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ID PMID Title PublicationDate abstract
17274865 [Macrophage activation syndrome in Chinese children with systemic onset juvenile idiopathi 2006 Nov OBJECTIVE: To review and analyze the clinical features, treatment, and outcome of macrophage activation syndrome (MAS) in children with systemic onset juvennil rheumatoid arthritis (SOJRA). METHOD: Retrospective review and analysis were performed on cases with MAS from a prospectively collected database of children with SOJRA from the year of 2003 to 2006 in the Hospital. RESULTS: Twenty four patients (21 boys, 3 girls) were diagnosed as having MAS with SOJRA. Mean age of the patients with MAS at diagnosis was 7 years, and the duration prior to diagnosis of MAS was 12 months. No trigger factors were found except in one case whose MAS was triggered by use of methotrexate and in another by parvovirus B19 infection. High grade fever, new onset hepatosplenomegaly and lymphadenopathy, pancytopenia, liver dysfunction were common clinical features in all the 24 cases (100%). Bleeding from skin, mucous membrane and gastrointestinal tract were noted in 9 cases (38%). Twelve (50%) cases had CNS dysfunction (high intracranial pressure, seizure and coma). Six cases (25%) developed ARDS. One patient suffered from renal damage. The laboratory test revealed elevated live enzymes and ferritin, decreased value of ESR, albumin, complete blood count and fibrinogen in all the 24 cases. Bone marrow examination supported the diagnosis of definite hemophagocytosis in the 24 cases. Lymph node biopsy was done for one case and histopathological examination showed that the node was full of activated macrophage. As to treatment, five cases only received high dose steroids (three of them died), 14 cases were treated with high dose steroids plus cyclosporine (one died), two were treated with steroids plus cyclosporine and etoposide (none died). The causes of deaths were ARDS and CNS involvement. In three of the cases who died, treatment was given up by their parents. CONCLUSIONS: MAS is a rare and potentially fatal complication of SOJRA. Most of our patients were male. Bone marrow studies support the diagnosis. CNS involvement and ARDS were poor prognostic signs. Early diagnosis and aggressive therapy are essential.
17645785 Celastrus aculeatus Merr. suppresses the induction and progression of autoimmune arthritis 2007 Complementary and alternative medicine products are increasingly being used for the treatment of autoimmune diseases. However, the mechanisms of action of these agents are not fully defined. Using the rat adjuvant arthritis (AA) model of human rheumatoid arthritis, we determined whether the ethanol extract of Celastrus aculeatus Merr. (Celastrus), a Chinese herb, can down-modulate the severity of AA, and also examined the Celastrus-induced changes in immune responses to the disease-related antigen mycobacterial heat-shock protein 65 (Bhsp65). AA was induced in the Lewis (LEW; RT.1l) rat by immunization subcutaneously with heat-killed M. tuberculosis H37Ra (Mtb). Celastrus was fed to LEW rats by gavage daily, beginning either before Mtb challenge (preventive regimen) or after the onset of AA (therapeutic regimen). An additional group of rats was given methotrexate for comparison. All rats were graded regularly for the signs of arthritis. In parallel, the draining lymph node cells of Celastrus-treated rats were tested for proliferative and cytokine responses, whereas their sera were tested for the inflammatory mediator nitric oxide. Celastrus feeding suppressed both the induction as well as the progression of AA, and the latter effect was comparable to that of methotrexate. Celastrus treatment induced relative deviation of the cytokine response to anti-inflammatory type and enhanced the production of anti-Bhsp65 antibodies, which are known to be protective against AA. Celastrus feeding also reduced the levels of nitric oxide. On the basis of our results, we suggest further systematic exploration of Celastrus as an adjunct therapeutic modality for rheumatoid arthritis.
18301366 Macrophage activation syndrome: a frequent but under-diagnosed complication associated wit 2008 Mar Macrophage activation syndrome (MAS) or hemophagocytic syndrome is a severe complication of chronic rheumatic diseases especially in systemic-onset juvenile rheumatoid arthritis (JRA). Although the cause of MAS is unknown, dysregulation of macrophage-lymphocyte interactions with subsequent increases in the levels of both T cell-derived and macrophage-derived cytokines could be involved in this syndrome, leading to an intense systemic inflammatory reaction, which accounts for the main clinical picture. Patients usually present with an acute febrile illness, hepatosplenomegaly, lymphadenopathy, cutaneous and mucosal bleeding, pancytopenia, and central nervous system, cardiac, and renal involvement. Treatment of MAS in patients with rheumatic diseases has not been standardized yet, but it commonly includes a variety of agents such as high-dose corticosteroids, cyclosporine, cyclophosphamide, etoposide, and intravenous immunoglobulin (IVIG). This article reviews the current literature about the pathogenesis, clinical manifestation, diagnosis, and treatment of this severe complication associated with rheumatic diseases.
19035421 Prevalence, severity, and predictors of fatigue in subjects with primary Sjögren's syndro 2008 Dec 15 OBJECTIVE: To investigate the relationship of fatigue severity to other clinical features in primary Sjögren's syndrome (SS) and to identify factors contributing to the physical and mental aspects of fatigue. METHODS: We identified 94 subjects who met the American-European Consensus Group criteria for the classification of primary SS. Fatigue was assessed with a visual analog scale, the Fatigue Severity Scale (FSS), and the Profile of Fatigue (ProF). Associations with fatigue were compared using multivariate regression. RESULTS: Abnormal fatigue, defined as an FSS score >or=4, was present in 67% of the subjects. Pain, helplessness, and depression were the strongest predictors of fatigue according to the FSS and the somatic fatigue domain of the ProF (ProF-S), both with and without adjustment for physiologic and serologic characteristics. Depression was associated with higher levels of fatigue; however, the majority of subjects with abnormal fatigue were not depressed. Anti-Ro/SSA-positive subjects were no more likely to report fatigue than seronegative subjects. The regression models explained 62% of the variance in FSS and 78% of the variance in ProF-S scores. Mental fatigue was correlated with depression and helplessness, but the model predicted only 54% of the variance in mental fatigue scores. CONCLUSION: Psychosocial variables are determinants of fatigue, but only partially account for it. Although fatigue is associated with depression, depression is not the primary cause of fatigue in primary SS. Investigation of the pathophysiologic correlates of physical and mental aspects of fatigue is needed to guide the development of more effective interventions.
18372542 [A non frequent cause of kidney disease]. 2008 Feb The EDTA (European Dialysis and Transplantation Association) statistic data on kidney diseases in ageing population point out vascular and/or metabolic origins. We report here a case of a rare cause of renal insufficiency, a Gougerot-Sjögren primary syndrome, diagnosed in an old patient. Renal and salivary gland biopsies were performed. The invasive investigation allowed an accurate diagnosis and an etiologic treatment.
16783831 Optimal nocturnal humidification for xerostomia. 2006 Sep BACKGROUND: Xerostomia is associated with a difficulty of maintaining oral hygeine and with symptoms of daytime oral discomfort and nocturnal sleep disturbance. METHODS: A pilot study was conducted to test the efficacy of a new method of delivering heated humidified air (37 degrees C, 100% water vapor saturated, at 20-25 L/min flow rate via specially designed nasal prongs) overnight for 2 weeks to 10 volunteers with Sjögrens Syndrome. Effectiveness was assessed by means of questionnaire, including the validated Xerostomia Inventory together with specific questions related to quality of sleep and oral comfort. RESULTS: Nine subjects completed the study. Daytime xerostomia symptoms were significantly reduced in participants (p = .004). Night-time oral comfort was significantly improved (p = .005) as was early morning mouth comfort (p = .008). Frequency of dry cough was also significantly reduced over the study period (p < .001). CONCLUSIONS: The body temperature, pressure saturated, delivery system was well tolerated and succeeded in alleviating the major oral and pharyngeal symptoms produced by xerostomia. These results have encouraged us to pursue further research in the application of this method of providing heated humidification.
16829497 Inflammatory cell and cytokine patterns in patients with chronic polyarthritis and temporo 2006 Aug OBJECTIVE: To investigate the occurrence of selected markers for inflammatory cells and cytokines in patients with chronic polyarthritis (CPA) and temporomandibular joint (TMJ) involvement. MATERIAL AND METHODS: Eleven patients (11 joints) with CPA and TMJ disorder were included in the study. Synovial specimens were obtained during TMJ open surgery and these were subjected to immunohistochemistry on frozen sections post-fixed with paraformaldehyde and with the cell membranes permeabilized by saponin. In all patients, the cytokines IL-1alpha, IL-1beta, IL-1ra, TNFalpha, IFNgamma, IL2, and TGFbeta were investigated using specific antibodies. The occurrence of macrophages and T-lymphocytes was investigated using immunohistochemistry with monoclonal antibodies against antigens CD68 and CD45RO, respectively. In addition, PCNA was used as a marker for cell proliferation. RESULTS: Staining of IL-1alpha, IL-1, and TGF was seen in all 11 specimens, IFN? in 1, TNFalpha in 4, and IL-2 in none. CD45RO-positive T cells were detected in 7 specimens, CD68-positive macrophages in 6, and cell proliferation seen with PCNA was noted in 8. CONCLUSIONS: The predominant cytokines of TMJ CPA were IL-1alpha, IL-1beta, and TGFbeta, and there appeared to be no differences between the subgroups (rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis) involved. Moreover, the cytokine pattern of TMJ CPA patients seemed to differ from patients with osteoarthritis, as shown in our previous study. The main difference was the absence of IFNgamma and TNFalpha in TMJ CPA patients and a stronger TGFbeta and IL-1alpha expression.
18564466 An unusual case of hypokalemic paralysis associated with primary Sjogren's syndrome. 2008 Jun 43-year-old Caucasian female presented with progressive weakness and dyspnea. She was diagnosed with hypokalemic paralysis from a severe distal renal tubular acidosis (RTA). Immunologic work-up showed a strongly positive ANA of 1:640 and positive antibodies to SSA and SSB. Schirmer's test was normal. Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede sicca complaints. The pathology in most cases is a tubulointerstitial nephritis causing among other things, distal RTA, and, rarely, hypokalemic paralysis. Treatment consists of potassium repletion, alkali therapy and corticosteroids. Primary SS should be a differential in premenopausal women with acute weakness and hypokalemia.
18411214 Autonomic nervous symptoms in primary Sjogren's syndrome. 2008 Jun OBJECTIVES: Objective signs of autonomic dysfunction (AD) have been reported in patients with primary SS (pSS) while the presence of associated symptoms has not been systematically studied. Therefore, the aims of this study were (i) to assess the presence and severity of various AD symptoms in pSS patients and (ii) to relate AD symptoms to other clinical features of pSS. METHODS: Thirty-eight pSS patients and 200 population-based controls were studied for presence and severity of AD symptoms using the Autonomic Symptom Profile (ASP), a validated self-completed questionnaire evaluating various AD symptoms. In addition, patients were investigated by three different objective autonomic nervous function tests. RESULTS: pSS patients scored significantly higher in the parasympathetic [secretomotor disorder, urinary disorder, gastroparesis (females only) and pupillomotor disorder] as well as sympathetic (orthostatic intolerance and vasomotor disorder) ASP domains compared with controls. Consequently, the standardized ASP total score was significantly increased in pSS patients [1.77 (0.57, 3.15) vs - 0.21 (-0.82, 0.72); P = 0.00] and 45% of pSS patients had an ASP total score >/=2 s.d. Furthermore, the autonomic nervous function tests showed signs of objective parasympathetic and sympathetic dysfunction as well. However, the ASP domain and total scores showed limited associations with the objective autonomic nervous function test parameters as well as clinical and serological factors of pSS. CONCLUSIONS: pSS patients showed subjective and objective signs of both a parasympathetic and a sympathetic dysfunction. However, AD symptoms showed limited associations with objective autonomic nervous function as well as other clinical features of the disease.
17334013 Primary Sjö-gren's syndrome presenting with distal, renal tubular acidosis and rhabdomyol 2006 Dec Primary Sjögren's syndrome (PSS) is rare in India. Clinically manifest renal disease in PSS is uncommon and is usually an autoimmune tubulointerstitial nephritis presenting with distal renal tubular acidosis (dRTA) or a urinary concentrating defect. Hypokalemic paralysis due to dRTA in PSS is rare but well documented in medical literature. Rhabdomyolysis as a consequence of hypokalemia in PSS is exceptional. We report a case of PSS with dRTA and rhabdomyolysis causing prolonged respiratory failure and quadriparesis.
17162200 Primary Sjögrens syndrome presenting as neuromyelitis optica. 2007 Jan This report presents a patient with Devics neuromyelitis optica associated with primary Sjögrens syndrome. Her first attack was right-sided optic neuritis at age 10 years. Attacks involving both optic nerves and medulla spinalis were recorded during the ensuing years. The diagnosis of Sjögrens syndrome could not be made until the second decade because it was not suspected.
16984944 Autoimmune thyroid disease is associated with a diagnosis of secondary Sjögren's syndrome 2007 Mar BACKGROUND: Autoimmune thyroid disease is common in systemic lupus erythematosus (SLE). About 20% of patients with SLE have secondary Sjögren's syndrome. METHODS: Families with more than one patient with SLE were identified. All patients met the revised classification criteria, although SLE-unaffected relatives were confirmed not to satisfy these criteria. Diagnosis of autoimmune thyroid disease and Sjögren's syndrome was made on the basis of a review of medical records, interview and questionnaire administered to patients with SLE, and by a questionnaire administered to SLE-unaffected subjects. RESULTS: Of a total of 1138 patients with SLE, 169 had a diagnosis of Sjögren's syndrome. Of these 50 (29.6%) patients also had autoimmune thyroid disease. Of the 939 patients with SLE with no diagnosis of Sjögren's syndrome, 119 (12.7%) had autoimmune thyroid disease (chi2 = 20.1, p = 0.000009). There was no association of a diagnosis of hypertension with secondary Sjögren's syndrome (42% vss 47%). Among 2291 SLE-unaffected relatives, 44 had diagnosed primary Sjögren's syndrome and 16 (36.3%) of these also had autoimmune thyroid disease. 265 of 2247 (11.8%) subjects had autoimmune thyroid disease but no Sjögren's syndrome (chi2 = 24.2, p<0.001). CONCLUSIONS: Autoimmune thyroid disease is found in excess among patients with SLE with a diagnosis of secondary Sjögren's syndrome, as well as among their SLE-unaffected relatives with a diagnosis of primary Sjögren's syndrome.
19120768 Postmenopausal frontal fibrosing alopecia in a Japanese woman with Sjögren's syndrome. 2008 Nov Postmenopausal frontal fibrosing alopecia (PFFA) is a rare alopecia that develops in the frontoparietal scalp of postmenopausal women. Etiology of PFFA is unknown. Most of cases of PFFA have been reported in European and North American countries. Herein, we report a Japanese case of PFFA associated with Sjögren's syndrome. A 66-year-old woman had had slowly progressive, band-like, scarring alopecia on her frontoparietal scalp. Hair follicles on the margin showed follicular keratosis. Histologically, fibrosis and lymphocytic infiltration were mild. This case suggests that PFFA may show mild inflammatory reaction and mild fibrosis in Japanese women. The association with immunological disorders including Sjögren's syndrome should be studied further.
18379789 Cryptogenic organizing pneumonia associated with primary Sjogren's syndrome. 2008 Aug The association of cryptogenic organizing pneumonia (COP) with primary Sjogren's syndrome (PSS) is extremely rare. We report a case of simultaneous diagnosis of PSS and COP. A 70-year-old female presented with fever, non-productive cough and dyspnea of 2 months' duration. She had experienced sicca symptoms for the past 2 years. The chest radiograph revealed a right lower lobe infiltrate, which was unresponsive to antibiotics. Bronchoscopy, bronchoalveolar lavage and an open lung biopsy established the diagnosis of COP, while a lip biopsy was consistent with PSS. The patient improved on steroids. Organizing pneumonia may be one of the early manifestations of PSS. Exclusion of PSS should be part of a thorough evaluation of the patient with COP.
18032543 Sjögren's Systemic Clinical Activity Index (SCAI)--a systemic disease activity measure fo 2007 Dec OBJECTIVE: This article describes the development of the Sjögren's Systemic Clinical Activity Index (SCAI) for the measurement of systemic disease activity in patients with primary Sjögren's syndrome (PSS). METHODS: A pilot tool was developed based on expert consensus and previous published data. One hundred and four patients with PSS were evaluated in a cross-sectional analysis, of whom 65 were reviewed at 3-monthly intervals, using this index, over a 12-month period. Factor analysis was used to evaluate the proposed domain structure. External validation was assessed by comparison with relevant domains of the Profile of Fatigue and Discomfort (PROFAD), Medical Outcomes Study Short Form-36 (SF-36) and The World Health Organization Quality of Life-Bref (WHOQOL-BREF). Sensitivity to change was assessed by comparing SCAI-derived flares with physician-designated disease flare and intention-to-treat analysis. A reliability and repeatability workshop was also held. RESULTS: Factor analysis supported the proposed domain structure. There were strong correlations between the SCAI fatigue, musculoskeletal and Raynaud's components and the PROFAD fatigue, arthralgia and vascular domains. There was a significant correlation between change in therapy and SCAI-defined flares (P = 0.01). The mean kappa-test results both for reliability of the SCAI and for physician repeatability were 0.71. CONCLUSION: This initial evaluation supports the potential for the SCAI as a tool for systemic activity assessment in patients with PSS but additional work is required to assess sensitivity to change in clinical therapeutic trials.
16957888 Successful treatment of acquired amegakaryocytic thrombocytopenia with cyclosporine in adu 2007 Jan Acquired amegakaryocytic thrombocytopenia (AAT) is a rare disorder, characterized by severe thrombocytopenia and selective, marked decrease or absence of megakaryocytes in the bone marrow. We describe a 29-year-old female with adult onset Still's disease preceding a diagnosis of AAT and autoimmune hemolytic anemia, which was successfully treated with cyclosporine. This is the first case of AAT in a patient with adult onset Still's disease.
18546945 [Transient evoked otoacustic emission in children with juvenile idiopatic arthritis]. 2007 Juvenile idiopathic arthritis (JCA) is an inflammatory systemic disease of connective tissue which etiology is still unknown. Progressive arthritis is the basic symptom, with pain, oedema, stiffness and movement impediment are typical for. Chronic process of arthritis might be accompanied by rheumatoid nodes, pericarditis, myocarditis, changes in the lungs and kidney, which appear with various frequency and indicates on systemic form of disease. The incudo-malleolar and incudo-stapedial joints are synovial in type. It should be expected that these joints might be affected by rheumatoid changes similar to those observed in joints in other parts of the body. Copeman was the first who described transient conductive hearing loss in patients with exacerbated rheumatoid process--and he called it oto-arthritis. He stressed that it was the consequence of the rheumatoid lesions in joints of ear ossicules. In adult patients with JCA in 60% of cases the perceptive hearing-loss is observed possibly because of the chronic course of the disease and its farmacological treatment. The aim of the study was the assessment of the functional state of the peripheral part of hearing organ on the basis of TEOAE. This study was preceded by examination of hearing with pure tone audiometry, speech audiometry, high frequency audiometry and impedance audiometry. The analysis of results was done in correllation to form of JCA and its activity. The research was carried out on the group of 45 children suffering from JCA; 14 boys and 31 girls between 5-18 years of age. In all children from experimental group the examination was performed in the active phase of disease process. Mean duration of the disease was 33.5 month. In all children TEOAE was obtained no matter on the form of JCA. Children with systematicus form of JCA--more aggressive type than others--have the recordings of TEOAE with small amplitude and narrow range of frequency. Those results show that rheumatoid lesions in conductive mechanism of the middle ear are insufficient for the development of the conductive hearing loss. As a results of chronic pathological process destructions of hearing organ may lead to the cochlear lesions and this way to perceptive hearing loss.
16924821 [Polyarticular gout in young adults: a curable rheumatic disease]. 2006 Jun Juvenile chronic gout in its polyarticular deformative form has rarely been described in medical literature. We report a rare case of destructive polyarticular tophaceous gout in a 31-year-old Senegalese man. He consulted for bilateral asymmetric polyarthritis with deformities of the hands and feet that had been ongoing in recurrent episodes since the age of 18 years in association with tophus. He had received no previous medication. All laboratory investigations were normal except hyperuricemia 104 mg/l. Radiographs of affected joints demonstrated evidence of destructive polyarthritis, i.e., articular narrowing and osteo-condensation of the left great toe. The patient responded favourably to colchicine, allopurinol and diet. Gouty arthropathy must be differentiated from rheumatoid arthritis, psoriasic arthritis and distal chronic osteoarthrosis. In our case, definitive diagnosis of gouty arthropathy was based on chronic polyarthritis associated with tophus, hyperuricemia and therapeutic response to colchicine. Polyarticular gout can be suspected in case of chronic seronegative polyarthritis and diagnosis can be confirmed on the basis of plain radiographs and laboratory investigations showing uricemia. Treatment is effective, well tolerated and inexpensive.
17893970 Advances in lupus and Sjögren's syndrome: a tribute to Josep Font. 2007 Jun Dr. Josep Font (Barcelona, 1953-2006) devoted his professional career to the care of patients with systemic autoimmune diseases (SAD). In 1995, he created the Department of Autoimmune Diseases at the Hospital Clinic in Barcelona, a pioneering unit in Europe specifically dedicated to the clinical management of these patients. The research output of Dr. Font has been prodigious, with a total of over 500 scientific articles published in 25 years, and was especially focused on the clinical and immunological characterization of systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (SS). This review briefly analyzes some of the main contributions of Josep Font to our current knowledge about these diseases. In SLE, the research was centered on the epidemiological and clinical characterization, the study of cardiovascular and vasculitic involvements, and the investigation of the etiopathogenic role of the innate immunity. In primary SS, basic research conducted by Dr. Font contributed to a better understanding of the autoimmune etiopathogenesis of the disease, while the clinical research expanded our knowledge about the systemic and immunological expression of the disease and its frequent association with lymphoma, other SAD, and chronic viral infections.
18942309 Tubercular synovitis mimicking rheumatoid nodules. 2008 Jun Tubercular tenosynovitis is an uncommon condition and usually affects the upper limb. We report a case of a patient with Systemic Lupus Erythematosus who presented with wrist swelling. The clinical findings were suggestive of rheumatoid nodules, but the radiographic finding of calcification associated with the nodules and marked erosive changes primarily of the radio-carpal joint with sparing of the metacarpal joints led the radiologist to believe that the nodules may not be rheumatoid nodules. The presence of solid and fluid nature of the nodule and hyperechoic small echogenic foci (matted rice bodies within thickened synovium) on ultrasound suggested the presence of chronic synovitis of tuberculous infection rather than rheumatoid nodule as in our case. We recommend the use of ultrasound to determine the nature of nodular swellings seen clinically in patients with arthropathy.