scholarly journals POS0323 ANTI PM-SCL ASSOCIATED AUTO IMMUNE DISEASES: MULTICENTRIC COHORT OF 128 PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 388.1-389
Author(s):  
P. Breillat ◽  
K. Mariampillai ◽  
P. Martins ◽  
P. Legendre ◽  
B. Dunogue ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Unknown Etiology ◽  
Scleroderma Renal Crisis ◽  
Raynaud Phenomenon ◽  
Muscle Involvement ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Frequent Relapses

Background:Autoantibodies permit to classify and subgroup connective tissue diseases (CTD) in homogeneous groups of patients in terms of phenotype and prognosis. Anti PM-Scl antibodies have been associated with different CTD categories such as: idiopathic inflammatory myositis (IIM), systemic sclerosis (SSc), Sjögren’s syndrome (SjS), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) or undifferentiated connective tissue disease (UCTD).Objectives:To determine clinical spectrum of anti-PM-Scl associated disease and if it an homogenous condition.Methods:This multicentric (four hospitals) observational and retrospective study included all consecutive patients with positive testing for anti PM-Scl antibodies on immunoblot assay and connective tissue disease (2011 -2020). Epidemiological, biological, clinical and radiological data were collected in standard form as well as patient’s outcome.Results:One hundred twenty height patients (female n=96;75%) were included. Median [quartiles] age at diagnosis was 50 [18;84] (IQR) and follow-up duration of 7 [3.75-12] years. Seventy-six (59.3%) patients were simple anti-Pm-Scl positive, and 40.7% were associated with other antibodies: anti-SSA/Ro52 (n=13; 10.92%), SSc associated antibodies (n=21; 16.4%), anti-dsDNA for (n=9; 7%), anti-RNP (n=6; 4.7%) and anti-CCP antibodies (n=6; 4.7%). Most patients had cutaneous involvement (n=106; 83%) with skin thickening (n=47; 36%), mechanics hands (n= 28; 22%), calcinosis (n=26; 20.3%) and subcutaneous edema (n=20; 15.62%). Vascular involvement was frequent with Raynaud phenomenon (n= 89; 69%), telangiectasia (n=36; 28%), skin ulcers (n=27; 21%), pulmonary hypertension (n=8/120; 6.7%) and scleroderma renal crisis (n=2; 1.5%). A majority of patients also displayed an interstitial lung disease (ILD) (n=83; 65.8%); nonspecific interstitial pneumonia (92.7%) and/or organizing pneumonia (25.3%). ILD was characterized by a subacute onset in 37/81 (45.7%); median [quartiles] forced vital capacity (FVC) and total lung capacity (TLC) at diagnosis of 88% [73-105] and 79.5% [68.5-101] respectively. Sixty patients (47%) had muscular sign including myalgia (47%), elevated CPK (n=51; 40%) and muscular weakness (Medical Research Council score <4) (n=19/124;15%). Finally, fifty-three (41.7%) had gastroesophageal reflux. Thirty-nine patients (30.4%) experienced at least one muscular or ILD relapse and 6 (4.84%) died during follow-up (2 breast cancer, 1 pneumonia, 3 unknown etiology). Concerning patients’ prognosis, relapses were associated with skeletal (n=29, 74.4% vs n=32, 35.96%, p < 0.001) or cardiac muscle involvement (n=7, 18.4% vs n=2, 2.5%, p=0.007), and subacute ILD (n=19, 65.5% vs n=18, 34.62%, p= 0.05) with organized pneumonia pattern (n=11, 32.3% vs n=10, 13.9%, p=0.05). Strikingly, ILD occurred mainly in men (90.6% vs 57.2%, p < 0.001) and was associated with anti-Scl-70 positivity (n=14, 16.67% vs 0%, p= 0.01). Muscle involvement was associated arthralgia (n=46, 76.67% vs n=34, 50.75%, p=0.005), respiratory signs at diagnosis: dyspnea NYHA ≥3 (n=46, 75.41% vs n=30, 44.78%, p < 0.001), sub-acute ILD (n=24, 61.54% vs n=13, 30.95%, p=0.0111) with lower FVC (73% [64;88] vs 98 [76;105], p < 0.001). Ulcers were associated with Anti-Scl70 positivity (n=9, 33.33% vs n=5, 4.95%, p < 0.001), Raynaud phenomenon (n=27, 100% vs n=62, 61.39%, p < 0.001), digestive involvement (n=20, 74.07% vs n=34, 33.66%, p < 0.001), ILD with chronic onset (n=15, 78.95% vs n=29, 46.77%, p=0.027) and increased incidence of deaths (n=4, 16% vs n=2, 2.02%, p= 0.01).Conclusion:Conducted on the largest cohort of Anti-PM-Scl patients, this study highlights two main phenotypes that determine different outcome and prognosis. One was associated with muscular disease and subacute onset ILD with more frequent relapses. The second with a vascular phenotype associated with chronic ILD, digestive involvement, chronic evolution and increased incidence of death. This could lead to a reclassification of PM-Scl associated auto immune diseases.Disclosure of Interests:None declared

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.

scholarly journals AB0623 RATE AND PREDICTIVE FACTORS ASSOCIATED WITH SUSTAINED REMISSION IN IDIOPATHIC INFLAMMATORY MYOSITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1607.1-1607
Author(s):  
T. Vojinovic ◽  
X. Gjoni ◽  
M. Fredi ◽  
F. Franceschini ◽  
I. Cavazzana
Keyword(s):  
Disease Activity ◽  
Clinical Features ◽  
Predictive Factors ◽  
Sustained Remission ◽  
Muscle Involvement ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Younger Age ◽  
Drug Free

Background:Idiopathic inflammatory myositis (IIM) is a group of heterogeneous connective tissue diseases, characterised by chronic muscle inflammation, myositis-specific or myositis-associated autoantibodies and different extra-muscular features. Achieveing low disease activity or remission in patients with IIM has proven to be difficult due to the wide clinical spectrum of the different IIM types.Objectives:To retrospectively assess any predictive factors for sustained remission in IIM patientsMethods:We retrospectively analyzed data taken from medical charts, which included age at disease onset, gender, laboratory data as well as clinical features present at onset, organ involvement and treatment history. A total of 151 adult patients with IIM followed-up for > 1 year were retrospectively enrolled. Remission was defined as no clinical and laboratory evidence of disease activity persistent for more than 6 months during follow-up, while undergoing myositis therapy or under no medication. The remission of cutaneous involvement was defined as no current activity of skin rash, absence of Gottron’s papules as well as heliotrope rash and erythema, whereas the remission of pulmonary involvement was considered as no requirement for intensification of immunosuppressive therapy during follow-up. Likewise, absence of muscle weakness or hyposthenia was taken into account for evaluating muscle involvement. Moreover, the clinical features were accompanied by normalization of myogenic enzymes such as creatine kinase (CK) and lactate dehydrogenase levels.Results:Among all 151 patients, 89 (58.9%) patients achieved sustained remission. By univariate analysis, overlap myositis (7.9% vs 27.4%; p=0.003; OR:0.22), cancer-associated myositis (CAM) (7.8% vs 19.35%; p=0.046; OR:0.3), as well as the presence of anti Ku (3.37% vs 12.9%; p=0.05; OR:0.23) and anti TIF-1 gamma (1.1% vs 8%; p=0.043; OR:0.13) antibodies and polyarthritis (11.2% vs 24.19%; p=0.045; OR: 0.397) at onset were significantly associated with active IIM, not achieving remission.Out of 89 patients in remission, 79 (88.8%) achieved long-term sustained remission, lasting at least 2 years, whereas 10 patients (11.2%) showed relapse. Most of relapsed patients showed a relapse/remitting disease, with no evident trigger for the relapse. We divided our cohort of 89 patients in remission in 2 subsets: 83 patients in remission undergoing therapy (93.3%) and 6 patients in drug-free remission (6.7%). No significant differences were found between two groups, except for younger age at onset (p=0.0002) found in patients achieving drug-free remission.Conclusion:Sustained remission occurs in about one half of patients with IIM. The presence of anti Ku and anti TIF-1 gamma antibodies as well as polyarthritis at onset lowers the chance of achieving sustained remission. Younger age at diagnosis has proved to predict drug-free long-lasting remission.Disclosure of Interests:None declared

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 &gt;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 Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

2010 ◽  
Vol 17 (6) ◽  
pp. 282-286 ◽  
Author(s):  
Shikha Mittoo ◽  
Thomas Jacob ◽  
Andrea Craig ◽  
Zoheir Bshouty
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Term Treatment ◽  
Kaplan Meier ◽  
Long Term Treatment ◽  
Survival Differences

BACKGROUND: Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.OBJECTIVE: To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.METHODS: A retrospective evaluation of 13 CTD-PH-ILD patients who were treated with bosentan, sildenafil or bosentan plus sildenafil, was conducted. Immunosuppressants were prescribed as indicated. Patients underwent pulmonary function testing and assessment of 6 min walk distance at the time of treatment initiation and during follow-up. Patients were followed until time of death, lung transplantation or the end of the study. Kaplan-Meier estimates of survival were calculated and log-rank testing was used to analyze survival differences according to CTD subtype.RESULTS: Thirteen patients (seven with systemic sclerosis [SSc], four with overlap syndrome, and two with rheumatoid arthritis) were followed for a mean (± SD) duration of 33.8±21.7 months. The survival estimate at a median duration of 34 months was 85%; two patients with SSc died. Mortality rates were greater among patients with SSc versus other CTD subtypes (P=0.04). No changes from baseline to follow-up in mean forced vital capacity or exercise capacity, and no treatment-related toxicity, were observed.CONCLUSION: Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.

scholarly journals Impaired endothelial function in patients with undifferentiated connective tissue disease: a follow-up study

Rheumatology ◽  
2014 ◽  
Vol 53 (11) ◽  
pp. 2035-2043 ◽  
Author(s):  
R. Laczik ◽  
P. Soltesz ◽  
P. Szodoray ◽  
Z. Szekanecz ◽  
G. Kerekes ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Endothelial Function ◽  
Connective Tissue Disease ◽  
Undifferentiated Connective Tissue Disease ◽  
Follow Up Study

scholarly journals Cardiac Catheterization versus Echocardiography for Monitoring Pulmonary Pressure: A Prospective Study in Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

Diagnostics ◽  
2020 ◽  
Vol 10 (1) ◽  
pp. 49
Author(s):  
Vasiliki Kalliopi Bournia ◽  
Iraklis Tsangaris ◽  
Loukianos Rallidis ◽  
Dimitrios Konstantonis ◽  
Frantzeska Frantzeskaki ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Negative Predictive Value ◽  
Connective Tissue Disease ◽  
Cardiac Catheterization ◽  
Predictive Value ◽  
Hemodynamic Deterioration ◽  
Pulmonary Arterial

Standard echocardiography is important for pulmonary arterial hypertension (PAH) screening in patients with connective tissue disease (CTD), but PAH diagnosis and monitoring require cardiac catheterization. Herein, using cardiac catheterization as reference, we tested the hypothesis that follow-up echocardiography is adequate for clinical decision-making in these patients. We prospectively studied 69 consecutive patients with CTD-associated PAH. Invasive baseline pulmonary artery systolic pressure (PASP) was 60.19 ± 16.33 mmHg (mean ± SD) and pulmonary vascular resistance (PVR) was 6.44 ± 2.95WU. All patients underwent hemodynamic and echocardiographic follow-up after 9.47 ± 7.29 months; 27 patients had a third follow-up after 17.2 ± 7.4 months from baseline. We examined whether clinically meaningful hemodynamic deterioration of follow-up catheterization-derived PASP (i.e., > 10% increase) could be predicted by simultaneous echocardiography. Echocardiography predicted hemodynamic PASP deterioration with 59% sensitivity, 85% specificity, and 63/83% positive/negative predictive value, respectively. In multivariate analysis, successful echocardiographic prediction correlated only with higher PVR in previous catheterization (p = 0.05, OR = 1.235). Notably, in patients having baseline PVR > 5.45 WU, echocardiography had both sensitivity and positive predictive values of 73%, and both specificity and negative predictive value of 91% for detecting hemodynamic PASP deterioration. In selected patients with CTD-PAH echocardiography can predict PASP deterioration with high specificity and negative predictive value. Additional prospective studies are needed to confirm that better patient selection can increase the ability of standard echocardiography to replace repeat catheterization.

scholarly journals Long-term follow-up of patients with anti-cyclic citrullinated peptide antibody-positive connective tissue disease: a retrospective observational study including information on the HLA-DRB1 allele and citrullination dependency

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Takeshi Iwasaki ◽  
Shuichiro Nakabo ◽  
Chikashi Terao ◽  
Kosaku Murakami ◽  
Ran Nakashima ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
X Rays ◽  
Bone Erosions ◽  
Cyclic Citrullinated Peptide ◽  
Drb1 Allele ◽  
Antibody Titers ◽  
Hands And Feet ◽  
Citrullinated Peptide

Abstract Background The anti-cyclic citrullinated peptide (CCP) antibody is a diagnostic biomarker of rheumatoid arthritis (RA). However, some non-RA connective tissue disease (CTD) patients also test positive for the anti-CCP antibody and, thus, may ultimately develop RA. We retrospectively investigated whether anti-CCP-positive non-RA CTD patients developed RA and attempted to identify factors that may differentiate RA-overlapping CTD from pure CTD. Methods In total, 842 CTD patients with a primary diagnosis that was not RA were selected from our CTD database as of December 2012. Anti-CCP antibody titers were obtained from a retrospective chart review or measured using stored sera. RA was diagnosed according to the 1987 revised American College of Rheumatology classification criteria. Thirty-three anti-CCP-positive non-RA CTD patients were retrospectively followed up for the development of RA. Bone erosions on the hands and feet were assessed by X-ray. Citrullination dependency was evaluated by an in-house ELISA, the HLA-DRB1 allele was typed, and the results obtained were then compared between RA-overlapping and non-RA anti-CCP-positive CTD patients. Results Two out of 33 anti-CCP-positive CTD patients (6.1%) developed RA during a mean follow-up period of 8.9 years. X-rays were examined in 27 out of the 33 patients, and only one (3.7%) showed bone erosions. The frequency of the HLA-DRB1 shared epitope (SE) and anti-CCP antibody titers were both significantly higher in anti-CCP-positive RA-overlapping CTD patients than in anti-CCP-positive non-RA CTD patients, while no significant differences were observed in citrullination dependency. Conclusions Anti-CCP-positive non-RA CTD patients rarely developed RA. HLA-DRB1 SE and anti-CCP antibody titers may facilitate the differentiation of RA-overlapping CTD from anti-CCP-positive non-RA CTD.

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