Sicca syndrome in the same family - the importance of anti-Ro60, anti-Ro52 and anti-La antibody profiling in Sjögren's syndrome.

Sicca syndrome, which is typical for Sjögren's syndrome (SS), both primary (pSS) and secondary (sSS), is relatively often comorbid with other autoimmune diseases. The current classification criteria for SS published in 2016 include only anti-SSA (anti-Ro) autoantibody, while the latest literature proposes that anti-Ro60/anti-Ro52 autoantibody profiles should be used instead, as these two types of antibodies correlate with specific clinical symptoms and laboratory test findings. The paper presents the case of a 41-year-old woman suffering from pSS and her three daughters, who were under observation for rheumatic disorders due to sicca symptoms, especially pSS, as well as a discussion on separate determination of anti-Ro60 and anti-Ro52 autoantibodies based on current literature in the PubMed database. When testing with antinuclear antibodies, the Ro60+Ro52+La+ autoantibody profile most closely matches for pSS. Further research is needed to find marker antibodies for SS and quantification methods.

[Late-onset sjögren's syndrom].

Sjögren's syndrome is a systemic autoimmune disease characterized by exocrine glands damage, resulting in the development of dry eyes and dry mouth, as well as extraglandular manifestation. It usually starts between the ages of 30 and 50. Late-onset Sjögren's syndrome should be considered as a disease that appears after age 65, but the literature also reports of the age 50, 60, or even 70. The prevalence of late-onset Sjögren's syndrome is estimated about 20%. The course of late-onset Sjögren's syndrome may differ when compared with patients with a younger onset. Lack of sicca symptoms and marker antibodies may be associated with a delay in Sjögren's syndrome diagnosis. Particularly in the elderly, the occurrence of sicca symptoms may be considered as age-related and medication-related.

Influence of antiphospholipid antibody positivity on glomerular filtration rate markers in a group of systemic sclerosis patients - a 24-month observation.

AIM OF THE STUDY: The aim of the study was the assessment of changes in the glomerular filtration rate (GFR) during long-term observation in a group of systemic sclerosis (SSc) patients with and without chronic antiphospholipid (aPL) antibody positivity. MATERIAL AND METHODS: The observation comprised 50 patients - 23 with diffuse cutaneous SSc - dcSSc and 27 limited cutaneous SSc - lcSSc. After 24 months we assessed 27 patients (9 died, 14 lost follow up); 24 patients (88%) were treated chronically with angiotensin-converting-enzyme inhibitors (ACEIs). Patients were investigated for the presence of aPL: to cardiolipin and to ß2 glycoprotein I in IgM and IgG classes. Serum levels of creatinine (S-Cr), cystatin C and creatinine clearance values were determined in all patients. According to the presence of a significant level of at least one of aPL antibodies, pts were divided into groups: group I aPL positive: 14 patients, group II aPL negative - 13 patients. RESULTS: We did not find significant differences in S-Cr, cystatin C levels and creatinine clearance before and after 24 months of observation between both groups. In follow up observations, the presence of anti-centromere antibodies was significantly more frequent in the aPL positive, as compared to the aPL negative group (p = 0.01). In follow up observations, the level of anticardiolipin antibodies in IgG class was significantly higher in dcSSc compared to lcSSc patients (p = 0.02). CONCLUSIONS: In long-term observation chronic positivity for aPL antibodies does not significantly decrease the GFR in patients with SSc treated with ACEIs.

Autoantibodies against gliadin in rheumatoid arthritis and primary Sjögren's syndrome patients.

INTRODUCTION: Patients with rheumatoid arthritis (RA) and those with primary Sjögren's syndrome (pSS), beside non-specific antibodies, present with organ-specific autoantibodies, including those typical of celiac disease (CD). In the pathogenesis of CD, a role is played by anti-tissue transglutaminase, anti-endomysium, and anti-gliadin (AGA) antibodies. CD can be comorbid with RA and pSS. The aim of the study was to evaluate the prevalence of AGA in RA and pSS patients and discussion of their clinical significance. MATERIAL AND METHODS: The study included 121 patients with RA and 30 patients with pSS. IgG AGA were determined by ELISA method. Additionally, the presence of IgG antimitochondrial antibodies (AMA) was determined in all patients. A further observation included patients with AGA. RESULTS: In the RA group, AGA were detected in five patients (4.1%), and in the pSS group - in two patients (6.7%). All patients in the pSS AGA (+) had AMA and autoimmune thyroid disease (AITD). These differences were statistically significant compared to the pSS AGA (-) and AMA (+) group (p=0.002) and the AGA (-) and AITD (+) group (p=0.003). At the time of the study, none of the patients had been diagnosed with CD. CONCLUSIONS: AGA, typical of CD, are significantly more frequently detected in patients with RA and pSS than in the general population. The presence of autoantibodies may have an impact on the clinical picture of the disease and further medical procedures. CD testing is warranted in selected patients.

[Evaluation of usefulness of Polycheck method in the detection autoantibodies in patients with systemic lupus erythematosus, Sjögren's syndrome and systemic sclerosis]. / Ocena przydatnosci metody Polycheck w wykrywaniu autoprzeciwcial u chorych na toczen rumieniowaty ukladowy, zespól Sjögrena i twardzine ukladowa.

INTRODUCTION: To recognize the connective tissue diseases (CTD), which include lupus erythematosus (SLE), Sjögren's syndrome (SS) and Systemic sclerosis (SSc) it is necessary to determine the presence of autoantibodies (Ab). There is still work going on to find effective and equivocal detection methods. THE AIM OF STUDY: To evaluate the usefulness, clinical value and innovativeness of the Polycheck method in the detection of autoantibodies in patients with connective tissue diseases. MATERIALS AND METHODS: The study involved 178 people: 153 patients of the Department of Rheumatology and 25 healthy people. According to the main diagnosis the patients were divided into 3 groups: SLE-59, ZS-45, SSc-49. The presence and concentrations of Ab were determined by using multiparametric enzyme-linked immunosorbent assay Polycheck Rheuma (Biocheck, GmbH, Münster, Germany). Statistical data analysis was performed using Statistica v10.0. RESULTS: In our study we found thatthe frequency of antibodies characteristic of the SS: anti-SS-A/Ro 52, anti-SS-A Ro 60 and anti-SS-B/La was significantly higher in patients with SS group compared to TRU, TU, and GK (p <0.05); concentrations of Ab were lower in TRU and in SSc group. Marker Ab for SLE anti-dsDNA were only present in patients with SLE. Anti-Sm antibodies were more common in this patient population too. Antibodies associated with diagnosis of SSc anti-SCL-70 and anti-CENP B were significantly more often observed in patients with SSc in higher concentrations compared to the other groups examined (anti-SCL-70 p<0,0005). The Ab concentration analysis performed with use of this analysis help us to confirm diagnosis in particular patients. CONCLUSIONS: The multiparametric enzyme-linked immunosorbent assay Polycheck Rheuma is helpful in making a quick and comprehensive identification of autoantibodies in pts with suspected CTD. The concentration analysis performed with use of this method enables an accurate diagnosis despite the ambiguous clinical picture.

[Biological treatment in severe Still's disease--a case report]. / Leczenie biologiczne w ciqikiej postaci choroby Stilla--opis przypadku.

Still's disease and systemic juvenile idiopathic arthritis (JIA) are multisystem inflammatory diseases of unknown etiology, different disease course and prognosis. Still's disease is characterized by hectic fever, arthritis, skin rash, organomegaly, elevated serum ferritin and inflammatory factors. Early diagnosis and intensive treatment can prevent disease progression and reduce complications such as amyloidosis, physical disability. The first choice of treatment are high doses of corticosteroids and synthetic disease-modifying drugs (DMARDs), including methotrexate (MTX), cyclosporine (CsA). Biologic agents are second line therapy when DMARDs aren't effective, e.g. monoclonal antibodies blocking the action of TNF-alpha (anti-TNF-α), interleukin-1 (ANK--anakinra) and interleukin-6 (TCZ--tocilizumab). We describe in details treatment strategies applied in a young woman with severe Still's disease treated with combination therapy of DMARDs and anti-TNF-α, including etanercept (ETA) or certolizumab (CER). TCZ was applied for the treatment of Still's disease following treatment failure with anti-TNF-α. We've achieved a complete remission of the Still's disease during treatment TCZ.

Prevalence of selected organ-specific autoantibodies in rheumatoid arthritis and primary Sjögren's syndrome patients.

OBJECTIVES: The aim of the study was to investigate the prevalence of selected organ-specific autoantibodies in rheumatoid arthritis (RA) and primary Sjögren's syndrome (pSS) patients, and discuss their clinical significance. MATERIAL AND METHODS: The study included 121 RA and 30 pSS patients. Sera were tested for the presence of autoantibodies to thyroid peroxidase (anti-TPO), thyroglobulin (anti-TG), TSH receptor (TRAbs), mitochondrial antigen M2 (AMA-M2-3E) and gliadin-analogous fusion peptides (anti-GAF(3X)) using the ELISA method. Non-organ-specific antibodies were determined: rheumatoid factor in IgM class, anti-citrullinated peptide antibodies and antinuclear antibodies. The occurrence of antibodies was also examined with regards to RA activity. RESULTS: The following autoantibodies were detected in RA patients: anti-TPO - 13 (10.7%), anti-TG - 6 (5%), AMA-M2-3E - 3 (2.5%), anti-GAF(3X) - 5 (4.1%). The respective levels of these autoantibodies in pSS patients were 3 (10%), 2 (6.7%), 4 (13.3%) and 2 (6.7%). Polyautoimmunity was confirmed in 34 RA patients (including 20 cases of autoimmune thyroid disease [AITD]) and in 6 pSS patients (6 cases of AITD). When RA patients were divided into anti-TPO positive and anti-TPO negative groups, we found a statistically significant relationship between groups regarding age and hemoglobin concentration. In pSS patients the anti-TPO positive group was less likely to use immunosuppressive drugs as compared with the anti-TPO negative group. Anti-TPO was significantly more frequently detected in RA + AITD vs. RA, RA + SS + AITD vs. RA and in pSS + AITD vs. pSS patients. CONCLUSIONS: Organ-specific autoantibodies are relatively frequently observed in patients with RA and pSS. Their presence is connected with the clinical picture of the diseases.

Presence of organ­specific antibodies in patients with systemic sclerosis.

INTRODUCTION According to the literature, organ­specific antibodies may be present in the course of systemic sclerosis (SSc). OBJECTIVES The aim of this study was to assess the prevalence of antithyroid antibodies (antithyroid peroxidase antibodies [anti­TPO] and antithyroglobulin antibodies) and of antimitochondrial antibodies (AMAs), as well as to evaluate their clinical significance in patients with SSc. PATIENTS AND METHODS The study involved 86 consecutive in­hospital patients with SSc (32 patients with diffuse cutaneous SSc [dcSSc] and 54 with limited cutaneous SSc [lcSSc]). Patients were observed for autoimmune thyroid diseases (ATDs) and primary biliary cirrhosis (PBC). Serum samples were obtained from each patient. RESULTS Positive antithyroid antibody titers were observed in 27 patients (31%) and positive AMA titers-in 11 patients (13%). ATD was diagnosed in 26 patients (30%) and PBC-in 10 patients (12%) with SSc. No significant differences in the prevalence of antithyroid antibodies were found between patients with dcSSc and those with lcSSc, but the prevalence of AMAs was significantly higher in patients with lcSSc compared with those with dcSSc. The prevalence of anti­Ro­52 antibodies was significantly higher in the SSc group with positive anti­TPO antibody titers compared with the SSc group with negative anti­TPO antibody titers. The prevalence of anticentromere antibodies (ACAs) was significantly higher in the SSc group with positive AMA titers compared with the SSc group with negative AMA titers. CONCLUSIONS The prevalence of organ­specific antibodies in SSc patients is relatively high. The prevalence of AMAs is higher in patients with lcSSc than in those with dcSSc and is strongly associated with the presence of ACAs. Patients with SSc should be evaluated for coexisting ATDs and PBC.

The occurrence of antibodies against Legionella pneumophila in patients with autoimmune rheumatic diseases.

INTRODUCTION: Patients with autoimmune rheumatic diseases are more susceptible to infection, owing to the underlying disease itself or to its treatment. Most commonly, infections affect the respiratory and urinary tracts. One of the etiological factors of infections in these patients is the bacteria of the genus Legionella. OBJECTIVES: The aim of the study was to assess the prevalence of anti-Legionella pneumophila (L. pneumophila) antibodies in patients with autoimmune rheumatic diseases and to analyze individual and environmental risk factors for the development of Legionella infection in patients with positive antibody results. PATIENTS AND METHODS: The study group consisted of 165 patients with autoimmune rheumatic diseases and 100 healthy subjects. Serum samples were tested for the presence of specific antibodies in the immunoglobulin (Ig) M and IgG classes against L. pneumophila serogroups 1 to 7 (SG 1-7) and the IgG class for serogroup 1 (SG 1). RESULTS: Antibodies against L. pneumophila were found in 7 patients (4%): 5 cases with antibody positivity only in the IgG class and 2 cases with antibody positivity in both classes. In patients with positive IgG antibodies for SG 1-7, specific antibodies for L. pneumophila SG 1 were not detected. In the control group, positive results were obtained in 9 cases (9%): IgM positivity in 6 (6%) and IgG positivity in 3 (3%). CONCLUSIONS: The frequency of antibodies to L. pneumophila in our patients is comparable to that in healthy individuals. L. pneumophila should be recognized as a potential pathogen in patients with autoimmune rheumatic diseases. Primary disease condition, immunosuppressive therapy, and other risk factors should not be ignored in these patients.

Relationship between rheumatoid arthritis activity and antithyroid antibodies.

INTRODUCTION: Rheumatoid arthritis (RA) is known to be associated with a higher prevalence of antithyroid antibodies and autoimmune thyroid disease, but there have been few studies regarding the correlations between the presence of these antibodies and RA activity. OBJECTIVES: The aim of this study was to analyze the relationship between antithyroid antibody titers and selected parameters of RA activity. PATIENTS AND METHODS: A total of 75 consecutive hospitalized patients with RA were enrolled into the study. Levels of antithyroid peroxidase antibodies (aTPO), antithyroglobulin antibodies (aTG), and antithyrotropin receptor antibodies (aTSH-R) were measured. The analysis of disease activity was based on the disease activity score with 28-joint count (DAS28), duration of morning stiffness, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) and hemoglobin levels. RESULTS: Antithyroid antibodies were present in 13.3% of the patients (n = 10), aTPO in 9.3% (n = 7), aTG in 8% (n = 6), and aTPO and aTG in 4% (n = 3); aTSH-R was not detected in any of the patients. Significant positive correlations (P <0.05) were observed between aTPO and DAS28 (r = 0.35, P = 0.002), aTG and ESR (r = 0.25, P = 0.02), and aTG and CRP (r = 0.23, P = 0.04). There were significant differences in the mean DAS28 between the aTPO­positive and aTPO­negative groups (5.35; 95% confidence interval [CI], 4.39-6.3 vs. 4.12, respectively; 95% Cl, 3.81-4.43; P = 0.017) and between the aTG-positive and aTG-negative groups (5.65; 95% Cl: 4.64-6.67 vs. 4.11; 95% Cl: 3.81-4.41; P = 0.005; respectively). CONCLUSIONS: Our results suggest that RA activity may be associated with the presence of antithyroid antibodies. This finding could be useful in the clinical evaluation of RA patients.

Identification of latent tuberculosis infection in rheumatic patients under consideration for treatment with anti-TNF-α agents.

INTRODUCTION: Immunosuppressive therapy with anti-tumour necrosis factor-α (TNF-α) agents in rheumatic patients modulates the immune system and may increase the risk of reactivating infections that are normally maintained in a latent state, such as tuberculosis. The purpose of this study was to analyse the value of QuantiFERON TB Gold In-Tube (QFT IT) and tuberculin skin test (TST) in BCG vaccinated patients with rheumatoid arthritis and ankylosing spondylitis who were qualified to receive TNF-α blockers. MATERIAL AND METHODS: Ninety patients with rheumatoid arthritis and ankylosing spondylitis were included in the study. The control group consisted of 20 healthy participants. Chest X-ray, TST and QFT IT were carried out in all persons. RESULTS: In rheumatic patients positive results of QFT IT and TST tests were identified in 15 cases (16.7%) whereas negative results of both tests were detected in 56 cases (62.2%). In the group of examined patients, 11 (12.2%) had QFT IT-/TST+ test results. In patients with QFT IT+/TST- status one active tuberculosis case was detected. In the control group QFT IT positive results were found in 4 cases (20%) and TST positive in 11 cases (55%). Treatment with TNF-α blockers was introduced in 26 rheumatology patients with the following test status: 3 with QFT IT+/TST+; 20 with QFT IT-/TST-; 3 with QFT IT-/TST+. CONCLUSIONS: In the BCG vaccinated population the QFT IT assay may potentially improve the identification and selection for therapy for latent TB infection before treatment with anti-TNF agents.

[The overlap of primary Sjögren's syndrome with antiphospholipid syndrome in man: case report]. / Zespól Sjögrena skojarzony z zespolem antyfosfolipidowym u mezczyzny--trudnosci diagnostyczne: opis przypadku.

We describe the case a 63-year-old man with overlap of primary Sjögren syndrome (pSS) and antiphospholipid syndrome (APS). According to literature the presence of antiphospholipid antibodies is observed in about 20% of patients with pSS. The coexistence of pSS and APS should be considered as an infrequent, but not exceptional, event.