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ID PMID Title PublicationDate abstract
1925257 [Significance of subjective symptoms in the diagnosis of hyperviscosity syndrome]. 1991 Apr A questionnaire including 9 main signs: headache, vertigo, vision and hearing derangement, fatigue, hemorrhage, somnolescence, loss of appetite has been elaborated and introduced into the clinical practice. It was revealed that patients with Sjögren's syndrome had an elevated viscosity of the serum in 30% of the cases. These patients complained of headache and vision derangement more often than those with normal indices, though in the first group it did not reach critical values corresponding to an extended clinical picture of the hyperviscous syndrome. The mentioned questionnaire can be used for early diagnosis of the rheological properties of the blood.
2244254 [An autopsied case of PSS associated with Sjögren's syndrome and secondary generalized am 1990 Jun Fifty year-old female with progressive systemic sclerosis associated with Sjögren's syndrome who died of meteorism, diarrhea, bleeding of digestive canal due to amyloid deposition in the intestine and jaundice was reported. Autopsy revealed pathological findings compatible with that of PSS and Sjögren's syndrome. Furthermore, deposition of amyloid materials were shown in tissue of digestive canal, thyroid gland, heart muscles, lymphnodes, and small vessel wall of the whole body. Amyloid material was demonstrated to be A-A protein based upon the loss of Congo red stain after exposure to potassium permanganate, and reaction to anti A-A protein antibody. Although obstruction of intrahepatic bile duct, especially in Hering canal, could be relating to some unknown factors secondary to intestinal amyloidosis, further studies will be required to clarify the pathogenesis of it.
2520352 Muscle ultrastructure in a case of mixed connective tissue disease and Sjögren's syndrome 1989 A 29-year-old black woman with clinical and serological diagnoses of Sjörgren's Syndrome (SS) and mixed connective tissue disease (MCTD) presented a very important muscular compromise with proximal, bilateral weakness and wasting, an abnormal electromyogram, and elevated muscle enzymes. Ultrastructural study of a muscle biopsy showed different degrees of atrophy and presence of subsarcolemmal loss of sarcomeric arrangement forming regions of contractil material with diverse electron densities. Capillaries were proliferative with distortion or occlusion of lumen and thickened basement membranes. An unusual capillary abnormality consisting of thick and convoluted basement membrane intermingled with portions of endothelium and macrophages is described. Mononuclear cell infiltration was formed by lymphocytes, plasma cells, macrophages and scarce mast cells. This work is the first study of the ultrastructural muscle pathology in a patient with diagnoses of MCTD and SS based on clinical findings and the presence of antibodies to RNP, SS-A and SS-B antigens.
3055038 Parotid gland: US findings in Sjögren syndrome. Work in progress. 1988 Dec To determine the value of sonography of the parotid gland in patients with Sjögren syndrome, six consecutive cases were retrospectively reviewed in which clinical findings indicated Sjögren syndrome. All patients were women. Two patients were found at sonography to have normal parotid glands. Four patients had multiple cystic changes in the parotid glands bilaterally. Each of these four patients had a clinical diagnosis of Sjögren syndrome. One of the four underwent sialography, which demonstrated characteristic sialectasia. Pathologic examination of an excised gland in one of the patients with Sjögren syndrome demonstrated typical benign lymphoepithelial aggregates, which cause obstruction and progressive ductal dilatation. While parotid enlargement in these patients may be due to salivary tumor, lymphoma, or sarcoidosis--all of which appear as solid lesions at sonography--this study suggests that bilateral cystic changes may be the rule in uncomplicated cases of Sjögren syndrome.
3706962 [Severe pulmonary fibrosis disclosing primary Gougerot-Sjögren syndrome. Apropos of 5 cas 1986 Five cases (four women and one man) with primary Gougerot-Sjögren syndromes presenting as interstitial pulmonary fibrosis are reported. The main point of interest was the severity of the pulmonary changes: effort dyspnoea from the outset, radiological interstitial pneumonitis, restrictive syndrome with a CO diffusing capacity less than 50 p. 100 of theoretical values in 4 cases. Broncho-alveolar lavage was performed in all patients: it showed a high polynuclear neutrophil and particularly high eosinophil counts. Lung biopsy showed severe fibrotic changes. The pulmonary changes dominated the clinical presentation in all patients and the Gougerot-Sjögren syndrome was only diagnosed after systematic examination completed by biopsy of the accessory salivary glands. In the absence of an associated connective tissue disease, these cases of the Gougerot-Sjögren syndrome were considered to be primary. Four patients were given steroid therapy but this did not prevent a fatal outcome in two cases after 6 and 14 months respectively. The clinical states of the other patients were unaffected by treatment. Twenty-one other, but less severe cases, have been previously reported. They confirm that interstitial pulmonary fibrosis observed during the Gougerot-Sjögren syndrome does not differ from idiopathic pulmonary fibrosis and that observed in other connective tissue diseases. Nevertheless, analysis of our cases showed that biopsy of the accessory salivary glands should be part of the routine investigation of apparently idiopathic pulmonary fibrosis. These pulmonary changes may be life-threatening when associated with a primary Gougerot-Sjögren syndrome.
3945875 Central nervous system manifestations of Sjögren's syndrome. A case report. 1986 Feb 1 A patient with primary Sjögren's syndrome and a previous history of systemic vasculitis is presented to document neurological disease which suggests involvement of the brainstem and cerebellar connections with neuropsychiatric manifestations. The investigations and management are briefly outlined.
2686802 Major upper airways obstruction associated with Sjögren's syndrome: a case report and lit 1989 Dec This short report describes the development of life-threatening upper airways obstruction in a patient with primary Sjögren's syndrome (PSS), and reviews the published literature on this unusual development.
3229032 Effects of nifedipine therapy on pulmonary Raynaud's in primary Sjögren's syndrome. 1988 Oct A case of Raynaud's phenomenon of the pulmonary vascular bed in Primary Sjögren's syndrome and subsequent response to calcium channel blockade is presented. Pulmonary Raynaud's phenomenon in Primary Sjögren's syndrome has not been previously described.
3621682 A mononuclear phagocyte subset associated with cell necrosis in rheumatoid nodules: identi 1987 Oct A subset of mononuclear phagocytes identified by mAb 5.5 has been found within rheumatoid subcutaneous nodules. These cells appear to be derived directly from blood-borne monocytes. They form a variable proportion of the mononuclear phagocyte population within the nodule and are intimately associated with the tissue necrosis which characterizes these lesions. Necrosis appears within small aggregates of mAb 5.5-positive cells. Similar cells are also associated with localized extensions of necrosis through the palisade zone. Centrifugal extension of the necrotic centers through incorporation of the inner cells of the palisade layer appears to be an independent phenomenon.
3602794 [Primary Gougerot-Sjögren syndrome with necrotizing polymyositis: favorable effect of hyd 1987 A 61-year-old woman was admitted for acute arthralgias and proximal weakness in all four limbs. Clinical examination found xerostomia, xerophthalmia, enlarged parotid glands. The Schirmer test was positive and the salivary gland biopsy showed a mononuclear, lymphoid, sometimes nodular infiltrate. A muscle biopsy showed necrotizing myopathy with perifascicular atrophy. The patient refused steroids and was given hydroxychloroquine (600 mg daily). She improved gradually, and one year later there were no further complications. The favorable course both of Sjögren's syndrome and polymyositis with hydroxychloroquine treatment is unexpected. This led us to suppose that both could have had a common cause.
2538774 Peripheral neuropathy in primary Sjögren's syndrome. 1989 Mar Sjögren's syndrome (dryness of eyes, mouth, and other mucous membranes) may be associated with disease of joints, blood, internal organs, skin, and central and peripheral nervous systems. We reviewed 33 cases of primary Sjögren's syndrome and peripheral neuropathy evaluated by neurologic examinations and EMG at the Mayo Clinic from 1976 to 1988, and studied sural nerve biopsy specimens in 11 of them. Symmetric sensorimotor polyneuropathy occurred most frequently, followed by symmetric sensory neuropathy. Autonomic neuropathy, mononeuropathy, or cranial neuropathy (especially trigeminal neuropathy) was superimposed on generalized neuropathy in approximately one-fourth of patients. The course generally was slowly progressive, except for a few patients who may have improved with prednisone therapy. Although spinal ganglion involvement might have accounted for some of the clinical and neurophysiologic findings, we found evidence that necrotizing vasculitis was involved in fiber degeneration. All nerve biopsies revealed perivascular inflammatory infiltrates and other vessel abnormalities, which were diagnostic in two cases and strongly suggestive of necrotizing vasculitis in six cases. Axonal degeneration predominated over demyelination and sometimes was focal or multifocal. In neuropathy of unknown cause, particularly if it is sensory, autonomic, or involves trigeminal nerve, consider Sjögren's syndrome.
3262752 Optic neuropathy as an initial manifestation of Sjögren's syndrome. 1988 Although optic neuropathy is a known complication of systemic lupus erythematosus, its association with other connective tissue diseases is less well documented. We describe 3 previously asymptomatic women presenting with optic neuropathy in whom changes highly suggestive of primary Sjögren's syndrome (SS) were found by minor salivary gland biopsy 1-6 years after initial presentation. Typical symptoms of SS were absent initially and developed slowly, while serologic abnormalities were consistently present. Optic neuropathy may be a presenting neurologic feature of SS and may antedate typical signs and symptoms of SS. Primary SS should be considered in the differential diagnosis of optic neuropathy.
3050084 Primary juvenile Sjögren's syndrome. 1988 Primary Sjögren's syndrome has rarely been reported in children. We report 3 patients who developed lacrimal and salivary gland involvement at ages 3, 7 and 9, respectively. The one who began at age 3 is the youngest we could find reported. Sjögren's syndrome-type involvement was documented objectively at both sites and associated connective tissue diseases were ruled out in all 3 patients.
2670260 Sjögren's syndrome and the gut. 1989 Aug Sjögren's syndrome is the result of lymphocyte-mediated destruction of exocrine glands that leads to diminished or absent glandular secretions and mucosal dryness. The manifestations from the alimentary system in patients with Sjögren's syndrome include, within the mouth, mucosal dryness, atrophy, accelerated dental decay and enlargement of the major salivary glands. Dysphagia is a common complaint and is probably secondary to oesophageal dysfunction. The symptoms from gastric involvement are nausea, epigastric pain and dyspepsia which might be attributable to chronic atrophic gastritis. Whether the small bowel is affected in Sjögren's syndrome patients is not clear. However, nutritional deficiencies have been noted in these patients. Pancreatic involvement is perhaps expressed as subclinical, acute or chronic pancreatitis, and finally there have been a large number of studies dealing with liver involvement in Sjögren's syndrome. From these reports it is clear that many patients with Sjögren's syndrome have abnormal biochemical liver function tests and some of them may also have abnormal liver biopsy. The pathogenic process responsible for the hepatic damage and for the salivary gland destruction could be similar.
2920746 Recurrent parotid gland enlargement as an initial manifestation of Sjögren syndrome in ch 1989 Feb Recurrent parotid gland enlargement is a common disorder in children, while that of auto-immune aetiology is rare. Three children with recurrent parotid swelling had autoantibodies including antinuclear antibody, anti-SS-A (Sjögren syndrome-A), SS-B (Sjögren syndrome-B) antibodies and rheumatoid factor, abnormal sialograms and lymphocytic infiltration of salivary glands, which were consistent with Sjögren syndrome. Initially, all three lacked symptoms of keratoconjunctivitis sicca. During follow up, two patients developed xerostomia and were diagnosed as having primary Sjögren syndrome. Recurrent parotid enlargement appears to be important as an initial manifestation of Sjögren syndrome in children.
2646362 Sinusoidal lymphocytosis of the liver in Felty's syndrome with a review of the liver invol 1989 Feb A 68-year-old woman with Felty's syndrome had massive hepatomegaly. Liver biopsy showed diffuse infiltration of the sinusoids with mature lymphocytes (sinusoidal lymphocytosis of the liver). Nodular regenerative hyperplasia of the liver and portal fibrosis were not found. Although liver involvement is frequent in association with Felty's syndrome, sinusoidal lymphocytosis of the liver is unusual.
3419610 Sensory neuronopathy and Sjögren's syndrome: clinical and immunologic study of two patien 1988 Oct In two patients, a sensory neuronopathy was the initial presentation of Sjögren's syndrome. There was no systemic or peripheral nerve vasculitis. Immunochemical studies failed to demonstrate specific antibodies against the nervous system. These features suggest that the damage to the sensory neurons might not be mediated by humoral immune mechanisms.
3381023 Association of Sjögren's syndrome and sarcoidosis. Report of a case. 1988 Mar We report a case of a 57 year-old woman with Sjögren's syndrome and pulmonary complaints. After an initial evaluation, a lung biopsy was made and disclosed sarcoid lesions. This association is very rare, and the present case does not fulfill the criteria for TASS (Thyroiditis, Addison's disease, Sjögren, and Sarcoidosis). Sarcoidosis and Sjögren can coexist since both diseases may share the same immunological profile. This case report warrants further research on the overlapping of these diseases.
3718016 HLA antigens in Italian patients with primary Sjögren's syndrome. 1986 May Twenty eight Italian patients with primary Sjögren's syndrome were typed for class I and class II alloantigens of the major histocompatibility complex. Patients with Sjögren's syndrome had higher prevalence of DR3 (46.4% v 14% in the control population, p corrected less than 0.02), while similar prevalence was found for DR2 and DRw52 alloantigens. DR3 correlated with DRw52 (p less than 0.0001), anti-Ro(SSA) (p less than 0.0002) and anti-La(SSB) (p less than 0.02) antibodies, and extraglandular manifestations (p less than 0.02). In addition, extraglandular involvement was associated with anti-Ro antibodies (p less than 0.05) and raised gammaglobulins (p less than 0.02). In Italian patients with primary Sjögren's syndrome DR3 is the genetic marker related to this clinical entity and seems to identify a disease subset characterised by autoantibody production and extraglandular manifestations.
2078973 Impaired oxidative metabolism of salicylate in Reye's syndrome. 1990 Administration of salicylates during prodromal viral illness has been associated with the development of Reye's syndrome (RS). We studied salicylate biotransformation in RS patients and compared it with those on chronic salicylate therapy for juvenile rheumatoid arthritis (JRA). Urine of RS patients contained significantly more salicylic acid and less gentisic acid than that of JRA patients while the conjugated metabolites were not different between the two groups. These results suggest decreased salicylate microsomal oxidation in RS. The role of altered salicylate metabolism in the pathogenesis of RS is unclear.