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ID PMID Title PublicationDate abstract
10608146 [Detection of anti-ENA autoantibodies in patients with systemic connective tissue diseases 1999 Nov This study was designed to assess the analytical sensitivity and rate of agreement between commercial methods and reagents, among the most used in Italy for the detection of autoantibodies to extractable nuclear antigens (ENA). Sixty-eight serum samples from patients with clinically diagnosed systemic rheumatic diseases were aliquoted and distributed to 4 hospital laboratories; three ELISA (Elias, Shield, Inova) and 1 immunoblot method (Euroimmun) were used. Overall agreement between the test reagents, for each anti-ENA specificity, was 69.1% for Ro/SSA, 83.3% for La/SSB, 70.6% for RNP, 73.5% for Sm, 91.1% for Jo1, and 82.3% for Scl70. Lack of specificity (i.e., false positive reactions) was the most important cause of low concordance. When the data were analysed according to the clinical diagnosis, total agreement and specificity improved. However, a significant difference in terms of sensitivity was observed in the SLE group (30 sera) for RNP (positivity ranged from 20% to 43%) and for Sm (from 7% to 37%), and in the Sjögren's syndrome group (13 sera) for anti-La/SSB (from 8% to 38%). Comparable data were obtained for anti-Ro/SSA (from 70% to 77%) both in the SLE and the Sjögren's syndrome group. Sensitivity of all 4 reagents was good in detecting anti-Scl70 autoantibodies in the 8 patients with diffuse systemic sclerosis, as well as anti-Jo1 autoantibody in the 5 polymyositis patients, with a 100% and a 95% agreement, respectively. These data suggest the need of a better standardization of commercial reagents and analytical procedures, and the opportunity that every laboratory should perform anti-ENA determination by at least two different methods, since none of the methods tested was completely reliable in detecting all anti-ENA autoantibody specificities.
10405700 Autonomic failure and proximal skeletal myopathy in a patient with primary Sjögren syndro 1999 Jul Autonomic failure and proximal skeletal myopathy are rare features of the Sjögren syndrome (SS). We describe a 51-year-old woman with primary SS who had development of esophageal dysmotility, urinary retention, severe orthostatism, and skeletal myopathy during a 3-month period after the diagnosis of SS. Her symptoms and signs responded well to corticosteroid therapy. Although dysfunction of the peripheral nervous system has a prevalence rate of 20% in patients with SS, most commonly the nerve dysfunction is a sensory deficit, and autonomic neuropathy is less frequent. Autonomic neuropathy due to SS may be underreported. The cause of our patient's myopathy remains undetermined. We speculate that the myopathy was due to either a form of polymyositis or an immune-mediated neuropathy with muscle involvement.
10382238 Lung adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary Sjögr 1999 Jun We experienced a rare case of lung adenocarcinoma associated with lymphocytic interstitial pneumonitis caused by primary Sjögren's syndrome. A 78-year-old woman was referred to our hospital because of progressive sicca syndrome and nodular opacities in the right lower lobe on chest radiograph. This patient was diagnosed as primary Sjögren's syndrome by a labial gland biopsy and classical clinical features including xerophthalmia, xerostomia and immunoserological findings. Pathological findings including immunohistochemical studies in a surgically resected lung revealed adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary Sjögren's syndrome. There was no evidence of malignant lymphoma in the lymph nodes or resected lung tissue. Pulmonary involvement of Sjögren's syndrome is now regarded both clinically and histopathologically as a wide spectrum of lymphoproliferative disorders ranging from benign to malignant. However, lung cancer associated with primary Sjögren's syndrome, as in our case, has apparently not been reported previously.
9973150 Antineutrophil cytoplasmic antibodies in primary Sjögren's syndrome: prevalence and clini 1998 Dec OBJECTIVE: To evaluate the prevalence of cytoplasmic (c) and perinuclear (p) antineutrophil cytoplasmic antibodies (ANCA) in patients with primary Sjogren's syndrome (SS), and to correlate the presence of ANCA with extraglandular and immunological manifestations related to SS. METHODS: In a cross-sectional study, we included 82 consecutive patients (75 female and seven male; mean age 61 yr; range 33-87 yr) attending our unit. All patients fulfilled four or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. Extraglandular manifestations such as arthralgia and/or arthritis, Raynaud's phenomenon, autoimmune thyroiditis, peripheral neuropathy, renal involvement and cutaneous vasculitis were also recorded. Serum samples were examined by indirect immunofluorescence (IIF) and by ELISA using as substrates myeloperoxidase (MPO) and proteinase 3 (PR3). RESULTS: ANCA were detected in nine (11%) patients: seven had pANCA and two an atypical pattern. These two atypical ANCA became cANCA when paraformaldehyde fixation was applied. ELISA findings showed that two patients had antibodies against MPO, and no patient had antibodies to PR3. The most common extraglandular manifestations in the ANCA-positive patients were articular involvement in six (66%) patients, peripheral neuropathy in five (55%), Raynaud's phenomenon in four (44%) and cutaneous vasculitis in four (44%). Of the four patients with cutaneous vasculitis and ANCA, two had a mononuclear inflammatory vascular disease (MIVD) in the biopsy specimen. When compared with patients without ANCA, those with these antibodies had a higher prevalence of cutaneous vasculitis (44% vs 8%, P = 0.01), Raynaud's phenomenon (44% vs 8%, P = 0.01) and peripheral neuropathy (55% vs 7%, P < 0.001). CONCLUSION: ANCA positivity can be found in patients with primary SS and its detection is associated with the presence of clinical manifestations attributable to vascular involvement (cutaneous vasculitis, peripheral neuropathy and Raynaud's phenomenon).
9935271 Autonomic neuropathy associated with sicca complex. 1999 Jan 15 We retrospectively studied 52 patients with sicca complex who presented primarily with peripheral neuropathic symptoms to assess the degree of autonomic involvement and its relationship to somatic neuropathy, serological tests and extraglandular disease. One patient presented with severe panautonomic dysfunction, with another six patients reporting prominent autonomic symptoms, and a further 40 patients having mild or asymptomatic disease. Eighty-seven percent of patients showed abnormalities on an autonomic reflex screen (ARS). Fifty-eight percent of patients showed a mixed pattern of autonomic dysfunction with abnormalities in two or more ARS subgroups of sudomotor, cardiovagal and adrenergic function. Three patients had a cholinergic autonomic neuropathy with sparing of adrenergic function. Tonic pupils were present in 13 patients. Autonomic neuropathy occurred in patients with a variety of somatic neuropathy subtypes, without a clear relationship between the type of autonomic neuropathy and the type of somatic peripheral neuropathy. Extraglandular disease was uncommon, occurring in only four patients. Serological abnormalities (extractable nuclear antigen [ENA], SS-A, SS-B) occurred in 20% of patients. We conclude that: (1) A wide spectrum of autonomic dysfunction is seen in sicca complex. (2) Severe autonomic involvement is uncommon, with most patients showing mildly symptomatic or asymptomatic disease. (3) Overall, characteristic patterns of autonomic dysfunction did not predominate, and there were no clear relationships with the different types of somatic peripheral neuropathy. (4) Clinical and serological evidence of non-neurological extraglandular disease is uncommon in this group of patients.
9513208 [Autoantibody to glutamate decarboxylase in a patient with spinocerebellar degeneration an 1998 Feb We report a 52-year-old woman with Sjögren syndrome from the age of 46, developed cerebellar ataxia, autonomic dysfunction and dysarthria at 50. She had no family history, and all known causes of cerebellar disease were excluded. Serum of the patient contained autoantibodies directed against glutamic acid decarboxylase (GAD) which was an enzyme involved in the biosynthesis of GABA. She also had autoantibodies that were specific with Sjögren syndrome (SS-A, anti-nuclear antibody). Anti-GAD antibody changed into negative after high dose intravenous and oral corticosteroid therapy, but symptoms did not improve. Western blot method revealed abnormal bands to human neuroblastoma cell line (10, 43, 49 kDa), considered relatively specific to nervous tissue. In this case cerebellar ataxia and atrophy were caused by autoimmune pathogenesis including cerebellar GABAergic system and central nerve cells.
9372328 Neurological manifestations of primary Sjögren's syndrome in Japanese patients. 1997 Oct The neurological manifestations of twenty-one Japanese patients with Sjögren's syndrome (SjS) were evaluated. All patients were women, and sixteen of the twenty-one cases (76%) demonstrated objective abnormal neurological symptoms, the most frequently observed of which was trigeminal neuropathy (50%). Multiple mononeuropathy was seen in almost one-third of the examined cases (31%). Central nervous system (CNS) involvement was observed in three cases (14%). All of these values differed greatly from those previously reported. Therefore, this study revealed characteristic features of Japanese SjS and also implied the existence of different immunopathological mechanisms associated with SjS in Japanese patients.
9292815 Botulinum toxin increases tearing in patients with Sjögren's syndrome: a preliminary repo 1997 Sep Three patients with Sjögren's syndrome (SS) who had severe xerophthalmia and blepharospasm received botulinum toxin injections for the treatment of their blepharospasm. They had a remarkable increase in tearing, measured by Schirmer's test, and a decrease in signs and symptoms of dry eyes after botulinum toxin injection periorbitally for blepharospasm. The mechanism for this increased tearing is unclear, but suggests a potential treatment for patients with severe xerophthalmia with SS.
9340475 [Hyperferritinemia in Still syndrome in the adult and reactive hemophagocytic syndrome]. 1997 Aug 15 This report describes the fatal outcome of a case of adult onset Still's disease in a 46-year old man. The diagnosis was made according to the 1992 criteria, proposed by Yamaguchi. Nine months after the initial disease manifestations a rapid deterioration with progressive hepatosplenomegaly developed. In parallel, pancytopenia and marked hyperferritinemia could be detected. Transjugular liver biopsy revealed the presence of a hemophagocytic syndrome. The course of the disease was refractory to any form of treatment and the patient died from disseminated intravascular coagulation, hepatic and pulmonary failure. Pathogenetic mechanisms and possible associations between Still's disease and reactive hemophagocytic syndrome are discussed.
10374300 [Study on the relationship between primary Sjögren syndrome and HLA-DRbeta gene]. 1997 Jun The pathogenesis of primary Sjögren syndrome (PSS) is unclear yet. In order to investigate the role of genetic factor playing in the mechanism of this disorder, we studied the HLA-DR beta gene distributed in 70 patients with PSS and 136 normal subjects by using PCR-SSP technique. The results showed that the gene frequences of HLA-DR3, DR52 and DR2 in patients with PSS were significantly higher than that in normal controls. However, the gene frequencies of HLA-DR5 and DR9 in PSS patients were lower than that in the control group. There were the same genetic phenomena in the siblings of two PSS patients' families. We also found the relationship between HLA-DR52 and the anti-antibodies of SSA or SSB. Our results concluded that the genetic factor is involved in the pathogenesis of primary Sjögren syndrome.
9582702 The level of cariogenic micro-organisms in patients with Sjögren's syndrome. 1997 Mar Sixteen patients with caries-inactive sjögren's syndrome with low parotid salivary flow rates (< 0.25 mL/min) and 18 caries-inactive control subjects with higher salivary flow rates were compared. Mutans streptococci (MS) and lactobacilli (LB) counts were measured by means of Dentocult SM strip mutans and LB assays. The group with Sjögren's syndrome displayed higher counts of MS (P = 0.014) and LB (p = 0.003) when compared with controls. The results of this study indicate that patients with caries-inactive Sjögren's syndrome and low salivary flow may have higher colonization of cariogenic micro-organisms than healthy individuals.
11128698 Evaluation and differential diagnosis of keratoconjunctivitis sicca. 2000 Dec Patients with S ogren's syndrome (SS) have a high incidence of immune mediated ocular inflammation, making them more susceptible to infectious agents. However, the more common manifestation seen in patients with SS is immune mediated noninfectious inflammation of the lacrimal glands and ocular surface, resulting in decreased tear production and inflammatory changes on the ocular surface known as SS associated keratoconjunctivitis sicca (SS-KCS). Two categories of SS-KCS are recognized: aqueous deficient dry eye (decreased tear production by lacrimal glands) and evaporative dry eye (increased evaporative loss of tears from the ocular surface). There are a variety of tests for the differential diagnosis of SS-KCS, including the Schirmer test, with or without anesthetic; vital dye staining of the ocular surface for surface abnormalities; tear osmolarity; and lid margins evaluation. Current treatment of KCS involves artificial tears and punctal occlusion. New and emerging treatment options using androgens to suppress glandular inflammation and muscarinic M3 agonists, i.e., cevimeline and pilocarpine, show promise as treatment options for SS-KCS.
18020565 Psoriatic arthritis: recognition and management. 1998 Apr Psoriatic arthritis is an inflammatory arthritis associated with psoriasis, which is usually negative for rheumatoid factor. The disease displays a wide spectrum of articular and periarticular features which may progress with time. Although psoriatic arthritis was thought to be a benign condition, it is now clear that up to 20% of patients progress to significant joint damage and functional disability. Recently, several predictors for progression have been described and suggest the need for earlier, more aggressive, use of therapy in these patients. The ideal treatment for psoriatic arthritis is unclear, and few randomised controlled trials have been reported to date in this condition. Prospective evaluation of the treatment of early disease is now required to assess the effect of current and future therapies on the clinical, radiological and functional outcomes of this condition.
11089500 [Rapidly progressive acute renal failure. A rare complication of primary Sjogren syndrome] 2000 Oct 14 BACKGROUND: Renal impairment, usually due to tubulointerstitial and rarely glomerular disorders occurs in 10 to 30% of patients with primary Sjögren's syndrome. Extracapillary proliferation may also be observed. CASE REPORT: A 62-year-old woman with primary Sjogren's syndrome diagnosed 12 years earlier, developed microscopic polyangeitis leading to rapidly progressive renal failure. Antipolynuclear anticyclosine antibody (ANCA) serology was positive (pANCA, anti MPO) and the renal biopsy evidenced necrotizing glomerulonephrities with extracapillary proliferation. Outcome was favorable despite a recurrence one year after onset. DISCUSSION: Extracapillary proliferative glomerulonephritis is characterized by hematuria and proteinuria associated with renal failure. Renal impairment may worsen rapidly, sometimes leading to an emergency situation because the renal prognosis is directly related to delay to treatment. This case illustrates an uncommon complication of Sjögren's syndrome, compared with the usual tubulointerstitial disorders. The mechanism remains unknown, but outcome can be favorable with rapidly initiated immunosuppressor treatment.
10868017 [Mucocutaneous candidosis in a patient with Sjögren syndrome]. 2000 Apr Oral candidiasis is common among patients with Sjögren's syndrome. For the first time we report on a 60-year-old female patient, who developed a non-familiar chronic mucocutaneous candidiasis of later life. She presented with the following symptoms: oral candidiasis and glossitis, angulus infectious, vulvovaginitis, lichen planus-like onychodystrophy of the finger-nails and chronic nail bed inflammation of the nails of the big toes. The knowledge of chronic mucocutaneous candidiasis is of prognostic and therapeutic relevance, since topical treatment often fails.
9754007 [Interstitial pneumonitis detected by bronchoalveolar lavage and transbronchial lung biops 1998 Jun A 44-year-old woman was admitted to our hospital because of a high fever that had continued for three weeks. She complained of a sore throat and arthralgia, and had a salmon-pink rash, lymphadenopathy, liver dys-function, and hyperferritinemia. Tests for RF and ANA were negative. Adult-onset Still's disease was diagnosed. Despite administration of steroids, pericarditis, interstitial pneumonitis, and disseminated interavascular coagulation developed. After cyclophosphamide was given, the patient's condition improved, but reticular shadows and volume loss remained on the chest X-ray film. A chest CT scan showed ground-glass-like opacities and linear shadows, and irregular bronchovascular bundles. Bronchoalveolar lavage and transbronchial lung biopsy were done. Alveolar macrophages accounted for 71% of the cells in the bronchoalveolar lavage fluid, and lymphocytes (CD 4/ CD 8 ratio = 1.01) accounted for 29%. Examination of a specimen obtained by transbronchial lung biopsy showed thickened alveolar walls and infiltration of lymphocytes. Reports of cases of adult-onset Stills disease that include results of bronchoalveolar lavage and transbronchial lung biopsy are rare.
9086296 Salivary mucous glycoprotein MG1 in Sjögren's syndrome. 1997 Mar 18 The aim of the study was to develop and apply a rapid method for the simultaneous analysis of the concentration and molecular weight of the human high-molecular weight mucin MG1 in small volumes of unprocessed saliva from healthy controls and from patients with Sjögren's syndrome (SS). In high performance liquid chromatography (HPLC) with a TSK 5000 PW size exclusion column, MG1 eluted with a retention time 10.6 min corresponding to a M(r) of 2 to 2.5 x 10(6). Molecular weight changes under various experimental conditions are compatible with the suggestion that the MG1 complex is composed of four 660 x 10(3) glycosylated subunits connected by disulphide bridges and associated with a 25-35 x 10(3) Da link protein. In SS the molecular weight of MG1 was normal and its concentration was high in resting (190 vs. 70 micrograms/ml, P = 0.001) but not in stimulated (46 vs. 48 micrograms/ml, P > 0.05) saliva; MG1 concentration in resting SS saliva did not vary in parallel with protein and the interindividual differences were considerable. Size exclusion HPLC is a rapid and reproducible method suitable for isolation and analysis of salivary MG1 from small volumes of unprocessed samples. The molecular weight or subunit structure of MG1 were not altered in SS. The high concentration of MG1 in resting saliva in SS, may be explained by the concentration effect, or alternatively by the low water retaining capacity, which may play an important pathogenic role in xerostomia of SS.
11544344 IL-10-deficient B10.Q mice develop more severe collagen-induced arthritis, but are protect 2001 Sep 15 IL-10 is a pleiotropic cytokine with stimulatory and inhibitory properties, and is thought to have a protective role in rheumatoid arthritis and collagen-induced arthritis (CIA). In this study, we investigated how IL-10 deficiency affects CIA and anti-collagen type II (CII) Ab-transferred arthritis in C57BL/10.Q (B10.Q) mice. The B10.Q.IL-10(-/-) mice had an 8-cM 129/Ola fragment around the IL-10 gene. The mice were treated with antibiotics, appeared healthy, and had no colitis. T cells from IL-10(-/-) mice expressed similar levels of IFN-gamma, IL-2, and IL-4 after mitogen stimulation; however, macrophages showed a reduced TNF-alpha production compared with IL-10(+/-) littermates. IL-10(-/-) mice had an increased incidence, and a more severe CIA disease than the IL-10(+/-) littermates. To study the role of IL-10 in T cell tolerance, IL-10(-/-) were crossed into mice carrying the immunodominant epitope, CII(256-270), in cartilage (MMC) or in skin (TSC). Both IL-10(-/-) and IL-10(+/-) MMC and TSC mice were completely tolerized against CIA, indicating that lack of IL-10 in this context did not break tolerance. To investigate whether IL-10 was important in the effector phase of CIA, arthritis was induced with anti-CII Abs. Surprisingly, IL-10(-/-) were less susceptible to Ab-transferred arthritis, as only 30% showed signs of disease compared with 90% of the littermates. Therefore, IL-10 seemed to have a protective role in CIA, but seemed to exacerbate the arthritogenicity of anti-CII Abs. These data emphasize the importance of studying IL-10 in a defined genetic context in vivo, to understand its role in a complex disease like arthritis.
9574681 Genotypic characterization of seven strains of Mycoplasma fermentans isolated from synovia 1998 May We performed a genotypic characterization of seven strains of Mycoplasma fermentans which have been isolated from the synovial fluid of patients with rheumatoid arthritis (n = 2), spondyloarthropathy (n = 1), and unclassified arthritis (n = 4). We compared them to three reference strains (strains PG18 and K7 and incognitus strain) and to a clinical isolate from the urethra of a patient with nongonococcal urethritis. The characterization methods included electrophoresis of native DNA, arbitrarily primed PCR, and restriction fragment length polymorphism analysis following conventional and pulsed-field gel electrophoresis. Southern blot analysis with a probe internal to an insertion sequence was performed with the restriction products produced by the last two techniques. No extrachromosomal DNA sequences were detected. The M. fermentans strains identified by these methods did not present a unique profile, but they could be separated into two main categories: four articular isolates were genetically related to PG18 and the three other isolates, the urethral isolate, and the incognitus strain were related to K7. We also looked for the presence of the bacteriophage MAV1 (associated with the arthritogenic property of Mycoplasma arthritidis in rodents) in the M. fermentans strains. MAV1 DNA was not detected in either the clinical isolates or the reference strains of M. fermentans.
10990245 Monozygotic twins with primary Sjögren's syndrome. 2000 Sep Sjögren's syndrome (SS) is a chronic autoimmune disease mainly characterized by dry mouth and dry eyes due to an inflammatory process in the exocrine glands. We describe a pair of Caucasian monozygotic twin sisters and their mother, all having primary SS. The twins had very similar clinical presentation and almost identical serological data, and histological examination of lower labial salivary glands gave a focus score of 3 in both twins. We also present their family medical history, which shows aggregation of immunological disorders among family members, although the twins and their mother were the only individuals with primary SS.