Comparison of Ankylosing Spondylitis Disease Activity Score and Bath Ankylosing Spondylitis Disease Activity Index tools in assessment of axial spondyloarthritis activity.

Axial spondyloarthritis (axSpA) is an inflammatory joint disease, in which the dominant symptom is inflammatory back pain. It affects approximately 1% of the population, with a higher incidence in males. Spinal pain associated with spondyloarthritis is referred to as inflammatory back pain. In clinical practice, it is extremely important to be able to assess the activity of inflammatory back diseases and to select appropriate treatment and monitor the therapy. Currently, two main tools are used for assessment of the activity of axial spondyloarthritis: BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) and ASDAS (Ankylosing Spondylitis Disease Activity Score). The BASDAI is a tool used for years for assessment of disease activity, determining eligibility for treatment, and making decisions about continuation of therapy. Since BASDAI depends entirely on patient self-assessment, it is considered less objective than the ASDAS index. In turn, the latter includes not only answers to questions provided by the patient but also a parameter of inflammation such as erythrocyte sedimentation rate or C-reactive protein (CRP). Additionally, increasing numbers of studies report advantages of the ASDAS index over BASDAI. Moreover, as indicated by ASAS/EULAR (Assessment in Spondyloarthritis International Society/European Alliance of Associations for Rheumatology) 2022, ASDAS, especially ASDAS-CRP is the preferred tool for assessment of the activity of axSpA, whereas BASDAI is used only when the evaluation of the ASDAS is not possible. This paper presents the definition and symptoms of axSpA and reviews the latest research on ASDAS and BASDAI, with emphasis on the objectivity of the ASDAS assessment also presenting the doubts and limitations concerning this tool.

Osteoporosis treatment with denosumab in routine clinical practice in Poland.

INTRODUCTION: The receptor activator for nuclear factor k B ligand (RANKL) inhibitor denosumab is approved for the treatment of osteoporosis in postmenopausal women and men at increased fracture risk. The objectives were to describe the characteristics of patients with osteoporosis initiating denosumab in Polish clinical practice and their clinical management during the first 12 months of denosumab treatment. MATERIAL AND METHODS: This prospective, observational study enrolled denosumab-naïve women and men in Poland with osteoporosis, who had received at least one denosumab injection in the 8 weeks prior to enrolment. Patients were enrolled from specialist osteoporosis treatment centres, and orthopaedic, rheumatological, and family doctor centres. Outcomes included patient characteristics, denosumab treatment patterns, bone mineral density (BMD), and fracture; all analyses were descriptive. RESULTS: The study enrolled 463 patients; most (96%) were women, aged ≥ 65 years (84%), with prior fractures (88%). Approximately two-thirds of the women had received prior osteoporosis therapy, with the main reasons for discontinuation being adverse events (75%) and lack of effect (73%). Across all patients, the most common reasons for prescribing denosumab were low bone mineral density (BMD/T-score) (93%) and history of osteoporotic fracture (78%). Mean BMD at denosumab initiation ranged from T-score -3.00 (lumbar spine) to T-score -2.6 (total hip), and BMD increased by 2.8-6.2% at month 12. Most patients completed follow-up (86%) and were due to receive a third denosumab injection (81%). CONCLUSION: The article presents detailed sociodemographic and disease-related characteristics of patients who routinely implemented denosumab therapy. Most of them continued denosumab for at least 12 months, with increased BMD T-scores.

Arterial hypertension in systemic sclerosis.

Introduction: Arterial hypertension (AH) is common in systemic connective tissue diseases. Aim: To evaluate the incidence of AH in patients with systemic sclerosis (SSc) and to present clinical characteristics of the group diagnosed with AH. Material and methods: The study involved 108 patients with SSc divided into two groups: with AH (+) - 45 and AH (-) - 63. Moreover, the serological profile, scleroderma renal crisis, involvement of internal organs and mortality were determined. The kidney function was assessed based on creatinine concentration and the estimated glomerular filtration rate (eGFR). Results: AH was diagnosed in 47/108 SSc patients (41.7%). The age difference among patients was statistically significant and was higher in the AH (+) SSc group (p = 0.026). The incidences of oesophageal involvement (p = 0.011), digital ulcerations (p = 0.017), and mortality (p = 0.019) were found to be significantly higher in the AH (+) SSc group. Scleroderma renal crisis was observed in 9/108 patients (8.3%). The incidence of chronic kidney disease (CKD) was higher in the AH (+) SSc group, both of stage 2 (p = 0.013) and 3 (p = 0.07). Stages 4 and 5 of CKD were found only in the group with AH. Moreover, this group had a higher incidence of elevated uric acid (p = 0.007). Conclusions: AH is relatively common in patients with SSc and is associated with a significantly more severe course of the disease and higher frequency of renal involvement.

Methods of assessment of joint involvement in various systemic connective tissue diseases.

Joint involvement is one of the most common clinical manifestations of systemic connective tissue diseases (CTD). Joint symptoms can take various forms, ranging from joint pain to mono-arthritis or symmetrical poly-arthritis. In most cases, arthritis takes a non-destructive form, such as in the course of systemic lupus erythematosus or primary Sjögren's syndrome, to destructive arthritis in overlap syndromes of CTD with rheumatoid arthritis. In addition, apart from the wide variety of forms of joint involvement, it should be noted that joint symptoms may be one of the domains suggesting a severe course of the disease. The study attempts to present the methods of assessing the involvement of the locomotor system. The search for appropriate scales to determine the degree of joint involvement is important in assessing the severity of joint changes, has an impact on the overall degree of disease activity, and allows for timely implementation of appropriate treatment.

The prevalence and significance of anti-PM/Scl antibodies in systemic sclerosis.

INTRODUCTION: Anti-PM/Scl (a-PM/Scl) antibodies are found in different systemic autoimmune diseases such as polymyositis, dermatomyositis, systemic sclerosis (SSc) and overlap syndromes. According to literature, they are detected in approx. 2% of SSc patients, but their presence is more common in SSc with myositis overlap. OBJECTIVE: The aim of the study was to assess the prevalence of a-PM/Scl in patients with SSc and to identify differences in the clinical profile of the disease in patients with the presence of a-PM/Scl. MATERIAL AND METHODS: The study was performed on 126 European Caucasian SSc patients (98 females and 28 males) hospitalized consecutively in the Department of Rheumatology and Connective Tissue Diseases. The study group was analyzed for the potential presence of a-PM/Scl using a commercial test - EUROLINE Systemic Sclerosis Profile. The detection and interpretation were carried out electronically using the specific Euroimmun - EUROLineScan programme. The subtype of SSc, incidence of internal organ involvement and serological profile were determined in the entire group. Due to the presence of a-PM/Scl, patients were divided into two groups: a-PM/Scl (+) SSc - 22 patients and a-PM/Scl (-) SSc - 104 patients. RESULTS: A-PM/Scl was detected in 22/126 patients with SSc (17.5%). A strong correlation was found between a-PM/Scl and myalgia or myositis (p = 0.0379), hand joints contractures (p = 0.0002) and the prevalence of overlap syndrome (p = 0.0142). There were no relationships between the presence of a-PM/Scl and subtypes of SSc, other organ involvement, digital ulcers or calcinosis. CONCLUSIONS: Anti-PM/Scl antibodies are fairly common in patients with systemic sclerosis. In SSc, anti-PM/Scl antibodies are frequently associated with myalgia or myositis, hand joint contractures and an overlap syndrome.

Clinical and serological parameters of progression and prognosis in patients with systemic sclerosis - a state of the art review.

OBJECTIVE: Systemic sclerosis (SSc) is a multi-organ connective tissue disease that leads to the dysfunction and the impaired morphology of blood vessels due to non-specific inflammation and progressive fibrosis. Due to the diversity of SSc and even though the factors predisposing to the severe course of SSc are known, it is not always possible to predict the disease progression and to determine the prognosis. Ideally, the group of patients with faster progression of organ lesions and a worse course of the disease should be identified and the early intensive treatment should be instituted. The aim of the article, is an attempt to identify the factors that worsen the prognosis in the course of SSc. The analysis of numerous studies demonstrated that patients with short-lasting SSc, with the presence of anti-RNA polymerase III antibodies, with a generalized type of SSc with quickly progressing skin lesions and males should be most strictly monitored. Moreover, vascular complications, tendon ruptures and fast capillaries loss observed in nailfold capillaroscopy are the factors deteriorating the prognosis in SSc. CONCLUSION: In conclusion, despite the known, the factors that worsen the prognosis, it is difficult to predict the course of systemic sclerosis. Due to its incompletely elucidated etiopathology as well as the diverse and unpredictable nature of the disease, reliable markers to determine the prognosis in SSc have not been found.

Haematological abnormalities in systemic sclerosis.

Systemic sclerosis (SSc) is a connective tissue disease characterised by extremely high heterogeneity. This heterogeneity concerns the organ involvement, course of disease and prognosis. Unlike in some other systemic connective tissue diseases, especially systemic lupus erythematosus, in SSc haematological disorders occur rarely. When they develop, they affect erythrocytes, leucocytes and platelets. The most common cause of this pathology of erythrocyte abnormalities is microcytic anaemia resulting from micro-haemorrhages with telangiectasias within the digestive mucosa in patients with SSc. In SSc patients with severe haematological disturbances, the differential diagnosis should include overlapping with another systemic connective tissue disease or a haemato-oncological disease (lympho/myeloproliferative syndrome). In SSc patients with monoclonal proteins or cryoglobulins, it is essential to consider a haemato-oncological disease. In such cases, the differential diagnosis should be focused on a paraneoplastic syndrome, especially when the haematological symptoms develop shortly after the diagnosis of SSc and in the elderly.

Clinical consequences of the presence of anti-RNA Pol III antibodies in systemic sclerosis.

INTRODUCTION: Anti-RNA polymerase III (a-RNA Pol III) antibodies are marker antibodies in patients with systemic sclerosis (SSc). AIM: To assess the prevalence of a-RNA Pol III in patients with SSc and to identify the differences in the disease picture in SSc patients with and without a-RNA Pol III antibodies. MATERIAL AND METHODS: The study was performed in 126 SSc patients. The subtype of SSc, incidence of internal organ involvement, malignancy, death and serological profiles were determined in the entire group. The study groups were studied according to the presence of antibodies by applying the commercial test - EUROLINE SSc Profile. Due to the presence of a-RNA Pol III, patients were divided into two groups: the a-RNA Pol III (+) SSc group of 19 patients and the a-RNA Pol III (-) SSc group of 107 patients. RESULTS: A-RNA Pol III were present in 19/126 patients with SSc (15%), 13/19 (68.4%) patients had no other SSc marker antibodies. A-RNA Pol III were more common in patients with diffuse cutaneous SSc (p = 0.049). We showed a significant positive association between a-RNA Pol III and occurrence of malignancy (p = 0.007), scleroderma renal crisis (p = 0.001) and decreased DLCO (p = 0.007). CONCLUSIONS: Anti-a-RNA Pol III antibodies are common in patients with SSc, particularly with a diffuse subtype. In more than 50% of patients with a-RNA Pol III antibodies, they may be present as the sole marker of antibodies. In SSc, a-RNA Pol III antibodies are frequently associated with malignancy occurrence, kidney and lung involvement.

[Late-age onset systemic sclerosis].

Systemic sclerosis is a multi-organ connective tissue disease characterised by dysfunction and impaired morphology of the blood vessels with non-specific inflammation and progressive fibrosis. In the majority of cases, the onset is observed between 30-50 years of age; in many cases, however, the diagnosis is established in patients < 20 years of age or > 75 years of age. The course of late-onset systemic sclerosis is markedly different from that in early- onset disease. In late-onset patients, limited systemic sclerosis, pulmonary hypertension, primary heart involvement, and anti-centromere antibodies are more commonly observed. Moreover, the diagnosis of systemic sclerosis in patients > 60 years of age is associated with poor prognosis, higher mortality rates, and an increased risk of neoplasms, as compared to younger patients.

Urticarial rash, fever, and arthritis: A case of refractory Adult-onset Still's disease with good response to tocilizumab.

Adult-onset Still's disease (AOSD) is a rare, systemic inflammatory disorder of not completely understood etiology. Aberrant activation of the innate immune system and overproduction of several pro-inflammatory mediators are considered a critical component in disease pathogenesis. AOSD still poses a challenge due to the broad range of differential diagnoses and no specific biomarkers. Four cardinal symptoms are quotidian spiking fever, joint involvement, evanescent salmon pink-rash rash, and leukocytosis with neutrophilia. We present a case of a 61-year-old female with a recurrent urticarial rash accompanied by attacks of high fever, tender joints, sore throat, enlarged liver, elevated inflammatory reactants, and hyperferritinemia. After an extensive workup, the patient fulfilled the criteria of AOSD. She was refractory to the glucocorticosteroids and disease-modifying anti-rheumatic drugs (DMARDs). Finally, after several unsuccessful attempts to achieve disease control with traditional DMAR's administration of Tocilizumab (TCZ), a humanized anti-IL-6 receptor antagonist resulted in substantial disease improvement. Since skin manifestations are a common feature of AOSD, it should be among differential diagnoses in patients with skin lesions and constitutional symptoms. Biologic agents represent a significant therapeutic advance in patients with AOSD refractory to conventional therapy.

Anti-CCP antibodies and rheumatoid factor in systemic sclerosis: Prevalence and relationships with joint manifestations.

BACKGROUND: It is known that anti-citrullinated protein (a-CCP) antibodies and rheumatoid factor (RF) can be present in systemic sclerosis (SSc) patients, particularly with joint involvement. OBJECTIVES: The aim of the study was to assess the prevalence of a-CCP antibodies and immunoglobulin M class (IgM) RF, and the relationships between their presence and joint manifestations in patients with SSc. MATERIAL AND METHODS: The study included 100 European Caucasian SSc patients hospitalized consecutively in the Department of Rheumatology and Connective Tissue Diseases (Lublin, Poland). Anti-citrullinated protein antibodies and IgM RF were determined using a commercial enzyme-linked immunosorbent assay (ELISA) test. RESULTS: Anti-citrullinated protein antibodies were found in 10 out of 100 (10%) SSc patients and IgM RF in 71 out of 100 (71%) SSc patients. In the study, 90/100 (90%) SSc patients had joint manifestations (arthralgia or arthritis), 34/100 (34%) had arthritis and 6/100 (6%) had a systemic sclerosis-rheumatoid arthritis (SSc-RA) overlap syndrome. Significantly higher a-CCP antibody levels (p = 0.012), erythrocyte sedimentation rate (ESR) (p = 0.029) and C-reactive protein (CRP) levels (p = 0.020) were observed in the SSc group with arthritis. A significant correlation was found between the group with arthritis and the presence of a-CCP antibodies, and between the arthralgia group and the presence of IgM RF. CONCLUSIONS: The prevalence of RF and a-CCP antibodies is relatively high in SSc, and joint involvement occurs frequently. There was a significantly higher prevalence of IgM RF in the group with joint manifestations. About 1/3 of SSc patients had symptoms of arthritis. Arthritis is connected with the presence of a-CCP antibodies, while arthralgia is connected with the presence of IgM RF.

Overlap syndromes in systemic sclerosis.

INTRODUCTION: It is known, that course of the disease differs between overlap syndromes (OS) and systemic sclerosis (SSc) group. AIM: To compare the prevalence of OS in limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous SSc (dcSSc) and to analyze the presence of different manifestations in the SSc and OS group. MATERIAL AND METHODS: The study included 126 European Caucasian SSc patients (99 females and 27 males) hospitalized consecutively in the Department of Rheumatology and Connective Tissue Diseases. Patients fulfilled the American College of Rheumatology (ACR) classification criteria of SSc (57 - dcSSc and 69 - lcSSc). The study groups were determined according to the subtype of SSc, coexistence of other connective tissue diseases (CTDs), and incidence of clinical and serological manifestations. RESULTS: In our SSc study group, 28/126 patients (22%) were affected by more than one CTD. The prevalence of OS was significantly higher in the lcSSc group - 33% (23/69) compared to the dcSSc group - 8% (5/57). We found that mortality and digital ulcers were significantly higher, whereas kidney involvement and arthritis were significantly lower in the SSc group compared to the OS group. The prevalence of anti-topoisomerase I (a-Scl-70) was significantly higher, and prevalence of anti-PM/Scl, anti-Ro-52 antibodies was significantly lower in the SSc group compared to the OS group. CONCLUSIONS: Overlap syndromes were more common in lcSSc than in dcSSc. The course of the disorder and internal organ involvement were different in OS compared to SSc patients.

[Clinical and serological diversity in systemic sclerosis].

Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by vascular damage with non-specific inflammation and progressive fibrosis. The disease exhibits a large heterogeneity in both clinical and serological features. This diversity refers to the course of the disease, which is difficult to predict in many cases. Although poor prognostic factors are well known, it is not always possible to predict the course of the disease and the prognosis. Furthermore, the activity of disease is difficult to assess since both reversible and irreversible lesions can occur in one patient. To date, numerous attempts have been made to assess the SSc activity. In 2017 the disease activity score was updated. Discrimination between an active phase of the disease and the disease with irreversible organ changes is of particular importance, when immunosuppressive treatment is considered, as this kind of therapy should be used in cases with active, reversible lesions. Moreover, while assessing the course of the disease, special attention is paid to the effects of serological markers on clinical symptoms and severity of the disease. Numerous ongoing studies focus on new serological biomarkers and their influence on internal organ involvement. This paper analyses our observations and presents literature review to determine the importance of various clinical and serological symptoms and their association with the course and the prognosis of SSc.

[Modern perspective on the development conditions and diagnosis of systemic sclerosis].

Systemic sclerosis (SSc) is a multisystem disorder of the connective tissue characterized by a great deal of heterogeneity. This variability is a result of a combination of vascular damage inflammation and fibrosis leading to internal organ complications. The pathogenesis of disorder is still unknown. A large part is given to genetic, epigenetic and environmental factors. There is ongoing research into the new cells and mediators involved in the complicated development of disease. The overlap of vascular, autoimmune/ inflammatory alternations and fibrosis causes the multidirectional and unpredictable course of disease. Vascular complications may dominate in one group, whereas fibrosis in others. In addition, the disorder is characterized by individual variability; therefore although certain prognostic markers do exist, the course of disorder in largely unpredictable. This complexity of SSc, makes it difficult in terms of diagnosis and activity assessment, especially in early stages. Over a number of years, further classification criteria, which were more sensitive and more specific, were developed. The 2013 European League Against Rheumatism/American College of Rheumatology - EULAR/ACR classification criteria for SSc, were revised to include diagnosis in early stages and particularly in limited SSc. Unfortunately, there still exist certain groups of population which do not fulfil these criteria but develop the disorder; therefore, the search for new diagnostic methods which could elevate SSc diagnosis continues.

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.

Coexistence of diabetes mellitus type 1 with diffuse systemic sclerosis - case report and literature review.

Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud's phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. The patient presented a typical capillaroscopic scleroderma-like pattern, antinuclear antibodies and sclerotic lesions in gastrointestinal system. Summing up, our case represents the rare coexistence of autoimmune diseases like diabetes mellitus type 1 and systemic sclerosis.

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.

[Autoimmune polyglandular syndromes - literature review and analysis of clinical course in chosen cases]. / Zespoly autoimmunologicznych zaburzen wielogruczolowych - przeglad pismiennictwa oraz analiza przebiegu choroby u wybranych przypadków.

Autoimmune polyglandular syndromes (APS) are the conditions characterized by coexistence of at least two organ-specific endocrine autoimmune disorders. The syndromes often coexist with connective tissue diseases with the presence non-organ specific antibodies. The aim of the study was to present two clinical cases of polymyositis and dermatomyositis in the course of APS type 3d. Case 1, a 24-year-old woman, with diabetes mellitus type 1 was diagnosed at the age of 17 years and polymyositis recognized at the age of 24 years based on clinical manifestations and additional tests (proximal muscle weakness, typical electromyographic changes, typical histopathological changes in skin and muscles biopsy, elevated muscle enzymes) accompanied by interstitial lung disorder. Moreover, Hashimoto`s autoimmune thyroiditis was diagnosed. Case 2, a 47-year-old man, with a several-year history of diabetes mellitus type 1, diagnosed with dermatomyositis and autoimmune thyroiditis. The immunosuppressive therapy was instituted in both cases, which reduced the symptoms of connective tissue disease. To sum up, about 25-30% of patients are affected by extraglandular autoimmune diseases, including connective tissue diseases, in the course of APS. An interdisciplinary approach is required in this group of conditions due to a multitude of disorders as well as diagnostic and therapeutic difficulties.

[Autoimmune diseases with the presence of anti-ku antibodies - analysis of three cases]. / Choroby autoimmunologiczne z obecnoscia przeciwcial anty-ku na podstawie analizy trzech przypadków klinicznych.

a-Ku are rare antibodies, which are reported in course of connective tissue diseases. Their prevalence ranges from 0 to 10% , 2%, on average. The main symptoms associated with the presence of a-Ku antibodies include: myositis, arthritis, Raynaud`s phenomenon and skin lesions. The above features are often defined as autoimmune clinical syndrome associated with a-Ku antibodies. In recent years, three cases with the presence of a-Ku antibodies were observed at the Department of Rheumatology and Connective Tissue Diseases. Case 1, a 77-year-old man, with the diagnosis of mixed connective tissue disease according to Raynaud`s phenomenon, myositis, arthritis and presence of a-ribonucleoprotein antibodies. Moreover, secondary Sjögren syndrome (SS) and myasthenia gravis were diagnosed. Case 2, a 56-year-old woman with longstanding history of Raynaud`s phenomenon, sclerodactyly, myositis and arthritis. Based on clinical manifestations and additional tests, systemic sclerosis and myositis were diagnosed. Case 3, a 46-year-old woman with SS diagnosis, long-standing history of Raynaud`s phenomenon, arthralgia and polyneuropathy. Moreover, HCV infection with the presence of cryoglobulin was confirmed. The presence of a-Ku antibodies in high titers was found in all cases. The clinical conditions improved after steroid and immunosuppressive therapy. In conclusion, clinical syndromes with the presence of a-Ku antibodies are associated with a wide range of non-specific symptoms, regarding muscle, joint and skin involvement, in particular. The conditions are more often diagnosed in the elderly; in the majority of cases, they are characterized by mild courses, good response to steroid therapy and good prognosis.