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 Thermal Disparity among Fingers and Its Amelioration by CO2-Water Bathing in Connective Tissue Disease Patients

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Shigeko Inokuma ◽  
Yasuo Kijima
Keyword(s):  
Carbon Dioxide ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Negative Correlation ◽  
Coefficient Of Variation ◽  
Peripheral Circulation ◽  
Baseline Measurement ◽  
Finger Temperature ◽  
The Mean ◽  
Carbon Dioxide Co2

Objective. Correlation between a low finger temperature and thermal disparity among fingers was studied in connective tissue disease (CTD) patients. Whether the thermal disparity may be ameliorated by hand immersion in a warm carbon dioxide- (CO2-) water bath was analyzed. Methods. CTD patients with suspected peripheral circulation disorder underwent a thermography test. From before to 30 min after hand immersion in CO2-water (CO2 bathing; 1000 ppm CO2, 42°C, 10 min), the nailfold temperatures were measured. The mean temperature (m-Temp) and the coefficient of variation of the temperature ( CV = SD / m ‐ Temp of one hand; the mean of CVs of both hands was adopted as the indicator of thermal disparity) were monitored. The correlation between m-Temp and CV was also analyzed. Results. Forty-seven (45 females and 2 males) patients were included, 32 of whom had Raynaud’s phenomenon. The m-Temp was 30.8 ± 3.0 ° C at the baseline, increased to 35.3 ± 1.0 ° C immediately after CO2 bathing, and remained significantly higher than that at the baseline until 30 min after ( 32.1 ± 1.9 ° C ). The CV was 0.0291 ± 0.0247 at the baseline, decreased to 0.0135 ± 0.0039 immediately after CO2 bathing, and remained significantly lower than the baseline until 30 min after ( 0.0163 ± 0.0143 ). Between m-Temp and CV, a negative correlation was observed throughout the measurements. Conclusion. Thermal disparity was observed at baseline measurement in CTD patients. Warm CO2 bathing markedly ameliorated the disparity, and this amelioration remained until after 30 min. Throughout the observation, the lower the m-Temp, the more severe the thermal disparity among fingers.

Effect of Cotreatment with Corticosteroids in Connective Tissue Related Lung Disease (CTD-ILD) Patients on Mycophenolate Mofetil (MMF)

2021 ◽  
Author(s):  
Roberto Caricchio ◽  
Erin R Narewski ◽  
Ryan Townsend ◽  
Stephen Codella ◽  
Jin Sun Kim ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Pulmonary Function Tests ◽  
Usual Interstitial Pneumonia ◽  
University Hospital ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Abstract Introduction: Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD) is often treated with immunosuppressant medications; common among these is Mycophenolate Mofetil (MMF). We hypothesized that co-treatment with corticosteroids would impact disease progression.Methods: We examined a consecutive cohort of CTD-ILD patients followed at Temple University Hospital in Philadelphia, PA since 2015 who had pulmonary function tests (PFTs) performed by American Thoracic Society (ATS)/European Respiratory Society (ERS) Criteria at least one year apart. All patients were treated for CTD-ILD with MMF used either as sole therapy or as combination therapy with prednisone. Univariate logistic analyses were performed revealing the odds ratio (OR) for improvement or worsening of several PFT values (including forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), and six-minute walk (6MW)) greater than the minimal clinically important difference (MCID) for each value.Results: We included 103 patients (74 women) with an average age of 60 ± 11 years, 49% of our cohort were current or former smokers, and mean BMI was 29 ± 7 kg/m2. Patients were observed on treatment for an average of 23 months. CTD distribution included 25% mixed connective tissue disease (MCTD), 24% systemic sclerosis (SSc), 17% rheumatoid arthritis (RA), 14% systemic lupus erythematosus (SLE), 10% other idiopathic inflammatory myositis (IIM) syndromes, 7% Antisynthetase Syndrome, 5% Sjӧgren’s syndrome. Non-specific interstitial pneumonia (NSIP) was the majority (45%) ILD pattern noted, Usual Interstitial Pneumonia (UIP) 35%, and other types were less prevalent (20%). The majority of patients received corticosteroids as co-treatment with MMF (75 patients (72%)) with a mean daily dose of 15 ± 16 mg of prednisone. Mean daily MMF dose was 1144 ± 675 mg. Glucocorticoid treatment was not associated with significant improvements in PFT values, including FVC, DLCO, and 6MW distance walked.Conclusion: In this small cohort, patients with CTD-ILD receiving MMF did not demonstrate improved lung function when receiving co-treatment with corticosteroids, but larger prospective studies are needed to better elucidate the effect of corticosteroids on this vulnerable group of patients.

Mixed Connective Tissue Disease- Physician’s Dilemma: A case report

2021 ◽  
Vol 11 (Number 1) ◽  
pp. 60-65
Author(s):  
Abu Saleh Shimon ◽  
Mahjuba Umme Salam ◽  
Monharul Islam Bhuiyan ◽  
Mashuq Ahmad Jumma ◽  
Imran Hussain ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
High Titer ◽  
Connective Tissue Diseases ◽  
Systemic Lupus ◽  
Serological Tests ◽  
Normotensive Woman ◽  
New Onset

Mixed connective tissue disease is an entity of autoimmune disease with overlapping features of systemic lupus erythematosus, scleroderma, rheumatoid arthritis, dermatomyositis and with positive anti-U1 RNP antibody. We report here a 52 year old non-diabetic, normotensive woman presenting with new onset dysphagia for two months with variable features of multiple types of connective tissue diseases for two years. Clinical features and type specific serological tests for different connective tissue diseases showed puzzling results. However, finally a high titer of anti-U1RNP antibody led to the diagnosis of mixed connective tissue disease.

Connective tissue disease

2019 ◽  
pp. 281-326
Author(s):  
Gavin Spickett
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Raynaud’S Phenomenon ◽  
Connective Tissue Diseases ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of connective tissue diseases. It covers a range of rheumatic disorders, from rheumatoid arthritis to Raynaud’s phenomenon, and also covers the undifferentiated diseases, overlap syndromes, and mixed connective tissue disease.

scholarly journals Detection of Specific Antinuclear Reactivities in Patients with Negative Anti-nuclear Antibody Immunofluorescence Screening Tests

2002 ◽  
Vol 48 (12) ◽  
pp. 2171-2176 ◽  
Author(s):  
Ilse EA Hoffman ◽  
Isabelle Peene ◽  
Eric M Veys ◽  
Filip De Keyser
Keyword(s):  
Connective Tissue ◽  
Confidence Interval ◽  
Connective Tissue Disease ◽  
Indirect Immunofluorescence ◽  
Connective Tissue Diseases ◽  
Clinical Suspicion ◽  
Screening Tests ◽  
Strongly Correlated ◽  
Nuclear Antigens

Abstract Background: For detection of anti-nuclear antibodies (ANAs) and antibodies to extractable nuclear antigens (ENAs), samples frequently are screened with indirect immunofluorescence (IIF); further determination of anti-ENA antibodies is performed only when the result is positive. However, because anti-ENA reactivities are found in samples with low fluorescence intensities, we determined anti-ENA antibodies in samples with negative IIF and thus calculated the sensitivity of IIF for specific ANAs. Methods: We collected 494 samples consecutively referred by rheumatologists for routine ANA testing. IIF on HEp-2 and HEp-2000 (HEp-2 cells transfected with Ro60 cDNA) and line immunoassay (LIA) for the detection of specific ANAs were performed on all samples. Results: Fluorescence intensities and patterns on HEp-2 were strongly correlated with those on HEp-2000 [Spearman ρ = 0.852 (P <0.001) and 0.838 (P <0.001), respectively]. Sixty-eight of 494 samples were positive on LIA, of which only 72% (confidence interval, 68–76%) were detected with HEp-2 and 75% (confidence interval, 70–78%) with HEp-2000. Of 291 samples negative on both substrates, 12 were positive on LIA. Connective tissue diseases were diagnosed in four of these patients and suspected in at least three others. Conclusion: The HEp-2 and HEp-2000 substrates perform comparably for fluorescence intensities and patterns and for detecting specific ANAs, but some patients with negative IIF show reactivity on LIA. We recommend testing for fine reactivities, regardless of the IIF result, when the clinical suspicion for rheumatic connective tissue disease is high.

Chemical prediction of water activity in processed cheese

1989 ◽  
Vol 56 (4) ◽  
pp. 665-668 ◽  
Author(s):  
María A. Esteban ◽  
Andrés Marcos
Keyword(s):  
Regression Analysis ◽  
Linear Regression ◽  
Water Activity ◽  
Negative Correlation ◽  
Linear Regression Analysis ◽  
Significant Negative Correlation ◽  
Regression Equation ◽  
Low Fat ◽  
Two Samples ◽  
The Mean

SummaryBy linear regression analysis, a highly significant negative correlation (r = −0·96) was found between the mean ash concentration values (g/100 g moisture) and water activity (aw) of six types of processed cheeses (low-fat, semi-fat, fat, extra-fat, double fat and special). The regression equation aw = 0·9951 − 0·0032* (ash), applied to 40 cheese samples, yielded aw values which differed by < 0·005 aw units from those measured experimentally in 75% of the samples. The maximum differences between the calculated and experimental aw values (found in only two samples) were ±0·01 aw units.

Tuberculosis heralding connective tissue disorder

2020 ◽  
pp. 004947552096274
Author(s):  
U Pratap ◽  
M Ravindrachari ◽  
A RamyaPriya ◽  
Pampa Ch. Toi ◽  
R Manju
Keyword(s):  
Weight Loss ◽  
Mycobacterium Tuberculosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lung Biopsy ◽  
Connective Tissue Diseases ◽  
Connective Tissue Disorder ◽  
Current Smoker ◽  
Tuberculosis Therapy ◽  
A Current

Connective tissue diseases and infections are amongst the causes for organising pneumonia. However, organising pneumonia preceding other connective tissue disease manifestations is rare. Mycobacterium tuberculosis is rarely associated with organising pneumonia. We report such a case. A 50-year-old diabetic male, a roadside shop keeper, a current smoker presented with fever, breathlessness, cough and weight loss for four months. Chest radiography demonstrated areas of consolidation with halo signs. Anti-nuclear antibody blot was positive for Scl-70 and Jo-1 suggestive of a syndrome of systemic sclerosis and polymyositis overlap. Fibre-optic bronchoscopy guided lung biopsy was suggestive of organising pneumonia, and broncho-alveolar lavage detected Mycobacterium tuberculosis. Mycobacterium tuberculosis should be investigated as an aetiology of organising pneumonia, as this may occur in unestablished cases of connective tissue disease even before clinical and radiological manifestations appear, as response can be achieved with anti-tuberculosis therapy alone, without additional use of systemic steroids.

scholarly journals Undifferentiated connective tissue disease: state of the art on clinical practice guidelines

RMD Open ◽  
2019 ◽  
Vol 4 (Suppl 1) ◽  
pp. e000786 ◽  
Author(s):  
Margarida Antunes ◽  
Carlo Alberto Scirè ◽  
Rosaria Talarico ◽  
Tobias Alexander ◽  
Tadej Avcin ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Practice Guidelines ◽  
Practice Guidelines ◽  
Connective Tissue Diseases ◽  
Preventive Measure ◽  
Undifferentiated Connective Tissue Disease ◽  
Consensus Definition ◽  
Severe Condition

The term ‘undifferentiated connective tissue disease’ (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations.No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition.Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.

scholarly journals Cutaneous mucinosis in mixed connective tissue disease*

2013 ◽  
Vol 88 (4) ◽  
pp. 635-638 ◽  
Author(s):  
Maria Helena Sampaio Favarato ◽  
Sofia Silveira de Castro Miranda ◽  
Maria Teresa Correia Caleiro ◽  
Ana Paula Luppino Assad ◽  
Ilana Halpern ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Clinical Response ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Connective Tissue Diseases ◽  
Skin Lesions ◽  
The Other

Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

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