Antineutrophil Cytoplasmic Antibody in Patients with Primary Sjo¨gren’s Syndrome

The incidence, specificity and clinical significance of positivity for serum antineutrophil cytoplasmic antibody (ANCA) was investigated in 60 patients with primary Sj?gren's syndrome (SjS). The indirect immunofluorescence (IIF) technique and an enzyme-linked immunosorbent assay (ELISA) were used to measure ANCA. Purified myeloperoxidase (MPO), lactoferrin (LF), cathepsin-G (CTG) and elastase (HLE) served as ANCA antigens for the ELISA. Ten (16.7%) of the 60 SjS patients showed positivity by IIF for perinuclear, but not cytoplasmic, ANCA. Four of the 60 sera were shown to be positive for LF, four for MPO, 0 for CTG and 0 for HLE by ELISA. There was no correlation between ANCA positivity and clinical features. ANCA in patients with SjS might be an epiphenomenon of polyclonal B-cell activation.     

Ultrasonography and Colour Doppler Sonography of Salivary Glands in Primary Sjo¨gren’s Syndrome

To examine either the ultrasonographic (US) features of the parotids and submandibular glands or the blood flow alterations that may occur in the salivary glands of patients with primary Sjo¨gren’s syndrome (pSS) we studied 30 female patients with pSS and 30 controls suffering from dry mouth not due to pSS. All measurements were taken by the same examiner, who used the same equipment to avoid interobserver variability. The US parameters recorded (parenchymal homogeneity, echogenicity, size of the glands and posterior glandular border) were scored according to a previously described scoring system. For each waveform, peak systolic velocity (PSV) and resistive index (RI) were measured at the external carotid artery in the examination of the parotids and at the facial artery within the submandibular glands before and during lemon juice stimulation. On the basis of the degree of chronic inflammatory changes at minor salivary gland (MSG) biopsy, chronic sialadenitis (CS) was defined as mild in 10 and severe in 20 pSS patients. Abnormal US scores were obtained in 26/30 (86.6%) pSS patients and in 9/30 (30%) controls. Moreover, in pSS patients the US scores were sigificantly higher than in the control group (p=0.0001). The mean (± SD) difference between the PSV values taken from parotids and submandibular glands before and during lemon juice stimulation was statistically significant (p=0.003 and p=0.01, respectively) in the controls. On the other hand, no significant changes in the PSV values were found in the whole group of pSS patients. However, the changes in PSV values before and during lemon juice stimulation were statistically significant in both parotids (p=0.019) and submandibular glands (p=0.012), and not significantly different from those in the controls in pSS patients with mild CS. The variability of RI taken from the salivary glands before and during lemon juice stimulation was not statistically significant in either pSS patients or controls. US abnormalities were detected in the majority of pSS patients and their severity was significantly greater than those recorded in the controls. Of the colour Doppler sonographic (CDS) parameters only PSV was influenced by the degree of chronic inflammation, as shown at the MSG biopsy, suggesting that PSV may reflect the vascular changes occurring in the salivary glands during the course of an autoimmune disease such as pSS. Received: 21 June 2000 / Accepted: 15 December 2000     

Hypothalamus-Hypophysis-Thyroid Axis, Triidothyronine and Antithyroid Antibodies in Patients with Primary and Secondary Sjo¨gren’s Syndrome

It has been well established that, anti-thyroglobulin antibodies (ATG) and anti-microsomal antibodies (AMC) may be present in various thyroid disorders and other systemic autoimmune diseases, including Sjo¨gren’s syndrome (SS). However, presence of circulating autoantibodies to thyroid hormones, i.e. both to triiodothyronine (T3) and tetraiodothyronine (T4), has not been studied extensively in SS. Autoantibodies to T3 and T4 are very important, because serum T3 and T4 levels may be detected spuriously higher or lower, due to the presence of these autoantibodies. Their presence should be suspected when measured serum thyroid hormone levels are not consistent with clinical status of the patient. SS is a slowly progressive, inflammatory autoimmune disease, affecting primarily the exocrine glands. Thyroid gland, being a target in some autoimmune diseases, is well known to be affected in SS as well. Keeping this possibility in mind, we investigated T3 autoantibody levels and thyroid gland involvement in patients with SS.  Twenty-six SS patients (F/M:22/4) with a mean age of 46,6 years, were recruited in this study.Twelve of them were accepted as primary SS (pSS), while others had secondary SS (sSS) (7 with rheumatoid arthritis (RA), 3 with systemic lupus erythematosus (SLE), 3 with progressive systemic sclerosis (PSS) and 1 with sarcoidosis). Thyroid function tests, including T3, T4, fT3, fT4, TSH, ATG, AMC, T3 antibody measurements, thyroid scintigraphy, thyroid ultrasonography and TRH stimulation tests were performed in all patients. We compared our results with those of the twenty healthy normal controls.  Serum ATG and/or AMC were detected in three patients with pSS (25%) and no patients with sSS. No significant difference could be shown in the other parameters, including T3 autoantibodies and thyroid function tests. TRH stimulation test was also normal, showing that the hypothalamus-hypophysis-thyroid axis was not affected in patients both with pSS and sSS.  In conclusion, we found that T3 autoantibody levels in pSS, were not significantly higher than sSS and normal controls. Received: 18 June 2000 / Accepted: 7 July 2000     

Neurological Involvement in Primary Sjo¨gren’s Syndrome: Clinical and Instrumental Evaluation in a Cohort of Italian Patients

To evaluate nervous system involvement in a cohort of Italian patients with primary Sj?gren's syndrome (pSS), 87 unselected patients (83 female, and four male) observed consecutively at our institution over a period of 5 years were screened by clinical and instrumental (MRI, SPECT, electrophysiological testing, CSF analysis) investigations for peripheral and central neurological abnormalities. Seroimmunological parameters and extraglandular features other than neurological manifestations were also evaluated. Seven patients had central nervous system (CNS) disease (8%), mostly non-focal dysfunction, and 12 had peripheral nervous system (PNS) disease (13.8%), mostly mild or severe sensory or sensory-motor polyneuropathies. One patient had concomitant CNS and PNS involvement. Compared with CNS disease, PNS involvement occurred in older patients (> 50 years), independent of the disease duration. Patients with and without neurological abnormalities did not differ for seroimmunological parameters (including antiphospholipid antibodies) or extraglandular manifestations. From a statistical point of view, the only relevant finding was the detection of a slight increase in serum IgA and IgM levels (p < 0.05) in patients with an intact nervous system. Neurological involvement in pSS, be it central or peripheral, is not a rare finding. A careful clinical neurological evaluation, combined with a multiplicity of instrumental investigations, is recommended in the global assessment of pSS patients.     

Remission of the Renal Involvement in a Patient with Primary Sjo¨gren’s Syndrome (SS) after Pulse High-Dose Corticosteroid Infusion Therapy

We report the case of a young female patient with primary Sjo¨gren’s syndrome (SS). In addition to sicca symptoms she also suffered from progressive renal insufficiency and renal tubular acidosis (RTA). She was treated with three sets of pulse high-dose corticosteroid infusion and subsequent low-dose corticosteroid oral administration. When the efficacy was evaluated about 6 months after the start of the therapy, dramatic improvements were seen with no adverse effects, not only in laboratory tests but also histopathologically, as indicated by the repeat kidney biopsy. This suggests that renal involvements of SS might be reversible in some cases, and that there might be a clinical benefit of pulse high-dose corticosteroid infusion therapy in SS with progressive renal involvement. Received: 2 June 2000 / Accepted: 15 December 2000     

Achilles Tendinitis as the Presentation Form of Löfgren’s Syndrome

L?fgren's syndrome is characterised by bilateral hilar adenopathy arthritis and erythema nodosum. Achilles tendinitis as the presentation form of L?fgren's syndrome is very unusual. Herein we present a case of bilateral achilles tendinitis as the presentation form of L?fgren's syndrome.     

Pulmonary Manifestations of Sjögren’s Syndrome

Sjögren’s syndrome (SS) is primarily defined by its impact on the oral and ocular system resulting in xerostomia and xerophthalmia. However, SS can also manifest throughout the respiratory system. Subclinical pulmonary involvement is common. Clinically significant involvement can result in a 4-fold increased risk of death. Thus, recognizing the many potential presentations of SS in the lung is critical in caring for patients with SS. Additionally, SS should be included in the differential diagnosis of a number of forms of interstitial lung disease.     

Elevated Nitric Oxide Production in Patients with Primary Sjögren’s Syndrome

Nitric oxide (NO) production is elevated in patients with inflammatory disorders. We have previously shown increased NO production in patients with rheumatoid arthritis and systemic lupus erythematosus. In this study we used nitrite and citrulline levels as surrogate markers of NO production in patients with primary Sj?gren's syndrome (SS) and measured their levels by spectrophotometry. Fifteen patients and 15 age- and sex-matched controls were studied. Mean nitrite levels in patients were 582.3+/-208.3 nmol/ml, but those in controls were significantly lower, at 203.2-106.9 nmol/ml (p<0.001). Citrulline levels were 2820.4+/-933.9 nmol/ml in patients and were significantly higher than 217.4+/-144.8 nmol/ml, the levels in controls (p<0.0001). Mean levels of both nitrite and citrulline were significantly higher in patients with arthritis than in those who had no joint manifestations (p<0.05). There was no correlation between NO production and other variables, such as age, disease duration, drug therapy and antinuclear antibodies or rheumatoid factor positivity. Increased NO production may be partly a reflection of the presence of arthritis in five patients. It is concluded that there is increased NO production in patients with primary SS, especially if they have associated arthritis.     

Vasculitis in Sjögren’s Syndrome

Sjögren’s syndrome is a chronic autoimmune disease that is commonly manifested by immune attack on the exocrine glands with resultant dry eyes and dry mouth. Sjögren’s syndrome patients also have disease in other organs. One of the most common extraglandular manifestations is vasculitis. Skin vasculitis, with palpable purpura clinically and leukocytoclastic vasculitis on pathological examination, is common. Although half of those individuals with subcutaneous vasculitis have only a single episode, skin vasculitic involvement is associated with more severe disease. Necrotizing vasculitis of medium-sized vessels resembling polyarteritis nodosa can occur in Sjögren’s syndrome patients. Experience in therapy for vasculitis is limited, but intravenous IgG may be effective. Recent data support a relationship between neuromyelitis optica (Devic disease) and Sjögren’s syndrome. Sjögren’s syndrome patients with optic neuritis or transverse myelitis have anti–aquaporin-4, which are characteristic of Devic disease. Devic disease patients have salivary lymphocytic infiltration similar to that found among Sjögren’s syndrome patients.     

Primary rheumatic diseases of secondary Sjögren’s syndrome

Abstract

The study aims to find out the distribution of the primary rheumatic diseases of secondary Sjögren’s syndrome (SS) in an unselected in-patient population. Patients with primary and secondary SS were collected from the Finnish hospitals’ National Discharge Registry during 1970–1991. In total, 676 cases with primary SS and 709 cases with secondary SS were found. Several rheumatic diseases may be associated with SS, but their figures vary a lot. Rheumatoid arthritis (RA) was the most common primary diagnosis in cases of secondary SS, but systemic connective tissue diseases were as frequent as primary diseases when the figures were compared with the incidences of the diseases. Ankylosing spondylitis (AS), juvenile RA (JRA) and psoriatic arthritis (PA) were less frequently associated with SS. There are major differences between the association figures of SS to other rheumatic diseases. The dysfunction of exocrine cells is probably part of the clinical picture of RA and some systemic connective tissue diseases. but not typically of AS, JRA or PA. Thus the term secondary SS may describe the clinical manifestation of the primary disease and no true combination of two diseases exists.     

Sjögren’s Syndrome — A House of Cards

Clinical Rheumatology - Sjögren’s syndrome is an autoimmune rheumatic disease that is the result of the interplay between a number of environmental and genetic factors. In this short...     

Primary rheumatic diseases of secondary Sjögren’s syndrome

The study aims to find out the distribution of the primary rheumatic diseases of secondary Sjögren’s syndrome (SS) in an unselected in-patient population. Patients with primary and secondary SS were collected from the Finnish hospitals’ National Discharge Registry during 1970–1991. In total, 676 cases with primary SS and 709 cases with secondary SS were found. Several rheumatic diseases may be associated with SS, but their figures vary a lot. Rheumatoid arthritis (RA) was the most common primary diagnosis in cases of secondary SS, but systemic connective tissue diseases were as frequent as primary diseases when the figures were compared with the incidences of the diseases. Ankylosing spondylitis (AS), juvenile RA (JRA) and psoriatic arthritis (PA) were less frequently associated with SS. There are major differences between the association figures of SS to other rheumatic diseases. The dysfunction of exocrine cells is probably part of the clinical picture of RA and some systemic connective tissue diseases. but not typically of AS, JRA or PA. Thus the term secondary SS may describe the clinical manifestation of the primary disease and no true combination of two diseases exists.     

Diagnosis of Liver Involvement in Primary Sj?gren Syndrome

Liver involvement was one of the first extraglandular manifestations to be reported in patients with primary Sjögren syndrome (SS). In the 1990s, a study of liver involvement in patients with primary SS integrated the evaluation of clinical signs of liver disease, liver function and a complete panel of autoantibodies. Recent developments in the field of hepatic and viral diseases have significantly changed the diagnostic approach to liver involvement in SS. The most recent studies have shown that, after eliminating hepatotoxic drugs and fatty liver disease, the two main causes of liver disease in primary SS are chronic viral infections and autoimmune liver diseases. The differential diagnosis of liver disease in primary SS (viral vs autoimmune) is clinically important, since the two processes require different therapeutic approaches and have different prognoses. With respect to viral infections, chronic HCV infection is the main cause of liver involvement in SS patients from the Mediterranean area, while chronic HBV infection may be the main cause of liver involvement in SS patients from Asian countries. After eliminating viral hepatitis, primary biliary cirrhosis (PBC) should be considered the main cause of liver disease in primary SS. PBC-related SS patients may have a broad spectrum of abnormalities of the liver, including having no clinical or analytical data suggestive of liver disease. Autoimmune hepatitis (AIH) is the second most frequently found autoimmune liver disease to be associated with SS (all reported cases are type I), and nearly 10% of these patients have an AIH-PBC overlap. Finally, IgG4-related disease must be investigated in patients with SS presenting with sclerosing cholangitis, especially when autoimmune pancreatitis or retroperitoneal fibrosis are also present.     

Classification of Disease Manifestations in Primary Sjögren’s Syndrome: Present Status and a New Proposal

People with chronic musculoskeletal conditions are high users of complementary and alternative medicines (CAM). This systematic review was conducted to evaluate the attitudes of rheumatologists towards CAM and to identify whether these attitudes are affected by the personal or practice characteristics of the rheumatologists. A systematic search of electronic databases identified five eligible studies and one supplementary abstract, published before 1 December 2012. Outcomes measuring rheumatologists’ attitudes towards CAM were extracted, as were any analysis of correlations with characteristics of the rheumatologist. Study quality was assessed using the STROBE checklist. Six studies from the USA, Canada and the Netherlands met inclusion criteria, with sample sizes ranging from 101 to 2,000. The studies were of variable methodological quality. Rheumatologists’ opinions towards CAM varied according to therapy type. Many held favourable opinions towards bodywork and meditation, believed in their benefits and provided referrals for use. Other therapies, such as energy-based medicine, were regarded with scepticism. There were no demographic characteristics that consistently correlated with CAM attitudes or use. The limited data describing rheumatologist’s attitudes to CAM is of varying quality but suggests that attitudes are influenced by the rheumatologist’s familiarity with the CAM therapy and the degree to which a therapy has been assessed in a scientific manner. Given the high use of CAM amongst individuals seen in rheumatology clinics, physicians should undertake high-quality research to assess effectiveness of CAM therapy.     

Diagnostic performance of Sjögren’s Syndrome Screening Questionnaire (SSSQ)

Clinical Rheumatology -