Autoantibodies and Clinical Correlations in Polish Systemic Sclerosis Patients: A Cross-Sectional Study.

We evaluated the prevalence of systemic sclerosis (SSc)-related autoantibodies and their clinical significance and compared the sensitivity of two line immunoblot assays on a prospective study group of 96 Polish SSc patients (ACR-EULAR 2013 criteria) whose sera were assessed by indirect immunofluorescence (HEp-2 and monkey liver) and line immunoblot assays: ANA Profile 3 and Systemic Sclerosis Profile by EUROIMMUN (Lübeck, Germany). Organ involvement was evaluated according to the EUSTAR Minimal Essential Data Set. The following autoantibodies' prevalence was found: Scl-70 (36%), Ro-52 (28%), CENP-B (22%), CENP-A (20%), PM-Scl-75 (20%), PM-Scl-100 (14%), fibrillarin (7%), Th/To (7%), RNA polymerase III 11 kDa (5%), RNA polymerase III 155 kDa (3%), PDGFR (3%), NOR-90 (2%), and Ku (1%). Significant associations between the autoantibodies' presence and organ involvement were found: ATA (dcSSc > lcSSc, less prevalent muscle weakness), Ro-52 (gangrene, DLCO < 60), CENP-B and A (lcSSc > dcSSc, normal CK), CENP-B (rarer digital ulcers and joint contractures), PM-Scl-100 and 75 (PM/SSc overlap, CK increase, muscle weakness, muscle atrophy), PM-Scl-100 (dcSSc unlikely), PM-Scl-75 (lung fibrosis), fibrillarin (muscle atrophy, proteinuria, conduction blocks, palpitations), Th/To (proteinuria, arthritis, muscle weakness, and rarer esophageal symptoms), RNA Polymerase III 11 kDa (arterial hypertension, renal crisis), RNA polymerase III 155 kDa (renal crisis), and PDGFR (dcSSc, tendon friction rubs). Additionally, the Systemic Sclerosis Profile was significantly more sensitive in detecting SSc-related autoantibodies than ANA Profile 3 (p = 0.002). In conclusion, individual autoantibodies associated with specific characteristics of SSc.

Relationship between changes observed in nailfold capillaroscopy and serological profile, lung fibrosis, and elevated risk of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease.

Introduction: Microvascular changes play a significant role in systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The most serious complications of SSc and MCTD are lung fibrosis (LF) and pulmonary hypertension (PH). Aim: To determine the relationship of the changes observed in capillaries with the serological profile, LF, PH, and finger ulcerations in patients with SSc and MCTD. Material and methods: The tested group comprised 80 persons (61 SSc, 19 MCTD); mean age 53.6 ±13.6 years. Patients were qualified to the LF group based on HRCT. Likelihood of PH was determined using echocardiography. The presence of antinuclear antibodies (ANA) was assessed using indirect immunofluorescence, while ANA profile, and sclerosis profile were assessed using EUROIMMUN kits, and antiphospholipid antibodies (aPL) using the ELISA method. Capillaroscopy was performed using the Nikon CPS 160 optical microscope. Results: The following were found: a relationship between occurrence of anti-SS-A (p = 0.006) and anti-centromere B antibodies (p = 0.012) and ramified vessels, between anti-SS-B and capillary haemorrhages (p = 0.019), a positive correlation between NOR90 antibodies and winding loops (p = 0.021), PM-Scl 100 antibodies and enlarged vessels (p = 0.033), a negative correlation between Scl-70 antibodies and winding loops (p = 0.033), and a relationship between aCL and winding loops (p = 0.002). No relationship between the capillaroscopy image and PH risk was found. A positive correlation was found between avascularisation areas and LF and between giant capillaries and finger ulcerations. A negative correlation was found between U1-RNP antibodies and finger ulcerations (p = 0.009), and a positive correlation between antibodies to fibrillarin and ulcerations (p = 0.028). Conclusions: SS-A, SS-B and anti-centromere antibodies are associated with the late phase of sclerodermic microangiopathy. Avascularisation areas significantly correlate with a higher prevalence of LF. U1-RNP antibodies have a protective role, while anti-fibrillarin antibodies are the risk factor for finger ulcerations.

IL-1ß, IL-10 and TNF-α polymorphisms may affect systemic lupus erythematosus risk and phenotype.

OBJECTIVES: Systemic lupus erythematosus (SLE) is an autoimmune disease, and IL-1ß, IL-10, and TNF-α genes are important in the pathogenesis of this disease. We studied the impact of IL-1ß-511, IL-1ß +3953, IL-10 -592, IL-10 -1082, TNF-α -308, TNF-α -238, and TNF-α +489 polymorphisms on SLE risk and phenotype in SLE patients and healthy controls. METHODS: We genotyped SLE patients and healthy controls by real-time PCR on QuantStudio 5 (Applied Biosystems) and measured levels of cytokines by enzyme-linked immunosorbent assay (ELISA). RESULTS: We indicated that TNF-α -308, IL-10 -592, IL-10 -1082, IL-1ß-511 and IL-1ß +3953 polymorphisms affect SLE risk. Furthermore, we exposed that some of the TNF-α +489, TNF-α -238, IL-10 -1082 and IL-1ß +3953 genotypes are connected with the SLE phenotype. Moreover, we discovered the linking between specific genotypes and the serum concentrations of TNF-α, IL-1ß, and IL-10. CONCLUSIONS: In conclusion, our study revealed that IL-1ß-511, IL-1ß +3953, IL-10 -592, IL-10 -1082, and TNF-α -308 polymorphisms may affect SLE risk and phenotype.

Vitamin D supply in patients with rheumatic diseases in Poland - a pilot study.

OBJECTIVES: In rheumatic diseases, vitamin D supply is recommended as part of the prophylaxis and treatment of osteoporosis, especially in patients undergoing glucocorticoid therapy, but also due to its immunoregulatory and anti-inflammatory properties. We aimed to evaluate serum 25-hydroxyvitamin D [25(OH)D3] levels in Polish patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and granulomatosis with polyangiitis (GPA), in relation to various clinical parameters, and to assess the initial range of doses for the purpose of further research. MATERIAL AND METHODS: 112 patients (39 with SLE, 44 with SSc and 29 with GPA), referred to the Department of Rheumatology and Internal Medicine in Poznan, Poland, were enrolled in this retrospective study. Demographic and clinical data were collected, including 25(OH)D3 serum levels, vitamin D supplementation doses and season of blood sampling. RESULTS: Mean (SD) serum 25(OH)D3 concentrations were 31 (19.4) ng/ml for SLE, 28.8 (12.5) ng/ml for SSc and 28 (15.2) ng/ml for GPA, and they did not significantly differ between the groups. Vitamin D levels below the optimal range were found in 43.8% of SLE, 65.9% of SSc and 72.4% of GPA patients. 80% of patients reported vitamin D intake, with a mean daily dose of 1398 IU for SLE, 1345 IU for SSc and 1689 IU for GPA. Vitamin D insufficiency and deficiency were frequent among patients with rheumatic diseases, independently of the diagnosis and season. CONCLUSIONS: Patients with rheumatic diseases seem to require higher doses of vitamin D than recommended for the general population. The present results indicate the necessity to use higher initial doses of vitamin D in this group of patients (2000 to 4000 UI) and to maintain the dose of vitamin D regardless of the change of seasons.

The Role of MECP2 and CCR5 Polymorphisms on the Development and Course of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic and systemic autoimmune disease. SLE is described by production of autoantibodies and causes damage of many organs. T-cells play a crucial role in SLE pathogenesis. T-cells intensify inflammation through a number of processes, which leads to autoimmunization. CCR5 and MECP2 genes are linked with T-cells and pathogenesis of SLE. Polymorphisms in these genes are related with the prognostic factors of risk of disease onset and disease severity. The aim of this study was to estimate the influence of polymorphisms in MECP2 and CCR5 genes on the development and course of systemic lupus erythematosus. We examined 137 SLE patients and 604 healthy controls. We studied polymorphisms for CCR5 gene: rs333 and for MECP2: rs2075596, rs1734787, rs17435, and rs2239464. We genotyped our MECP2 samples and we performed a restriction fragment length polymorphism (RFLP) analysis for CCR5 samples. We showed a risk factor for allele T in rs17435 and for allele A in rs2075596 in MECP2. We noticed that MECP2 rs2075596 G/A, rs1734787 C/A, rs17435 A/T, and rs2239464 G/A polymorphisms are more prevalent in SLE patients than in healthy controls. We believe that above-mentioned MECP2 polymorphisms can be considered as SLE susceptibility factor.

Genetic Background of Hypertension in Connective Tissue Diseases.

Peroxisome proliferator-activated receptors (PPAR gamma-2) and beta-3-adrenergic receptors (ADRB3) are involved in the risk of hypertension. But their exact role in blood pressure modulation in patients with connective tissue diseases (CTD) is still not well defined. In this study, 104 patients with CTD and 103 gender- and age-matched controls were genotyped for Pro12Ala and C1431T polymorphisms of the PPAR gamma-2 gene and Trp64Arg polymorphism of the ADRB gene. Anthropometric and biochemical measurements were evaluated, followed by genotyping using TaqMan® SNP genotyping assays and polymerase chain reaction-restriction fragment length polymorphism. The prevalence of analyzed genotypes and alleles was comparable between patients with CTD and the control group, as well as hypertensive and normotensive subjects. Patients with CTD have lower body fat and higher body water amount, serum glucose, and triglyceride (TG) levels. Hypertensive subjects are older and have higher body mass, BMI, waist circumference (WC), body water content, glucose, and TG concentration. The multivariate analysis revealed that hypertensive subjects with Ala12/X or Trp64Trp have higher body mass and WC when compared to normotensive subjects. Trp64Trp polymorphism was also characterized by a higher TG level, while T1431/X subjects had higher WC. The presence of CTD, visceral fat distribution, and increased age are the predictors of hypertension development. Hypertensive patients with CTD and Trp64Trp polymorphism have an increased risk of visceral obesity development and metabolic complications, which in turn affects the value of blood pressure. In addition, either Ala12/X or T1431/X predicts the visceral body fat distribution in hypertensive subjects.

Immunodeficiency and autoimmunity during biological disease-modifying antirheumatic drug therapy.

Biological disease-modifying antirheumatic drugs target specific components of the immune response related to pathogenesis of autoimmune and inflammatory diseases. Introduction of biologic therapies has enabled better disease control than conventional drugs and thus a reduction in comorbidity and mortality. However, there is concern about adverse effects of these drugs including infections, cancers and drug-induced autoimmune diseases. Patients undergoing biologic treatment are at small but significant risk of serious infections. The overall risk of malignancies in patients on biologics compared with the general population is not increased, but there is evidence of a higher risk of individual cancers. Surprisingly, biological treatment may induce autoantibody production and, rarely, development of autoimmune diseases. A growing body of literature has evaluated the risk of adverse effects during biologic therapies. This paper outlines adverse effects of biological disease-modifying antirheumatic drugs related to immune system disorders, both immunodeficiency and autoimmunity.

Exclusively ocular and cardiac manifestation of granulomatosis with polyangiitis - a case report.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibodies (ANCA)-associated necrotizing granulomatous vasculitis that affects small to medium size vessels. While the classical form with renal and respiratory tract involvement is mainly seen, a limited form (i.e., with no renal disease) may also occur. We present an unusual case of GPA manifesting merely as a bilateral ocular involvement and complete heart block. CASE PRESENTATION: We report a case of a 60-year-old male patient with a limited form of GPA who initially presented with bilateral chronic conjunctivitis and complete atrioventricular block. His visual acuity subsequently declined due to progression to bilateral panuveitis with exudative retinal detachment. The laboratory investigation revealed the elevation of acute phase reactants and strongly positive cytoplasmic ANCA (c-ANCA). Despite negative conjunctival and musculocutaneous biopsy results, the positive c-ANCA, and the clinical manifestation, i.e., heart and ocular involvement, led to the diagnosis of GPA. The remission was achieved with cyclophosphamide and methylprednisolone systemic therapy. CONCLUSIONS: A limited form of GPA may be a diagnostic chameleon. Though rare, it is essential to consider even extremely uncommon findings. Our patient is the first case of such a unique demonstration of the limited GPA manifesting as a bilateral ocular involvement and complete heart block.

IL-6 and TGF-ß gene polymorphisms, their serum levels, as well as HLA profile, in patients with systemic lupus erythematosus.

OBJECTIVES: The aim of the study was to explore whether TGF-ß and IL-6 gene polymorphisms may be associated with SLE and assess the frequency of HLA-DRB1 alleles in Polish systemic lupus erythematosus (SLE) patients. METHODS: 216 SLE patients and 552 healthy individuals were examined for TGF-ß rs1800469 and rs1800470 by TaqMan SNP genotyping assay and for and IL-6(rs2069827 and rs1800795 using the PCR- RFLP method. RESULTS: An increased frequency of TT genotype and T allele of the TGF ß -509 C/T was found in SLE patients (p=0.02). The TGF-ß 869 C allele was more frequent in SLE patients. The genotype-phenotype analysis showed association between the TGF ß -509 C/T and mean value of CRP, ESR, haemoglobin, APTT, Pt and INR (p=0.05, p=0.03, p<0.001, p=0.03, p=0.03 and p=0.05, respectively) as well as anti-SSA and anti-Sm presence (p=0.04 and p=0.03, respectively); the TGF- ß 869 T/C and mean value of APTT and INR (p=0.01 and p=0.05, respectively); the IL-6 -174 G/C and SLICC (p=0.05), anti-SSA (p=0.05) and anti-SSB (p=0.05). A higher TGF-ß and IL-6 serum level were found in SLE patients compared to controls (both p<0.0001). In SLE patients with the TGF-ß -509 TT genotype have shown positive association with the TGF-ß serum levels. Polish SLE patients have strong positive association with HLA-DRB1*52.1, and negative with the HLA-DRB1*07:01 allele. HLA-DRB1*52.1 was also associated with higher TGF-ß serum levels in the Polish population. CONCLUSIONS: Our results suggested that the TGF ß -509 C/T variant may be considered as a genetic marker for SLE in the Polish population.

Cytokine secretion and the risk of depression development in patients with connective tissue diseases.

Research in the past two decades has revolutionized our understanding of depressive illnesses. Proinflammatory cytokines have become a point of interest in the interconnecting areas of neuropsychiatric and autoimmune diseases. The cytokine hypothesis of depression suggests that pro-inflammatory cytokines play a primary role in the mediation of the pathophysiological characteristics of major depression, in which an inflammatory process may be induced by external and internal stressors, such as psychological and inflammatory diseases, respectively. The higher prevalence of depression, particularly in patients with chronic autoimmune connective tissue disorders (CTD), suggests that depression may present a dysfunctional adaptation of cytokine-induced sickness, which could manifest in times of an exacerbated activation of the innate immune system. Inflammation is thought to contribute to the development of clinical depression through its ability to induce sickness behaviors corresponding to the neurovegetative features of depression, through the dysregulation of the hypothalamic-pituitary-adrenal axis, alterations in neurotransmitter synthesis and reuptake, and through its involvement in the neuroprogression pathways. This review explores the complex interrelationships in which inflammatory responses alter neuroendocrine and neuropsychological regulation contributing to depressive symptoms in CTD. The prevalence and characteristics of depression, and its correlation to the levels of inflammatory cytokines and disease activity among different CTD will be reviewed.

Systemic Sclerosis and Serum Content of Transforming Growth Factor.

Systemic sclerosis is a connective tissue disease characterized by tissue fibrosis leading to interstitial lung disease. Transforming growth factor-ß (TGF-ß) has been of interest as a potential diagnostic marker and also as a drug target in systemic sclerosis. The aim of this study was to assess the serum content of TGF-ß1 in patients with systemic sclerosis and to assess its potential role in tissue fibrosis. The study included 30 patients, 5 men and 25 women, of the mean age of 46.9 ± 12.8 years, diagnosed with systemic sclerosis. The control group consisted of 19 women of the mean age of 28.4 ± 7.8 years, diagnosed with primary Raynaud's disease. TGF-ß1 serum levels were measured, chest imaging examinations were performed, and fibrotic tissue changes were assessed using the modified Rodnan Skin Score. We found that the mean serum TGF-ß1 content in patients with systemic sclerosis was 598.7 ± 242.6 pg/mL, whereas it was 568.4 ± 322.2 pg/mL in the control group (p = 0.378). We also failed to substantiate any significant relationship between TGF-ß1 serum levels and the severity of pulmonary and skin fibrosis in systemic sclerosis. In conclusion, systemic sclerosis does not seem a disease that would be accompanied by a specific enhancement of serum TGF-ß1. Thus, this cytokine is rather unlikely to play an essential role in the development and course of the disease, nor can it be considered diagnostic or prognostic marker.

Chemerin, Resistin, and Adiponectin in Patients with Connective Tissue Diseases.

BACKGROUND: The exact role of cytokines in inflammation and metabolic disorders in case of connective tissue diseases (CTDs) is under discussion. METHODS: In this study, we intended to find the relationship between the selected cytokines in inflammatory and metabolic disorders in patients with CTDs (n=55) and compared the results with those of control group subjects (n=25) matched by age and body mass. We estimated their nutritional status by the bioimpedance method. The levels of basic biochemical parameters and the levels of adiponectin, resistin, and chemerin were also estimated. Multiple regressions and area under the curve in receiver operating characteristic (AUC-ROC) curve were used to find the associations of aforementioned parameters. RESULTS: Patients with CTDs exhibited higher levels of chemerin than that of control group subjects. We found an inverse relationship between chemerin, RBC count, and hemoglobin levels. The concentration of adiponectin inversely correlated with the levels of platelets and concentrations of glucose and triglycerides as well as the erythrocyte sedimentation rate, whereas the concentration of resistin was positively correlated with WBC count, C-reactive protein (CRP), and the amount of used oral glucocorticosteroids. The mean ± standard deviation for the AUC-ROC curve in case of chemerin was the highest (AUC-ROC=0.714, p=0.0005) than that of both resistin and adiponectin. CONCLUSIONS: Chemerin and resistin levels are related to the inflammatory state in patients with CTDs, whereas adiponectin levels seem to be correlated with a protective effect. Chemerin can be considered as a marker differentiating a proinflammatory state present in CTDs.

Antinuclear antibodies in healthy people and non-rheumatic diseases - diagnostic and clinical implications.

The presence of antinuclear antibodies (ANA) is mainly associated with connective tissue diseases (CTD). In addition, their presence is found in healthy people. These antibodies are more common in women and the elderly. Some drugs and xenobiotics are also important for the development of autoimmunity and ANA synthesis. Moreover, the deficiency of vitamin D in the body of patients correlates with occurrence of these antibodies. Unlike the healthy group, a positive ANA count was observed in patients with atopic dermatitis (AD) and in people with immune disorders. Antinuclear antibodies in low counts are also found in the course of chronic bacterial or viral infection and in patients with hematological malignancies. Also the possibility of false positive results, which may be caused by the choice of method used to determine antibodies, should be borne in mind. Taking into account all these factors, it is concluded that the ANA result itself has no diagnostic value.

Clinical implications of systemic lupus erythematosus without and with antiphospholipid syndrome in peri- and postmenopausal age.

Antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE) displays a severe disease sub-phenotype with vascular manifestations ranging from peripheral thrombosis to neurologic and ophthalmic symptoms. The prevalence of morbidities including thrombosis, renal lesions, and cognitive impairment contributes to a higher risk of organ damage and a reduced quality of life in patients. In addition to the clinical heterogeneity, the diagnostic challenge is heightened in elderly patients as APS-related SLE is primarily diagnosed in young females. Many patients reach menopause due to the clinical association of premature menopause and improvements in diagnostic and therapeutic strategies in recent years. Although obstetric morbidity is not a concerning feature of the disease within this age group, a number of manifestations which may contribute to a decreased quality of life are present and must therefore not be disregarded. An improved prognosis derives from successful therapeutic regimens with minimal adverse effects in individual patients. The multifaceted management involves patient evaluation and risk stratification, followed by thromboprophylaxis efforts through the correction of modifiable risk factors, lifestyle recommendations, and pharmacological therapy. This review highlights the role of estradiol in the disease pathogenesis as well as the clinical complications and management of APS-related SLE in perimenopausal and postmenopausal patients.

Clinical manifestations of granulomatosis with polyangiitis: key considerations and major features.

BACKGROUND: Granulomatosis with polyangiitis (GPA) presents a wide spectrum of manifestations from the common respiratory symptoms to infrequent neurological and cardiac complications. The challenge in diagnosis and management makes the rapidly progressive disorder one of the most challenging dilemmas in clinical medicine. METHOD: An extensive electronic literature search was done using multiple databases (e.g. PubMed) from January 2007 to August 2017 to gather information on the etiopathogenesis, clinical presentation, and current treatment management plan of GPA. The language used was English. Search items included 'Granulomatosis with Polyangiitis symptoms,' 'Wegener's Granulomatosis,' and 'Treatment of Granulomatosis with Polyangiitis' for the databases. Inclusion criteria consisted of published articles in the English language which reported on the clinical manifestations of GPA, as well as on the treatment of the disease. Exclusion criteria included articles that: (1) did not specifically report on GPA vasculitis but also focused on discussion of other vasculitis such as MPA or Churg-Strauss; (2) reported on treatment/symptoms of GPA prior to 2007. RESULT: This review provides an overview of work undertaken in key areas of GPA research, including its complex multifactorial etiology, clinical manifestations, treatment strategies, and treatment-related adverse effects. CONCLUSION: Major advances in the understanding and treatment of GPA over recent decades have contributed to the notable decline in morbidity and mortality of patients. The ultimate goal is an improved prognosis through outcome measures which assesses the disease control with minimal adverse effects of intensive immunosuppressive regimens, an integral part of the clinical approach to improve the quality of life of GPA patients.

Satisfaction and discontent of Polish patients with biological therapy of rheumatic diseases: results of a multi-center questionnaire study.

OBJECTIVES: Biologics are medications widely applied in the management of inflammatory rheumatic diseases. The drugs were found to be effective but their application is associated with some disadvantages. Medication with biologics is relatively expensive, and in Poland, it is carried out in specialized centers. The study was designed to evaluate various aspects of satisfaction and dissatisfaction of Polish patients treated with biologics. MATERIAL AND METHODS: An anonymous questionnaire was distributed in 23 Polish rheumatological centers involved in the treatment; 1212 returned questionnaires were used for analysis. Responses were received from 606 patients with rheumatoid arthritis, 427 with ankylosing spondylitis, 117 psoriatic arthritis, and 62 adult patients with juvenile idiopathic arthritis (in whom administration of the drugs had been introduced before they were 18 years old). The investigated group constituted about one-fifth of all rheumatic patients on biologics in Poland. RESULTS: A beneficial or very beneficial influence of the medication on the state of physical health was found mostly in patients with rheumatoid arthritis (51.3 and 30.5%) and ankylosing spondylitis (51.0 and 36.8%). Family life was improved by the treatment especially in patients with ankylosing spondylitis (40.7 and 35.6% beneficial and very beneficial, respectively), sleep quality and sexual life mostly in those with ankylosing spondylitis (beneficial/very beneficial influence 41.5/38.4, and 38.7/23.9, respectively). There was a rather small influence of biological treatment on the financial situation of the patients. In general, satisfaction with the treatment was evaluated as positive or very positive in 88% of all investigated patients.In a significant part of the patients, transportation to the medical center was considered as a disadvantage of the treatment. About one-third of the patients considered laboratory and imaging tests to be done before initiation of the medication as a difficulty, and for about 40% waiting time for qualification for the medication was a significant disadvantage. The route of drug administration was without importance for 4/5 of the patients. CONCLUSIONS: Summing up, the results were similar in the patients suffering from various diseases although those with psoriatic arthritis felt the highest satisfaction (possibly due to the positive aesthetic effect), and those with ankylosing spondylitis had significant improvement in sexual life (probably due to younger age). Relatively low satisfaction was found in patients with juvenile idiopathic arthritis. There was a small influence of medication on financial status of the patients. Application of biologics has few disadvantages and most of them are associated with the organization of health services (waiting time for the tests, transportation to the medical centers).

Prevalence of pulmonary hypertension in patients with systemic sclerosis and mixed connective tissue disease.

Systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) are 2 conditions in which pulmonary hypertension (PH) can develop.We retrospectively analyzed the probability of PH in case of 83 patients (69 SSc and 14 MCTD). The European Society of Cardiology/European Respiratory Society (ESC/ERS) echocardiographic guidelines of 2015 were used for the evaluation.On the basis of an echocardiography, the patients were divided into 2 subgroups: patients with elevated probability of PH (EP) (n = 16) versus the group with a low probability of PH (LP) (n = 67). Of the 16 patients in the EP group, 15 were SSc patients and 1 was an MCTD patient, respectively, that is, 21.7% and 7.1% of all patients. Of the 16 patients with EP, 10 with SSc had right-heart catheterization, which excluded PH in 7 patients; hence, PH was estimated to be 11.6% in the SSc group. The distribution of the individual causes of PH was arterial PH 2.9%, PH associated with interstitial lung disease 4.3%, PH associated with left ventricular disease 1.5%, and PH of unknown origin 2.9%. Further, there was a significant difference between EP and LP in the incidence of the right bundle branch block in standard electrocardiography, left atrial and right ventricular dimension, tricuspid annular plane systolic excursion, and Doppler-derived tricuspid lateral annular systolic velocity (S') in echocardiography.Echocardiography, particularly those evaluating the parameters included in the ESC/ERS guidelines of 2015, appears to be a useful tool in the detection of patients with a high PH probability. Additional tissue Doppler echocardiography seems to be a good option.