psychotropic drugs, making sure to avoid lupus-inducing drugs in SLE and drugs with anticholinergic effects in SS. The existence of psychiatric manifestations in SLE and SS constitutes an indisputable clinical reality that each practitioner must be able to recognize and treat.
11398338 [Effect of Bakumondo-to on increased sensitivity of the cough reflex in a Sjögren syndrom 2001 Apr A 63-year-old woman had complained of a persistent dry cough. Comparison of chest roentgenograms obtained 3 months earlier and at hospital admission, revealed progressive infiltrative shadows in bilateral middle and lower lung fields and mild volume loss. Hematologic study showed polyclonal hyper-gamma-globulinemia, positive anti-nuclear antibodies, and positive antibodies to Ro (SS-A) antigens. Ophthalmologic study demonstrated keratoconjunctivitis sicca. Parotid sialography showed an apple tree sign. Histopathologic features of labial salivary gland biopsy revealed agglomeration of at least 50 mononuclear cells. Chest CT showed non-segmental dense opacity in outer zones of middle and lower lung fields. Lung function test revealed a restrictive pattern with low diffusion capacity. Blood gas analysis showed hypoxemia (PaO2: 74 Torr, PaCO2: 32 Torr). Bronchoalveolar lavage fluid disclosed increased lymphocytes, and histologic features of a transbronchial lung biopsy included thickening of alveolar walls, infiltration of lymphocytes in the interstitium, and foamy cells in alveolar spaces. Therefore, the patient was diagnosed with primary Sjögren syndrome with interstitial lung disease. Bakumondo-to (Mai men dong tang) was started for the troublesome dry cough. The cough was subjectively relieved, cough scores were decreased, QOL scores improved, and sensitivity of the cough reflex measured by inhalation of capsaicin improved, but the chest roentgenogram was unchanged. Steroid hormone therapy was added, and radiographic abnormalities improved subsequently. Bakumondo-to, a traditional Chinese blended medicine, has notable antitussive activity in bronchitic guinea-pigs and in patients with postinfectious cough. It was concluded that Bakunondo-to may provide effective antitussive activity in some cases of interstitial lung disease with increased sensitivity of the cough reflex.
10833874 Multiple neuromas coexisting with rheumatoid synovitis and a rheumatoid nodule. 2000 May The authors present a rare case of multiple intermetatarsal neuromas coexisting with rheumatoid synovitis and a rheumatoid nodule. A brief review of rheumatoid nodules as a source of forefoot pain and a review of the relevant literature are provided. A rheumatoid nodule is just one of the many diagnoses that must be considered when one encounters pedal symptoms similar to those associated with Morton's neuroma.
9568728 Single-blinded controlled trial of low-dose oral IFN-alpha for the treatment of xerostomia 1998 Apr A single-blinded controlled trial was conducted to test the efficacy of low-dose oral human interferon-alpha (IFN-alpha) to improve salivary function in patients with Sjögren's syndrome. Fifty-six outpatients with primary and 4 patients with secondary Sjögren's syndrome were assigned randomly into treatment groups of either IFN-alpha or sucralfate (control). The IFN-alpha (150 IU) or sucralfate (250 mg) was given orally three times a day for 6 months. Saliva was quantitated monthly by the Saxon test. After 6 months of treatment, 15 of 30 (50%) IFN-alpha-treated patients had saliva production increases at least 100% above baseline, whereas only 1 of 30 (3.3%) sucralfate patients had a comparable increase (p < 0.001). The increase in saliva production, by treatment group, was significantly greater (p < 0.01) in the IFN-alpha treated group at every month after treatment. Serial labial salivary gland biopsies of 9 IFN-alpha responder patients showed that lymphocytic infiltration was significantly decreased (p < 0.02) and the proportion of intact salivary gland tissue was significantly increased (p = 0.004) after the IFN-alpha treatment. In this study, IFN-alpha therapy significantly improved Sjögren's syndrome salivary gland dysfunction.
11762685 Magnetic resonance imaging and magnetic resonance sialography of parotid glands in primary 2001 Dec OBJECTIVE: To look for structural parotid gland changes on magnetic resonance (MR) imaging and MR sialography of primary Sjögren's syndrome (SS) patients and healthy control subjects and to compare these methods with each other. METHODS: MR imaging and MR sialography of both parotid glands were performed on 26 patients and 7 healthy controls. Bilateral surface coils were used to obtain high spatial resolution. RESULTS: Twenty-two of the 26 patients had abnormalities on MR imaging. Twenty-one had a nodular or dendritic parenchymal pattern, 5 had cavities, and 6 had duct dilatations. On MR sialography, 25 of the 26 patients had abnormalities of the ducts, and 16 of them also had cavities. One patient and all 7 controls had normal results with both methods. The structural appearance of the parotid glands on MR images had marginal linear association with the duct system changes but no correlation with the cavitary changes seen on MR sialography. Furthermore, duct system abnormalities did not correlate with cavitary changes. Both parenchymal and sialographic abnormalities were associated with the presence of Ro/SSA antibodies but not with age of the patient, disease duration, salivary flow rate, or the presence of hypergammaglobulinemia or extraglandular manifestations. CONCLUSION: MR imaging and MR sialography are noninvasive methods that provide definitive information of morphologic changes in parotid glands and can be used as diagnostic indicators of primary SS. Because these methods give information on different aspects of glandular pathology, both should be performed when evaluating parotid glands of SS patients. MR sialography is more sensitive, but conventional MR imaging gives complementary information on the progressive pathologic changes of glandular parenchyma.
11757220 [Pseudolymphoma in patients with primary Sjogren's syndrome]. 2001 Aug Sjögren's syndrome is an inflammatory autoimmune disease affecting primarily the exocrine glands. In the abscence of other autoimmune diseases it is classified as primary Sjögren's syndrome. Patients with primary syndrome have about 40 times higher relative risk of developing lymphoma then normal population, which offers a possibility to study malignant transformation's mechanisms in these patients. In the study we report a case of a woman with pseudolymphoma. The clinical diagnose used to be based on symptoms of proliferate disease, quetionnable histological evaluation and good response to steroids. Nowadays, applying advanced molecular techniques make it possible to diagnose lymphoma in labial salivary biopsy much earlier. It allows recognizing an incipient lymphoma in a group of patients with primary Sjögren's syndrome. Therefore so-called pseudolymphoma can usually be diagnosed as either benign or malignant lymphoproliferative lesions.
11469519 Sjögren's syndrome as seen by an oral physician. 2001 This article provides a brief overview of the various orofacial presentations of Sjögren's syndrome. The paper summarises the clinical features of patients with xerostomia, and alludes to differential diagnosis, investigative procedures and therapeutic modalities for the oral component of Sjögren's syndrome.
10765751 Orofacial disease: update for the dental clinical team: 8. Salivary complaints. 1999 Oct Certain lesions exclusively or typically affect the salivary glands; these are discussed in this article.
10343542 Lung involvement in primary Sjögren's syndrome is mainly related to the small airway dise 1999 Jan OBJECTIVE: To evaluate lung involvement in patients with primary Sjögren's syndrome. METHODS: Sixty one consecutive, non-smoking patients, 58 women and three men, were evaluated clinically, physiologically, and radiologically. A bronchial and/or transbronchial biopsy was performed on 13 of the patients. Physiological data were compared with that of a control group of 53 healthy non-smoking subjects matched for age and sex. RESULTS: In 41% of the patients the main symptom was dry cough. Physiological studies revealed that the patients presented significantly lower expiratory flow values (% pred) when compared with those of the control group: the forced expiratory volume in one second (FEV1) (mean (SD)) was 96% (16) v 111% (13) (p < 0.0001), the maximal expiratory flow at the 50% of the vital capacity (MEF50) was 72% (24) v 103% (17) (p < 0.0001), and the maximal expiratory flow at the 25% of the vital capacity (MEF25) was 49% (25) v 98% (20) (p < 0.0001). No significant difference was noted for the carbon monoxide diffusion value (% pred), between patients and controls. Blood gases were evaluated in 44 patients: mild hypoxemia was observed, and the alveolo-arterial oxygen difference (P(A-a)O2) correlated significantly with MEF50 (r = 0.35, p < 0.01) and MEF25 (r = 0.33, p < 0.01) values. Chest radiography showed mild, interstitial-like changes in 27 patients while slightly increased markings were present in 21. High resolution computed tomography of the lungs was performed in 32 patients (four with a normal chest radiograph, six with suspected interstitial pattern, 19 with apparent interstitial pattern, and three with hyperinflation) and revealed predominantly wall thickening at the segmental bronchi. All positive findings by computed tomography derived from the patients with abnormal chest radiographs. Transbronchial and/or endobronchial biopsy specimens in 10 of the 11 sufficient tissue samples revealed peribronchial and/or peribronchiolar mononuclear inflammation, while interstitial inflammation coexisted in two patients. CONCLUSION: The airway epithelia seem to be the main target of the inflammatory lesion of the lung in patients with primary Sjögren's syndrome. It seems to be common, subclinically leading to obstructive small airway physiological abnormalities.
9630765 A case of primary Sjögren's syndrome in combination with chronic mesangiocapillary glomer 1998 The authors present an extremely rare case of combination of primary Sjögren's syndrome and chronic glomerulonephritis, which was subsequently found to be mesangiocapillary on pathohistologic examination. METHODS: This case of mesangiocapillary glomerulonephritis in combination with interstitial nephritis is characterized in terms of the clinical, laboratory, immunologic and instrumental methods for diagnosis. Percutaneous kidney biopsy was performed and the characteristic findings on light microscopy were recorded. RESULTS: The therapeutic regimen consisting of pulse therapy with Immunovenin-intact and cyclophosphamide resulted in long-term clinical and laboratory remisson of the glomerulopathy and positively influenced the remaining syndromes. CONCLUSION: Pulse therapy with these drugs is an alternative to conventional pathogenetic therapy; it can also be the therapeutic modality of choice in cases similar to the one described here having in mind the long-term therapeutic remission.
9442830 Expression of glycoconjugates in normal and Sjögren's syndrome labial glands. 1997 Nov Glyconjugate expression in formalin-fixed, paraffin-embedded Sjögren's syndrome labial glands (nine cases) was compared with that in normal glands (12 samples) using a wide panel of lectins. Mucous cells expressed mainly mucous-type glycoproteins including sialyl, fucosyl, galactosyl and galactosaminosyl residues, and some N-linked glycoconjugates. Demilunar cells expressed mainly alpha D-mannose, GlcNAc and Gal beta 1.3-GalNAc. Duct cells and cellular glycocalyx expressed O-linked and N-linked residues. Sjögren's syndrome samples showed a statistically significant increase in the expression of GlcNAc. A significantly decreased expression of Gal beta 1,3-GalNAc and alpha D-mannose, residues not usually present in mucous cells, was found (p < 0.05). This suggests that anatomic rather than functional alterations determine mucous cell impairment and the symptoms in Sjögren's syndrome.
9272293 A longitudinal study of lung impairment in patients with primary Sjögren's syndrome. 1997 Jul OBJECTIVE: A longitudinal evaluation of lung involvement in primary Sjögren's syndrome (SS). METHODS: Eighteen non-smoking women fulfilling the European criteria for primary SS were followed for 55 months (range 26-137 mos.). These were consecutive patients with exclusion for current smokers and patients with lung diseases. Every patient underwent clinical examination, chest radiographs and lung function tests (spirography, flow/volume loop and CO lung diffusing capacity measurements). No patient was given any immunosuppressive or mucolytic therapy. RESULTS: Cough, dyspnea on exertion and recurrent bronchitis were observed in 50, 40 and 20% of the patients respectively and their frequency did not change with time. Chest radiographs were and remained normal. At presentation, lung volumes and diffusing capacity were in the normal range, whereas expiratory flows in the small airways tended to be in the low range. With time, the peak expiratory flow (PEF) significantly increased (95.8 +/- 4.6 v 103.5 +/- 4.6, mean +/- SE, % of predicted, Wilcoxon, p < 0.05) whereas the lung transfer factor for CO (TLCO) and the transfer coefficient (KCO = TLCO/alveolar volume) decreased (92.9 +/- 4.0 v 87.0 +/- 4.0 and 89.7 +/- 2.4 v 84.2 +/- 2.6 respectively, p < 0.05 for both). The TLCO decrease, corrected for the duration of follow-up, correlated with the titers of IgA circulating immune complexes (CIC) at presentation and to a lesser extent with the occurrence of cough. CONCLUSION: During the follow-up of these primary SS patients, respiratory symptoms did not change, the recurrent respiratory infection rate was low, and no cases of pulmonary hypertension or lymphoma was observed. The diffusion capacity decrease was associated with IgA CIC titers at presentation. This impairment could contribute to dyspnea during its evolution over a lifetime but is too slight to explain the dyspnea on exertion seen in most of our patients.
10985364 Frequency and evaluation of t(14;18) translocation in Sjögren's syndrome. 2000 Aug In most cases of follicular lymphoma, t(14;18) chromosomal translocation can be detected in lymphocytes of peripheral blood and bone marrow. Nevertheless, certain other types of diseases can also be characterised by the presence of the translocation. Patients of Sjogren's syndrome have an increased frequency of developing non-Hodgkin's lymphoma, e.g. follicular lymphoma; in turn, they may have translocation-bearing cells. One hundred Sjögren's syndrome patients were screened using a nested polymerase chain reaction technique to identify whether they had the translocation in their peripheral blood lymphocytes. Five percent of that population revealed a temporary or long-lasting presence of the translocation, sometimes even in the lymphocytes from bone marrow. Our results indicate that in addition to the conventional diagnostic methods of lymphoma, there are certain other factors, e.g. the duration of the presence of t(14; 18) translocation and the source of lymphocytes, that should be considered for successful early diagnoses and perhaps for treatment of the lymphoma in the Sjögren's patients.
10555894 Sex differences in primary Sjögren's syndrome. 1999 Nov OBJECTIVE: To examine the clinical and serologic characteristics of 14 men compared to 28 women with primary Sjögren's syndrome (SS) and contrast these findings with studies evaluating sex differences in primary SS. METHODS: Patient information was collected from patients seen at the National Institutes of Health Salivary Gland Dysfunction Clinic from 1987 to 1998. A total of 14 male patients were diagnosed with primary SS during this period. The control group consisted of 28 female patients matched according to focus score of the labial minor salivary gland biopsy. RESULTS: Women had significantly higher antinuclear antibody titers and erythrocyte sedimentation rate than men. A significant sex difference was also noted in extraglandular manifestations, with more women reporting fatigue compared to men (68 vs 21%, respectively). CONCLUSION: This study indicates that women may have more positive serological findings than men and a higher prevalence of fatigue. No sex differences could be established with other extraglandular manifestations of SS.