scholarly journals AB0461 “SCLERODERMA-LIKE” PATTERN AS A PRESENTING FEATURE OF CONNECTIVE TISSUE DISEASES

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1257.1-1257
Author(s):  
S. Lambova
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Rheumatic Diseases ◽  
Clinical Presentation ◽  
Connective Tissue Diseases ◽  
Final Conclusion ◽  
Pathological Feature ◽  
Microvascular Pathology

Background:The role of capillaroscopy for early diagnosis of systemic sclerosis (SSc) is well-known and pathological capillaroscopic pattern is a component of the new set of criteria for SSc (EULAR/ACR 2013). While it is also known that similar microvascular changes i.e., “scleroderma-like” could be observed in other rheumatic diseases i.e., undifferentiated connective tissue disease (UCTD), overlap syndromes, systemic lupus erythematosus (SLE), etc., the data about the time of their appearance in other rheumatic diseases different from SSc are scarce.Objectives:The aim of the study was to evaluate the prevalence of capillaroscopic features of microangiopathy in Raynaud’s phenomenon (RP) patients at the time of their first referral to rheumatology setting.Methods:22 in- and outpatients were included in the study that were referred for consultation in our rheumatology unit in the last 6 months. Inclusion criteria were presence of RP at their first consultation or still unclear diagnosis. Presence of known rheumatic disease diagnosed at previous consultation with rheumatologist as well as signs of definite diagnosis SSc were exclusion criteria. All the patients underwent capillaroscopic examination with USB capillaroscope Dinolite (magnification 200x). Routine laboratory tests were ordered i.e., complete blood count, ESR, CRP, biochemistry as well as immunological tests. ANA test was performed in all patients while antibodies against extractable nuclear antigens, antiphospholipid antibodies or other tests were ordered depending on the clinical presentation and overall context. The patients signed an informed consent for participation of the study.Results:12 of the examined patients were diagnosed with primary RP and their capillaroscopic examination revealed absence of microangiopathy i.e., normal pattern or non-specific changes (mainly dilated capillaries). In 7 patients the final diagnosis was UCTD and 4 of them exhibited microvascular pathology i.e. “scleroderma-like” pattern, while in 3 cases the capillaroscopic findings were non-specific. Among other patients 1 case was diagnosed with prescleroderma with “early” phase “scleroderma” pattern (according to definition of Cutolo et al., 2000 (1)), 1 case was with onset of SLE (“scleroderma-like” pattern, active phase) and in one case the microvascular pathology that included single giant capillary loop no other signs of connective tissue disease were found and the final conclusion was “suspected secondary” RP with necessity for a regular follow-up.Conclusion:In conclusion, definite features of microvascular pathology, known as “scleroderma-like” capillaroscopic pattern, could be observed as an initial pathological feature in CTD different from SSc such as UCTD and SLE and the overall diagnosis should be made in the overall context. Capillaroscopy inherits high significance in patients with UCTD, in whom clinical presentation could be obscure in the beginning and identification of microvascular capillaroscopic pathology is among the crucial signs to support the diagnosis. Future studies are necessary to delineate the role of microvascular pathology for prediction of future evolution of UCTD.References:[1]Cutolo M, Sulli A, Pizzorni C AS. Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol. 2000;27(1):155–60.Disclosure of Interests:None declared.

scholarly journals Role of Osteopontin as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis and other Connective Tissue Diseases (CTDs)

2021 ◽  
Vol 14 (5) ◽  
pp. 394
Author(s):  
Mattia Bellan ◽  
Cristina Piccinino ◽  
Stelvio Tonello ◽  
Rosalba Minisini ◽  
Ailia Giubertoni ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Connective Tissue Diseases ◽  
Cross Sectional ◽  
Potential Biomarker ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTD). Its early diagnosis is essential to start effective treatment. In the present paper, we aimed to evaluate the role of plasma osteopontin (OPN) as a candidate biomarker of PAH in a cohort of CTD patients. OPN is a pleiotropic protein involved in inflammation and fibrogenesis and, therefore, potentially promising in this specific clinical context. We performed a cross-sectional observational study on a cohort of 113 CTD patients (females N = 101, 89.4%) affected by systemic sclerosis N = 88 (77.9%), mixed connective tissue disease N = 10 (8.8%), overlap syndrome N = 10 (8.8%) or undifferentiated connective tissue disease N = 5 (4.4%). CTD-PAH patients showed significantly higher OPN plasma values than patients with CTD alone (241.0 (188.8–387.2) vs. 200.7 (133.5–281.6) ng/mL; p = 0.03). Although OPN levels were directly correlated with age and inversely with glomerular filtration rate, they remained associated with PAH at multivariate analysis. In conclusion, OPN was significantly associated with PAH among patients with CTD, suggesting it may have a role as a non-invasive disease biomarker of PAH.

Pulmonary Hypertension Complicating Connective Tissue Disease

2017 ◽  
Vol 38 (05) ◽  
pp. 619-635 ◽  
Author(s):  
Rajan Saggar ◽  
John Belperio ◽  
Elizabeth Volkmann ◽  
Augustine Chung ◽  
Joseph Lynch
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Pulmonary Complications ◽  
Clinical Markers ◽  
Major Focus ◽  
Specific Autoantibody ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) may complicate connective tissue disease (CTD; particularly systemic sclerosis [scleroderma]), and is associated with increased mortality. More than 70% of cases of PH complicating CTD occur in patients with systemic sclerosis (SSc), which is the major focus of this article. Pulmonary complications (i.e., interstitial lung disease [ILD] and PH) are the leading causes of SSc-related deaths. “Isolated” PH (i.e., without ILD) complicates SSc in 7.5 to 20% of cases; secondary PH may also occur in patients with SSc-associated ILD. Several clinical markers and specific autoantibody profiles have been associated with PH in SSc. The role of PH-specific therapy in improving CTD-PH outcomes is under investigation, as prognosis and responsiveness to therapy appear to be worse in SSc-associated PH compared with idiopathic pulmonary arterial hypertension. We discuss medical therapies for CTD-associated PH and the role of lung transplantation for patients who fail medical therapy.

scholarly journals Nailfold Capillaroscopy in Rheumatic Diseases

2020 ◽  
Author(s):  
Abhishek Patil ◽  
Isha Sood
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Early Diagnosis ◽  
Rheumatic Diseases ◽  
Connective Tissue Diseases ◽  
Nailfold Capillaroscopy ◽  
Indispensable Tool

Nailfold capillaroscopy (NFC) has developed into an indispensable tool for rheumatologists in the evaluation of rheumatic diseases. It offers various advantages in being rapid, noninvasive, and inexpensive. With NFC we are able to visualize the microcirculatory changes in the nail beds. These changes are key to the pathogenesis of connective tissue diseases such as systemic sclerosis. Hence NFC helps in early diagnosis of various connective tissue diseases. There is a lack of standardization in the techniques used and various capillary parameters studied, which could lead to variation in the reporting of the parameters studied. In this chapter we shall try to highlight the most common parameters studied in capillaroscopy and its utility in various connective tissue diseases.

scholarly journals SAT0582 RISK OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASES: A NATIONWIDE, POPULATION-BASED COHORT STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1250.1-1250
Author(s):  
H. H. Chen ◽  
K. H. Ng ◽  
W. C. Chao ◽  
C. H. Lin
Keyword(s):  
Cohort Study ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Rheumatic Diseases ◽  
Population Based ◽  
Autoimmune Rheumatic Diseases ◽  
Comparison Groups

Background:To date, very few studies had investigated the epidemiology of interstitial lung disease (ILD) among patients with systemic autoimmune rheumatic disease (SARD).Objectives:To study the risk of interstitial lung disease (ILD) among patients with various systemic autoimmune rheumatic diseases (SARDs) including rheumatoid arthritis (RA), dermatomyositis (DMtis), polymyositis (PM), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and primary Sjögren’s syndrome (pSS).Methods:Using 1997–2013 claims data from the Taiwanese National Health Insurance Research Database, we identified 63,277 newly diagnosed patients with various SARDs after excluding those with overlapping SARD diagnoses from 2001-2013 and randomly selected 253,108 non-SARD subjects matching (1:4) SARD patients for SARD diagnosis, age, sex and the year of the index date. We calculated the incidence rates (IRs) of ILD (ICD-9 code 515) in various SARD groups and the corresponding non-SARD comparison groups and estimated the IR ratios (IRRs) with 95% confidence intervals (CI) of ILD development. Using multivariable cox regression analyses, we estimated hazard ratios (HRs) with 95% CIs of ILD in various SARD groups compared with their comparison groups after adjusting for age, sex, Charlson comorbidity index, amiodarone use and methotrexate use. Sensitivity analyses were conducted by using a narrow definition of ILD.Results:As shown in Table 1, the IRs of ILD were greatest in SSc patients (2,523 per 105years), followed by patients with DMtis (2,463 per 105years), PM (1,956 per 105years), SS (601 per 105years), RA (279 per 105years), and SLE (276 per 105years). Multivariable analyses showed that the risks of ILD were significantly increased in patients with SSc (HR, 66.01; 95% CI, 32.73—133.13), DMtis (128.74, 95% CI, 40.19—412.47), PM (HR, 30.39; 95% CI, 11.24—82.15), pSS (HR, 8.76; 95% CI, 7.03—10.90), RA (HR, 4.22; 95% CI, 3.51—5.08), and SLE (HR, 13.98; 95% CI, 9.25—21.14).Conclusion:This nationwide, population-based, matched cohort study demonstrated that the risks of ILD were significantly increased in patients with SARDs.References:[1]Su R, Bennett M, Jacobs S, Hunter T, Bailey C, Krishnan E, et al. An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis. The Journal of rheumatology. 2011;38(4):693-701.[2]Hu Y, Wang LS, Wei YR, Du SS, Du YK, He X, et al. Clinical Characteristics of Connective Tissue Disease-Associated Interstitial Lung Disease in 1,044 Chinese Patients. Chest. 2016;149(1):201-8.Araki T, Putman RK, Hatabu H, Gao W, Dupuis J, Latourelle JC, et al. Development and Progression of Interstitial Lung Abnormalities in the Framingham Heart Study. Am J Respir Crit Care Med 2016;194:1514-1522.Disclosure of Interests:None declared

scholarly journals Relating Interferon Regulatory Factor 5 Rs2004640 Gene Polymorphism To Increased Risk Of Systemic Sclerosis In The Patients: Russian Federation Cohort

2020 ◽  
Vol 9 (4) ◽  
Author(s):  
Mikhail Yu. Krylov ◽  
Lidia P. Ananieva ◽  
Irina A. Guseva
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Gene Polymorphism ◽  
Connective Tissue Diseases ◽  
Interferon Regulatory Factor ◽  
Regulatory Factor ◽  
Type 1 Interferon ◽  
Interferon Regulatory Factor 5

Background ― A number of studies confirmed a crucial role of type 1 interferon in pathophysiology of connective tissue diseases. Interferon regulatory factors (IRF) coordinate an expression of type 1 interferon, while interferon regulatory factor 5 (IRF5) gene was recently identified as causing predisposition to systemic lupus erythematosus and Sjögren syndrome. The objective of our study was to identify possible association of IRF5 rs2004640 (G/T) single nucleotide polymorphism with systemic sclerosis (SSc). Material and Methods―The study involved 236 individuals, including 105 patients with SSc diagnosis and 131 control individuals from Moscow region. The latter were healthy, unrelated to each other, their genders and ages were matched to those of SSc patients. Allele-specific real-time polymerase chain reaction (PCR) was used to study IFR5 rs2004640 polymorphism. Results ― We detected significantly higher percentage of IRF5 T-allele carriers in all patients (59.5%), those with diffuse cutaneous SSc (67.3%), patients with interstitial lung lesions (62.3%), and those with positive titers of anti-topoisomerase I antibodies (66.3%), compared with control group (46.2%). The odds ratios (OR) were: 1.71 (р=0.00), 2.40 (р=0.0004), 1.93 (р=0.002), and 2.30 (р=0.0008), correspondingly. Conclusion ― The replacement of nucleotide G by T in the IRF5 rs2004640 gene polymorphism was associated with a predisposition to SSc. Our data implied an existence of a novel SSc pathogenetic pathway associated with important role of type 1 interferon in pathophysiology of connective tissue diseases.

scholarly journals P170 The validity and utility of the SLE-key® rule-out serological test when applied in a selected cohort of patients with SLE and other connective tissue diseases

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Sheilla Achieng ◽  
John A Reynolds ◽  
Ian N Bruce ◽  
Marwan Bukhari
Keyword(s):  
Linear Regression ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Negative Correlation ◽  
Connective Tissue Diseases ◽  
Inflammatory Myositis ◽  
The Mean ◽  
Negative Rule ◽  
Spearman’S Correlation ◽  
Rule Out

Abstract Background/Aims  We aimed to establish the validity of the SLE-key® rule-out test and analyse its utility in distinguishing systemic lupus erythematosus (SLE) from other autoimmune rheumatic connective tissue diseases. Methods  We used data from the Lupus Extended Autoimmune Phenotype (LEAP) study, which included a representative cross-sectional sample of patients with a variety of rheumatic connective tissue diseases, including SLE, mixed connective tissue disease (MCTD), inflammatory myositis, systemic sclerosis, primary Sjögren’s syndrome and undifferentiated connective tissue disease (UCTD). The modified 1997 ACR criteria were used to classify patients with SLE. Banked serum samples were sent to Immune-Array’s CLIA- certified laboratory Veracis (Richmond, VA) for testing. Patients were assigned test scores between 0 and 1 where a score of 0 was considered a negative rule-out test (i.e. SLE cannot be excluded) whilst a score of 1 was assigned for a positive rule-out test (i.e. SLE excluded). Performance measures were used to assess the test’s validity and measures of association determined using linear regression and Spearman’s correlation. Results  Our study included a total of 155 patients of whom 66 had SLE. The mean age in the SLE group was 44.2 years (SD 13.04). 146 patients (94.1%) were female. 84 (54.2%) patients from the entire cohort had ACR SLE scores of ≤ 3 whilst 71 (45.8%) had ACR SLE scores ≥ 4. The mean ACR SLE total score for the SLE patients was 4.85 (SD 1.67), ranging from 2 to 8, with mean disease duration of 12.9 years. The Sensitivity of the SLE-Key® Rule-Out test in diagnosing SLE from other connective tissue diseases was 54.5%, specificity was 44.9%, PPV 42.4% and NPV 57.1 %. 45% of the SLE patients had a positive rule-out test. SLE could not be ruled out in 73% of the MCTD patients whilst 51% of the UCTD patients had a positive Rule-Out test and >85% of the inflammatory myositis patients had a negative rule-out test. ROC analysis generated an AUC of 0.525 illustrating weak class separation capacity. Linear regression established a negative correlation between the SLE-key Rule-Out score and ACR SLE total scores. Spearman’s correlation was run to determine the relationship between ACR SLE total scores and SLE-key rule-out score and showed very weak negative correlation (rs = -0.0815, n = 155, p = 0.313). Conclusion  Our findings demonstrate that when applied in clinical practice in a rheumatology CTD clinic setting, the SLE-key® rule-out test does not accurately distinguish SLE from other CTDs. The development of a robust test that could achieve this would be pivotal. It is however important to highlight that the test was designed to distinguish healthy subjects from SLE patients and not for the purpose of differentiating SLE from other connective tissue diseases. Disclosure  S. Achieng: None. J.A. Reynolds: None. I.N. Bruce: Other; I.N.B is a National Institute for Health Research (NIHR) Senior Investigator and is funded by the NIHR Manchester Biomedical Research Centre. M. Bukhari: None.

scholarly journals AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
High Resolution Computed Tomography ◽  
Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

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