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ID PMID Title PublicationDate abstract
8531362 [Renal involvement in Sjögren's syndrome--interstitial nephritis and glomerulonephritis]. 1995 Oct Renal involvement is well recognized extraglandular manifestation of primary Sjögren's syndrome (SS). The most common histopathological lesion is an interstitial lymphocytic infiltrate with tubular atrophy and fibrosis. The clinical presentation may be hyposthenuria, overt or latent distal renal tubular acidosis and less commonly Fanconi's syndrome. These tubular dysfunctions correlate with the presence of interstitial lymphocytes. Immunoregulatory alterations consisting of impaired T-cell function and B-cell hyperactivity probably play a pathogenetic role in the development of interstitial nephritis in SS. Glomerulonephritis in SS has been described in a limited number of case reports. Variant modes of pathogenesis have been proposed in these cases where glomerulonephritis has been associated with immune complex deposition and cryoglobulinemia.
1578459 Low dose methotrexate treatment in adult Still's disease. 1992 Mar Six patients with adult Still's disease who had either failed to respond to or had adverse effects from previous therapy were given weekly low dose methotrexate (MTX) therapy. They were followed for 4-28 months (mean 14 +/- 9 months). At 12 months of therapy 3 patients were evaluated as having a complete response. One patient had a partial response to MTX after 7 months. Therapy was discontinued in 1 patient at 4 months due to flares of rash and arthralgias after each MTX administration. One patient failed to respond to therapy despite maximum dosage, but was able to reduce his corticosteroid dose. MTX may be a useful therapy to consider in patients with adult Still's disease who are resistant to conventional therapy and may allow a reduction in the concomitant dose of corticosteroids.
8839813 a 49-year-old woman with dyspnoea, palpitations and syncope. 1996 Jul 5 Pulmonary hypertension is rarely described in association with Sjögren's syndrome. The authors report the case of a patient in which pulmonary hypertension was the inaugural clinical manifestation of primary Sjögren's syndrome. Clinical assessment, differential diagnosis, etiopathological implications, and therapeutic approach are discussed.
8150385 Trigger wrist induced by finger movement. Pathogenesis and differential diagnosis. 1994 Jan Four cases of trigger wrist induced by finger motion are reported. The cause of trigger wrist induced by finger motion was a rheumatoid nodule in one case, giant cell tumor of the flexor tendon sheath in one case, partial laceration of the flexor digitorium superficialis tendon in one case, and lipofibroma in one case. Triggering or snapping at the wrist is also induced by motion of the wrist and forearm. Reported cases were also analyzed and the clinical entity of the true trigger wrist and differential diagnosis were discussed.
8256579 Aseptic meningoencephalitis in primary Sjögren's syndrome. Response to plasmapheresis and 1993 Oct It has been suggested that cerebral vasculitis is the pathogenetic mechanism of the central nervous system manifestations of primary Sjögren's syndrome. We present a fatal case of aseptic meningoencephalitis in an 18-year-old woman with primary Sjögren's syndrome in whom there was no evidence of cerebral vasculitis at autopsy. On two occasions her condition improved markedly following plasmapheresis.
8358991 Astasia-abasia revealing a primary Sjögren's syndrome. 1993 Jun A 63-year-old woman presented with mild arthralgias and inability to stand and walk without other significant neuropsychiatric abnormality. Neurological explorations showed intrathecal oligoclonal immunoglobulin production in CSF and numerous bright foci at brain MRI. A primary Sjögren's syndrome was concomitantly evidenced and was thought to be the cause of the astasia-abasia symptom by multiple brain involvement. Patient's condition gradually improved with hydroxychloroquine and corticosteroid therapy. Central nervous system involvement by primary Sjögren's syndrome is discussed.
8531355 [Detection of retrovirus in salivary glands of patients with Sjögren's syndrome]. 1995 Oct As similar symptoms and glandular pathology are observed in certain persons infected with human immunodeficiency virus (HIV) and human T lymphotropic virus type I (HTLV-I), a search was initiated for a possible retroviral etiology in Sjögren's syndrome (SS). Sera from 33% of 15 SS patients reacted against p24 (gag) of HIV. Salivary gland (SG) biopsy specimens from 47% of the 15 SS patients contained an epithelial cytoplasmic protein, reactive with a monoclonal antibody to p24 of HIV. We also detected Mn2+ dependent, Mg2+ independent reverse transcriptase activity in the SG 3 of 10 SS patients. Target genes for HIV and HTLV-I were not found in any of the SG or peripheral blood mononuclear cells from SS patients. These data suggest the presence of an unknown retrovirus, similar to HIV, in the SG, which could contribute to the chronic inflammation of SS.
7481594 Small nerve fibre dysfunction in a patient with Sjögren's syndrome. Neurophysiological an 1995 An morphological confirmation of neurophysiologically assessed small nerve fibre dysfunction in a patient with Sjögren's syndrome was sought. Conventional motor and sensory nerve conduction studies were normal, while examination of the thermal specific and thermal pain sensitivity and an autonomic nervous system evaluation showed striking abnormalities. Sural nerve electron microscopy showed almost normally large nerve fibres and abnormalities in unmyelinated nerve fibres or Schwann cells, and verified morphologically the neurophysiologically supposed small nerve fibre involvement.
8239176 Morphometric analysis of airways in Sjögren's syndrome. 1993 Nov Sjögren's syndrome is characterized by a decrease in exocrine gland secretion and the destruction of secretory tissue and cells, that is, salivary and lacrimal glands. Although patients with Sjögren's syndrome have chronic cough and sometimes sputum, we have little knowledge concerning the morphology of their airways. We performed morphometric analysis of goblet cells and bronchial glands and compared them with intraluminal mucus volume by measuring the mucus-occupying ratio (MOR) in the airways of patients with Sjögren's syndrome. A total of 6 nonsmoking patients with Sjögren's syndrome who had no significant prolonged airway infections (Group SG) (6 women, 55 +/- 2 yr) were compared with four control patients with no pulmonary diseases (Group NL) (1 woman and 3 men, 64 +/- 2 yr) and five patients with chronic bronchitis (Group CB) (1 woman and 4 men, 52 +/- 5 yr). Autopsied lungs from 15 patients were used for morphometry by a digitalizing computer. Area proportion of bronchial glands (gland %) to bronchial wall, goblet cell-occupying ratio to total epithelial layer (goblet %), and MOR were measured in central (3-8 mm in diameter) and peripheral airways (2 mm or less in diameter). Gland % and goblet % in central and MOR in peripheral airways from Group SG were significantly larger than those from Group NL: 15 +/- 2 versus 6 +/- 1% (p < 0.01), 6 +/- 1 versus 2 +/- 1% (p < 0.05), and 9 +/- 2 versus 0.6 +/- 0.3% (p < 0.05), respectively. Further, these values from Group SG did not significantly differ from those from Group CB.(ABSTRACT TRUNCATED AT 250 WORDS)
8314692 Hypokalemic flaccid paralysis as the presenting symptom of autoimmune interstitial nephrop 1993 May We describe a patient who presented dramatically with life-threatening hypokalemic flaccid paralysis. Following resuscitation, extensive investigation showed that the patient had lupus erythematosus (SLE) and Sjogren's syndrome (SS) of which she was unaware. A search for the cause of the profound hypokalemia led to the finding of obligatory renal potassium wasting due to distal renal tubular acidosis (RTA), secretory type. The acid base status of the patient showed hyperchloremic metabolic acidosis compatible with distal renal tubular acidosis. Our case is a further proof that patients with "overlap syndrome" (SLE-SS) may have RTA, which may lead to life-threatening hypokalemia. Others have shown that the SLE-SS complex may occasionally be complicated by hyperkalemia. Thus, an autoimmune disease that is relatively common may present with devastating electrolyte abnormalities.
8438844 Esophageal motor function in primary Sjögren's syndrome. 1993 Mar To evaluate esophageal motor function in patients with primary Sjögren's syndrome (PSS) and its relation to the presence of dysphagia, 20 outpatients, who met the four criteria for PSS proposed by Fox et al. were prospectively studied by esophageal manometry after dry or wet swallows. Dysphagia was present in 15 (75%) patients, although the presence or severity of the symptom was not related to any specific motility pattern. Moreover, this study did not show any correlation between dysphagia and degree of inflammatory infiltrate of the labial minor glands, the parotid flow rate, or the presence of autoantibodies. In conclusion, although dysphagia is a common complaint in patients with PSS, esophageal motor studies have failed to show any consistent pattern.
1586250 Nervous system disease, immunological features, and HLA phenotype in Sjögren's syndrome. 1992 Apr Twenty seven patients with primary or possible Sjögren's syndrome with neurological manifestations were compared immunologically with 21 patients with Sjögren's syndrome with an intact nervous system. Patients with Sjögren's syndrome were divided into seropositive and seronegative subgroups with respect to the occurrence of one or more autoantibodies (antinuclear antibodies, rheumatoid factor, antibodies to SS-B) in their serum samples. This study of 48 patients indicates that the spectrum of nervous system disease in seronegative and seropositive subgroups is almost indistinguishable. No significant differences were found in the occurrence of circulating immune complexes, the levels of serum complement C3 and C4, or serum IgA, IgM, and beta 2 microglobulin with respect to the neurological manifestations. The serum IgG level, however, was significantly higher in the patients with Sjögren's syndrome with intact nervous systems than in those with neurological manifestations. No significant association was found between the HLA phenotype and nervous system disease. The occurrence of HLA-B8 and DR3 antigens was, however, significantly higher in those patients with antibodies to SS-B than in those without. This finding supports the suggestion that HLA-B8/DR3 may modulate autoantibody responses rather than disease expression in Sjögren's syndrome.
9452640 Whole saliva and the diagnosis of Sjögren's syndrome: an evaluation of patients who compl 1996 Jul Sjögren's syndrome (SS) is a common autoimmune disorder characterised by generalised desiccation, exocrine hypofunction and serologic abnormalities, More than 90% of the patients are women. OBJECTIVE: To determine if whole saliva could be used to diagnose this disease. SETTING: The study was conducted at the School of Dental Medicine, SUNY, at Stony Brook. PATIENTS: There were 49 subjects (48 F; 1 M), the mean age was 54 +/- 13 years. In order to be admitted into the study, they had to complain of dry mouth and dry eyes. TESTS: Whole saliva was collected by the spitting method. "Screening Tests" were employed to measure the salivary flow rate, pH, buffer capacity; lactobacillus and yeast concentrations. Chemical tests were performed to determine protein, albumin, sodium and amylase activity. Lacrimal dryness was assessed by the Schirmer and Rose-Bengal methods. RESULTS: Based on the sialometric findings, the patients were divided into 3 groups: Group 1: those with abnormally low resting (RFR) and stimulated (SFR) flow rates; Group 2: those with a low RFR but normal SFR; and Group 3: those with normal salivary flow rates. The group 1 patients were unique: their saliva demonstrated a low pH and buffer capacity, high lactobacillus and yeast concentrations, decreased protein output and amylase activity, and elevated albumin and sodium. Moreover, virtually all of them had abnormally low lacrimal flow rates. CONCLUSIONS: The findings suggested that whole saliva could be used to provisionally diagnose SS. Critical to this diagnosis was an abnormally low stimulated whole saliva flow rate. Other requisites included a low resting flow rate, the presence of dry mouth and dry eyes and evidence of lacrimal hypofunction. All of these attributes can easily be obtained by dentists in their clinics.
1603205 Labial salivary gland biopsy in Sjögren's syndrome. 1992 Apr Labial salivary gland histopathology has long been considered to be the most disease-specific single test for diagnosis of Sjögren's syndrome. However, the diagnostic yield of widely used grading systems of focal lymphocytic sialoadenitis is rather low. Determination of the percentages IgA-, IgG-, IgM-, kappa-, and lambda-containing plasma cells in minor salivary glands obtained from a lower lip biopsy has recently been shown to be extremely valuable in Sjögren's syndrome, not only for diagnosis, but also for prognosis with regard to the development of systemic monoclonal lymphoproliferative disease. This technique greatly improves the value of labial salivary gland biopsy in Sjögren's syndrome.
8700353 [Unstable bladder and Sjögren syndrome. Clinical case]. 1996 May A female patient developed severe detrusor instability years after a diagnosis of Sjogren syndrome. A common etiology could be possible if an association with viral demyelinization in Sjogren patients is confirmed. Vaginal Sicca syndrome was present and responded to a topical androgen-oestriol association with definite cytologic improvement. Anticholinergics were tolerated and moderately effective. Low initial dose were slowly increased (over three months) to avoid excessive mouth dryness and avoid gastrooesophageal symptoms.
1308274 Extrapulmonary location of sarcoidosis. 1992 Jan Two cases of sarcoidosis located outside the lungs are reported. In both cases the symptoms of the sicca syndrome were in the foreground. In the first case other clinical manifestations included mediastinal and retroperitoneal lymphadenopathy, and lacrimal gland impairment. In the other case the symptoms of the sicca syndrome were associated with polyneuropathy and maculopapular skin rash. The diagnosis of sarcoidosis was confirmed by histological examination of a biopsy specimen of lower lip mucosa in the first case, and biopsy of skin lesion in the second case. Abnormal results of laboratory tests included only raised erythrocyte sedimentation rate, in both cases and presence of circulating immune complexes in one case. Serological tests failed to demonstrate the presence of rheumatoid factor, antinuclear and anti-ds DNA antibodies. No decrease was observed also in the haemolytic activity of the complement, and cryoglobulins were absent. Regression of clinical signs was obtained in both cases after treatment with glucocorticosteroids.
8835252 Still's disease in a patient infected with human immunodeficiency virus type I. 1995 Nov An autoreactive cellular immune response may be a part of HIV-1 infection. This autoimmune phenomenon also includes antibodies to collagen that can be associated to arthritis as a clinical condition. In this paper we describe a case of Still's disease in an adult HIV-1 infected patient who presented a marked amelioration in his condition after 15 days of steroid therapy, as well as an improvement of the arthritis. This rare autoimmune condition, which is connected with the disregulation of the immune system, must be taken into account in HIV-1 infected individuals who present with fever of unknown origin.
8531360 [Immunological study of tissue-infiltrating lymphocytes in Sjögren's syndrome: virus spec 1995 Oct The possible role of Epstein-Barr virus (EBV) in the pathogenesis of Sjögren's syndrome (SS) has been suggested. However, the evidence has been circumstantial and it is not known at all whether cellular immune responses to EBV are linked to the mechanism of the disease. Although the oligoclonal expansion of T lymphocytes in salivary glands of SS was indicated by some analyses of the T cell receptors, the corresponding ligands are unknown. The key question may be of whether this disorder involves virus specific immune attacks on the salivary glands being a site for EBV latency. We introduce here our functional study of the salivary gland-infiltrating T lymphocytes in patients with SS for detecting EBV specific memory T cells. The potential association of the state of EBV-specific CTL in the lesion with the reactivation of EBV will be discussed.
8574618 Osteomalacia as a presenting manifestation of Sjögren's syndrome. 1995 Jul Osteomalacia is still common in Morocco, where the leading causes are nutritional deficiencies followed by intestinal diseases. Osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to a connective tissue disease such as Sjögren's syndrome. The case of a 40-year-old woman who presented with a five-year history of generalized bone pain, severe weight loss and a waddling gait is reported. She had low levels of serum phosphate (0.74 mmol/L), serum calcium (1.97 mmol/L), and urinary calcium (1.22 mmol/24 h). Serum alkaline phosphatase was 210 IU/L. Roentgenograms showed Looser's zones (right femoral neck, sixth and seventh right ribs). There was bilateral parotid gland enlargement, dryness of the mouth, nose and eyes, and bilateral punctate keratitis. A lip biopsy showed changes corresponding to stage II of the Chisholm and Mason classification. Tests for rheumatoid factor (latex and Waaler-Rose) and antinuclear factor were negative. The alkaline reserve was 18 mmol/L, serum potassium was 3.5 mmol/L, serum chloride was 112 mmol/L and urinary pH was 6.5. A renal biopsy showed tubulointerstitial lesions, lymphoplasmocytic infiltrates and interstitial sclerosis with patchy tubular atrophy. The patient was given bicarbonates, high-dose vitamin D followed by 1-alpha-hydroxycholecalciferol (0.3 microgram/d), and calcium (1 g/d). Follow-up was 42 months at the time of this writing. The role of tubular disorders in the genesis of osteomalacia is discussed, and the renal manifestations of Sjögren's syndrome are reviewed.
7825407 Ocular surface damage and tear lactoferrin in dry eye syndrome. 1994 Aug We studied the relationship between the severity of ocular surface damage and the level of tear lactoferrin in primary and secondary Sjögren's syndrome and keratoconjunctivitis sicca not associated with Sjögren's syndrome. A significant negative correlation was found between Rose Bengal staining score and level of tear lactoferrin in all three groups. Analysis of covariance disclosed no significant differences in regression lines for Rose Bengal staining score vs tear lactoferrin level among the three groups. The three regression lines appeared to be identical to each other. These findings indicate that the severity of ocular surface damage due to dry eye largely depends on the tear secretory function of the lacrimal gland, and that the function of the lacrimal gland can be evaluated by determination of level of tear lactoferrin using the same standards regardless of differences in pathogenesis of underlying diseases.