Elevation of serum soluble intercellular adhesion molecule-1 (sICAM-1) and beta-2-microglobulin in Sjögren's syndrome

Summary Sjögren's syndrome is a chronic inflammatory disease which is believed to be immunologically mediated. It is marked by inflammation and destruction of the salivary glands and lacrimal glands. We have found significantly higher serum levels of sICAM-1 and beta-2-microglobulin in both primary Sjögren's syndrome patients (429.1±114.33 ng/ml, 4.4±1.5 ug/ml resp.) and secondary Sjögren's syndrome patients (424±140.73 ng/ml, 4.4±2.67 ug/ml resp.) in comparison with healthy controls (189.1±42.34 ng/ml, 1.3±0.38 ug/ml resp.). The serum level of beta-2-microglobulin was significantly correlated with the serum level of sICAM-1 in secondary Sjögren's syndrome patients' group.     

Hepatitis C virus and Sjögren's syndrome

Summary Viral infection has been suggested as a possible cause of Sjögren's syndrome. After we had noted Sjögren's syndrome in several patients infected with hepatitis C virus (HCV), we set up a prospective study to investigate the association of Sjögren's syndrome and HCV liver disease. We studied 10 patients with primary Sjögren's syndrome and observed four with HCV infection. A striking association between HCV infection and Sjögren's syndrome was found; however, a direct link could not be proved.

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Hepatitis C Virus und Sjögren-Syndrom

Zusammenfassung Als mögliche Ursache für das Sjögren-Syndrom werden Virusinfektionen diskutiert. Wir haben bei mehreren Patienten mit Hepatitis C Virus (HCV)-Infektion ein Sjögren-Syndrom beobachtet und deshalb eine prospektive Studie begonnen, um die Verbindung zwischen Sjögren-Syndrom und HCV-Lebererkrankung zu erforschen. Wir untersuchten 10 Patienten mit primärem Sjögren-Syndrom und fanden vier Fälle mit HCV-Infektion. Es stellte sich eine bemerkenswerte Assoziation zwischen HCV-Infektion und Sjögren-Syndrom heraus, doch konnte eine direkte kausale Verknüpfung nicht bewiesen werden.

     

SJOGREN'S SYNDROME PRESENTING AS A SEVERE SENSORY NEUROPATHY INCLUDING INVOLVEMENT OF THE TRIGEMINAL NERVE

Primary Sjögren's syndrome (SS) can be associated and maypresent with neurological complications. We describe a 32-year-oldwoman with a debilitating sensory neuropathy, including trigeminalnerve involvement which was the initial manifestation of thisdisease. KEY WORDS: Sjögren's syndrome, Sensory neuropathy, Trigeminal neuropathy     

Les classifications anatomopathologiques du syndrome de Gougerot-Sjögren primitif sont-elles corrélées entre elles et à la clinique?

Are Sjögren's disease histological scales correlated together and to clinical criteria?Seventy-two cases of primary Sjögren's syndrome, according to the European classification criteria, were studied looking for a correlation between anatomic criteria and clinico-biological signs in this disease. Labial salivary gland biopsy was performed in all patients and anatomic criteria were evaluated according to both Chisholm and Chomette scales. Work-up included recording of functional and clinical signs. Our study shows no clinico-histological correlation in Sjögren's syndrome. Moreover, we did not find any correlation between the two histological scales. We think, therefore, that labial salivary gland biopsy appears to be an important step in Sjögren's syndrome diagnosis according to European criteria. However, it does not present any benefit in patient treatment.     

Leucocytoclastic vasculitis as presenting feature of primary Sjögren's syndrome

Summary Described are two patients whose primary Sjögren's syndrome presented as leucocytoclastic vasculitis of the skin. One patient initially admitted complaints of dryness of the eyes and the mouth after direct questioning, and serologic testing revealed the presence of Ro/SS-A and La/SS-B antibodies. In the other patient the presence of antinuclear antibodies and rheumatoid factors in serum were the only suggestions of the presence of primary Sjögren's syndrome. Primary Sjögren's syndrome should be considered in patients presenting with leucocytoclastic vasculitis.     

Esophageal manometric findings in autoimmune rheumatic diseases: is scleroderma esophagus a specific entity?

Summary In order to assess whether distal esophageal hypomotility in scleroderma is unique to this disease or not, we studied 25 normal volunteers and 109 patients with autoimmune rheumatic diseases (27 with primary Sjögren's syndrome, 25 with idiopathic Raynaud's phenomenon, 25 with rheumatoid arthritis, 19 with scleroderma, 5 with undifferentiated connective tissue disease, 3 with systemic lupus erythematosus, 2 with mixed connective tissue disease, 2 with sclerodermatomyositis, and one with morphea). Esophageal dysfunction typical of scleroderma was present in 17 patients (15.6%), of whom 13 had scleroderma (68%) and one each primary Sjögren's syndrome, rheumatoid arthritis, undifferentiated connective tissue disease, and mixed connective tissue disease. Twenty-two percent of all patients had nonspecific esophageal motility changes, clustered among primary Sjögren's syndrome, idiopathic Raynaud's phenomenon, and rheumatoid arthritis. We conclude that lower esophageal hypomotility, although most frequent in scleroderma, is not unique to this disease and can be encountered in several other autoimmune rheumatic diseases.     

Raised C-reactive protein response in rheumatoid arthritis patients with secondary Sjögren's syndrome

Summary The levels of serum C-reactive protein (CRP) were found to be significantly higher in the presence than in the absence of secondary Sjögren's syndrome in patients with rheumatoid arthritis, while the values of erythrocyte sedimentation rate and serum fibrinogen were not significantly different. The levels of CRP were found to be normal in 22 out of 24 patients with primary Sjögren's syndrome.     

Serum soluble Fas/APO-1 is increased in patients with primary Sjögren's syndrome

The aim of the study was to elucidate the involvement of Fas antigen in human autoimmune disease, by analysing serum levels of soluble Fas/APO-1 protein in patients with various autoimmune diseases, including system lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), Behçet's syndrome and Sjögren's syndrome (SjS). The levels of soluble Fas/APO-1 in sera were quantitated by a sandwich enzymelinked immunosorbent assay. Soluble Fas/APO-1 levels were significantly increased in serum from patients with primary Sjögren's syndrome (1° SjS) compared with control subjects. However, no significant differences in soluble Fas/APO-1 levels were noted in patients with secondary Sjögren's syndrome (2° SjS) nor in patients with any of the other autoimmune diseases. The soluble Fas/APO-1 level in 1° SjS patients with extraglandular diseases was significantly higher than that in patients without extraglandular diseases. These results suggest that soluble Fas/APO-1 protein may play an important role in the pathogenesis of 1° SS.     

Pulmonary function in patients with primary Sjögren's syndrome

Summary In 43 women and 3 men suffering from primary Sjögren's syndrome pulmonary function was correlated to various clinical, haematological and serological disease activity parameters. Primary Sjögren's syndrome was defined as the presence of keratoconjunctivitis sicca and xerostomia, in the absence of other well-defined chronic inflammatory connective tissue diseases. Only objective tests were used in the evaluation of the patients [2]. Total lung capacity, residual volume, vital capacity, functional residual capacity and ventilatory capacity were normal. The diffusion capacities measured for CO were significantly reduced compared with the predicted values. This indicates that pulmonary interstitial disease is a common feature of primary Sjögren's syndrome. There was a statistically significant negative correlation between reduced diffusion capacity and previous pneumonia, previous pleurisy, tiredness, dyspnoea, ESR and p-orosomucoid. No correlation was found to certain other clinical disease parameters, the duration of disease or tobacco smoking.     

THYROID DISEASE AND OTHER AUTOIMMUNE PHENOMENA IN A FAMILY STUDY OF PRIMARY SJOGREN'S SYNDROME

Autoimmune diseases and autoantibodies have been documentedin 42 index cases with definite primary Sjögren's syndrome(1° SS), 207 relatives and 39 spouses. The results werecompared with control data from a local population survey. Thyroiddisease, 1° SS and their associated autoantibodies werethe commonest autoimmune abnormalities observed and found predominantlyin older female relatives. The HLA-DR3 phenotype associatedwith 1° SS, antinuclear factor, hypothyroidism, and thyroidmicrosomal antibody. Rheumatoid arthritis and systemic lupuserythematosus were not found in excess in the families. PrimarySjögren's syndrome is frequently associated with thyroiddisease and we suggest that there is a common genetic predispositionbetween these diseases which differs from 2° SS associatedwith rheumatoid arthritis and systemic lupus erythematosus.This includes MHC and non-MHC genes. KEY WORDS: Thyroid disease, Genetics, HLA-DR3, Sjögren's syndrome     

Multicentric Castleman's disease in a patient with primary Sjögren's syndrome

Summary A 38-year-old woman suffering from primary Sjögren's syndrome for 2 years developed angiofollicular hyperplasia (multicentric Castelman's disease). In Sjögren's syndrome (SS) a number of findings indicate the presence of a B-cell hyperactivity that may evolve to a lymphoproliferative disorder. This report adds another pathological event to the complex spectrum of lymphoproliferative diseases in SS.Deccased     

The clinical significance of the detection of anti-Ro/SS-A and anti-La/SS-B autoantibodies using purified recombinant proteins in primary Sjögren's syndrome

Summary Recently, an enzyme-linked immunosorbent assay (ELISA), using purified recombinant non-fusion proteins, has been introduced to detect and quantify Ro/SS-A and La/SS-B autoantibodies. We compared this method with anti-Ro/SS-A and anti-La/SS-B detection by means of counterimmunoelectrophoresis and immunoblotting in patients with primary Sjögren's syndrome (SS), patients suspected of the syndrome and controls. The sensitivity and specificity of the newly developed ELISA for anti-Ro/SS-A were 53% and 100%, respectively, and for anti-La/SS-B, 40% and 98%, respectively. No significant difference was found between these results and those obtained from both other assays. Titres of Ro/SS-A and La/SS-B autoantibodies correlated with the presence of an abnormal parotid gland sialogram and hypergammaglobulinaemia. We concluded that the new ELISA did not enhance the diagnostic yield in cases of suspicion of primary Sjögren's syndrome. Longitudinal studies of large groups of patients with primary Sjögren's syndrome are necessary to demonstrate whether following the course of the titres of these autoantibodies would be of value for prediction of disease exacerbations.     

Sjögren's syndrome associated with autoimmune hepatitis. A case report

Summary Liver involvement in patients with primary Sjögren's syndrome is rare, usually without clinical significance and histologically characterized by a feature like stage 1 primary biliary cirrhosis. We describe herein a case of acute and severe autoimmune hepatitis in a patient suffering from primary Sjögren's syndrome. The diagnosis of Sjögren's syndrome was performed in 1989.In June 1995 the patient presented severe weakness, jaundice and elevation of transaminases; moreover IgG raised to 5560 mg/dl and ANA titre increased to 1:20480. The patient denied alcohol and drug use and a viral hepatitis was excluded. Antimitochondrial antibodies, anti-smooth muscle antibodies and antibodies against liver kidney microsomes were negative. An abdomen ultrasound examination revealed hepatomegaly, with irregular echogenic structure and lymphoadenomegaly near the celiac tripod. Liver biopsy demonstrated a picture of autoimmune hepatitis.The patient was treated with prednisone 50 mg/day and azathioprine 50 mg/day, with improvement in clinical and liver function indices. At present, the patient is given only 10 mg/day of prednisone.The association of Sjögren's syndrome with autoimmune hepatitis is very rare: in the literature only one other similar case has been reported.     

Syndrome de Gitelman acquis au cours d’un syndrome de Gougerot-Sjögren associé à une sclérodermie

Tubulopathy can complicate autoimmune diseases. It is usually a distal tubular acidosis, but Fanconi syndrome or Bartter syndrome has been exceptionally reported. We report a case of acquired Gitelman syndrome in a 32-year-old male who also presented diffuse scleroderma autoimmune thyroiditis, and Sjögren's syndrome. Only three cases of Sjögren syndrome associated with Gitelman syndrome have been previously reported in literature. The absence of other cases in the family and absence of mutation SLC12A3 emphasise the relation between autoimmune disease and this tubulopathy.     

OSTEOARTICULAR TUBERCULOSIS OF THE SYMPHYSIS PUBIS PRESENTING AS A HYPOGASTRIC CYSTIC MASS IN A WOMAN WITH PRIMARY SJOGREN'S SYNDROME

A 79-year-old woman with primary Sjögren's syndrome andimmune thrombocytopenia presented with a hypogastric cysticmass. Twenty-three months previously she received a 7-monthcourse of prednisone at moderate doses to control the thrombocytopenia.A computed tomography showed a cystic mass, destruction of theleft pubis with involvement of the symphysis and erosion ofthe right pubis. The cystic mass was surgically removed. Epithelioidgranulomata were found on pathological examination and Mycobacteriumtuberculosis was grown in Löwenstein medium. This is thefirst reported case of osteoarticular tuberculosis with sucha presentation and only the second localized to the pubis. KEY WORDS: Osteoarticular tuberculosis, Primary Sjögren's syndrome     

A case of sjögren's syndrome associated with sweet's syndrome

Summary We report a case of Sjögren's syndrome whose clinical course had been indolent until the patient presented with Sweet's syndrome (acute febrile neutrophilic dermatosis). This patient showed renal failure and renal tubular acidosis. Sweet's syndrome resolved within 3 weeks without corticosteroid therapy. Renal biopsy findings were consistent with interstitial nephritis. His renal manifestations responded to corticosteroid therapy and the renal function remained stable during 6 years follow-up without recurrence of Sweet's syndrome. Although close association of both syndromes is already known, in our case Sjögren's syndrome may have been exacerbated by occurrence of Sweet's syndrome.     

Renal tubular acidosis, arthritis and autoantibodies: primary Sjögren's syndrome in childhood

SIR, Primary Sjögren's syndrome (PSS) is rare in the paediatricpopulation [1, 2]. We describe an 8-yr-old female who presentedwith arthritis, distal renal tubular acidosis and an autoantibodyprofile suggestive of Sjögren's syndrome without siccasymptoms. An 8-yr-old female first presented in November 2004. Her symptomsstarted 9 months previously with swelling, pain and morningstiffness of her left ankle and knee, and pain in her wristsand hands. She was treated with ibuprofen and started physiotherapy.The pain improved but stiffness remained. In     

Agranulocytosis in a patient with primary Sjögren's syndrome

Summary The authors describe a case of primary Sjögren's syndrome, which was complicated with severe autoimmune agranulocytosis quite sensitive to immunosuppressive therapy. Agranulocytosis is a very rare complication of this autoimmune rheumatic disease as opposed to leucopenia. A remarkable feature of the presented case is the fact that correct diagnosis of primary Sjögren 's syndrome has not been settled for almost 25 years. The disease has manifestated only in the form of arthropathy imitating rheumatoid arthritis.     

THE MOLECULAR BASIS OF THE SSA/Ro ANTIGENS AND THE CLINICAL SIGNIFICANCE OF THEIR AUTOANTIBODIES

The SSA/Ro antigens are nuclear and cytoplasmic polypeptideswhich serve as autoantigens in systemic lupus erythemato-sus(SLE) and Sjögren's syndrome (SS). They contain two majorisoforms of 60 and 52 kD. The former is the native antigen whilethe latter is a major autoantigen in its denatured form. A thirdprotein of 46 kD termed ‘calreticulin-Ro’ is anautoantigen found in the sera of some patients with SLE. However,it is probably unrelated to the SSA/Ro system. The clinicalrelevance of anti SSA/Ro antibodies in rheumatic diseases hasalso been considered. Initially these antibodies were thoughtto be an epiphenomenon of autoimmune diseases. Recent studieshave shown that they are associated with specific clinical manifestationsand disease subsets. Furthermore, animal models have demonstratedthat they may enhance tissue damage. It seems that anti-SSA/Roantibodies may play a role in the pathogenicity of SLE and SS. KEY WORDS: SSA/Ro antigen, Autoantibodies, Systemic lupus erythematosus, Sjögren's syndrome     

Renal tubular acidosis in primary Sjögren's syndrome

Summary Renal tubular acidosis (RTA) is a frequent extraglandular manifestation of Sjögren's syndrome; however, no distinction on the incidence of this renal tubular defect between primary and secondary Sjögren's syndrome has been reported. This study was undertaken in order to define the frequency of RTA and the possible pathogenetic mechanisms in a group of 21 randomly selected primary Sjögren's syndrome patients. RTA was found in 7 (33%) patients. The incomplete type of the disorder was the most frequent. It seems that the etiology of RTA is multifactorial. Renal excretion of monoclonal proteins and the immunologically-induced interstitial inflammation are the main possible factors of this renal tubular defect.