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Ingrid E Lundberg - One of the best experts on this subject based on the ideXlab platform.
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response to comment on idiopathic inflammatory myopathies and Antisynthetase Syndrome contribution of Antisynthetase antibodies to improve current classification criteria by greco et al by knitza et al
Annals of the Rheumatic Diseases, 2020Co-Authors: Anna Tjarnlund, Ingrid E LundbergAbstract:We have with great interest read the letter entitled ‘Response to: ‘Idiopathic inflammatory myopathies and Antisynthetase Syndrome: contribution of Antisynthetase antibodies to improve current classification criteria’ by Greco et al ’ by Knitza et al , to be published in the Annals of the Rheumatic Diseases .1 We appreciate the thoughtful comments made by the authors and agree on the importance of aminoacyl transfer RNA synthetase (ARS) autoantibodies, as well as other autoantibodies for correct classification of the associated diseases. The discussion stems from the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) …
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anti pl 7 anti threonyl trna synthetase Antisynthetase Syndrome clinical manifestations in a series of patients from a european multicenter study eumyonet and review of the literature
Medicine, 2012Co-Authors: Ane Labiruaiturburu, Albert Selvaocallaghan, Melinda Vincze, Katalin Danko, J Vencovsky, Benjamin A Fisher, P Charles, Maryam Dastmalchi, Ingrid E LundbergAbstract:Autoantibodies against several aminoacyl-transfer-RNA synthetases have been described in patients with myositis; anti-threonyl-tRNA synthetase (anti-PL-7) is one of the rarest. We describe the clinical and laboratory characteristics of a cohort of European anti-PL-7 patients, and compare them with previously reported cases. This multicenter study of patients positive for anti-PL-7, identified between 1984 and 2011, derives from the EUMYONET cohort. Clinical and serologic data were obtained by retrospective laboratory and medical record review, and statistical analyses were performed with chi-squared and Fisher exact tests. Eighteen patients, 15 women, were anti-PL-7 antibody positive. Median follow-up was 5.25 years (interquartile range, 2.8-10.7 yr), and 4 patients died. All patients had myositis (12 polymyositis, 5 dermatomyositis, and 1 amyopathic dermatomyositis), 10 (55.6%) had interstitial lung disease, and 9 (50%) had pericardial effusion. Occupational exposure to organic/inorganic particles was more frequent in patients with interstitial lung disease than in the remaining patients (5 of 10 vs. 1 of 7; p = 0.152), although the difference was not significant. Concurrent autoantibodies against Ro60 and Ro52 were seen in 8 of 14 (57%) patients studied. In the literature review the most common manifestations of anti-PL-7 Antisynthetase Syndrome were interstitial lung disease (77%), myositis (75%), and arthritis (56%). As in other subsets of the Antisynthetase Syndrome, myositis and interstitial lung disease are common features of the anti-PL-7 Antisynthetase Syndrome. In addition, we can add pericarditis as a possible manifestation related to anti-PL-7 antibodies.
Samuel Katsuyuki Shinjo - One of the best experts on this subject based on the ideXlab platform.
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high ykl 40 serum levels and its expression in the muscle tissues of patients with Antisynthetase Syndrome
Advances in rheumatology (London England), 2021Co-Authors: Renata Casseb De Souza Carboni, Gustavo Luiz Behrens Pinto, Samuel Katsuyuki ShinjoAbstract:The protein chitinase-3-like-1 (YKL-40) is rarely analyzed in patients with myositis. Therefore, we aimed to evaluate YKL-40 serum levels; correlate them with laboratory and clinical parameters, disease status, and treatment schemes; and analyze the YKL-40 expression in the muscle tissues of patients with Antisynthetase Syndrome (ASSD). This cross-sectional single-center study included 64 adult patients with ASSD who were age-, gender-, and ethnicity-matched to 64 healthy control individuals. Their YKL-40 serum levels were analyzed using the Enzyme-Linked Immunosorbent Assay (ELISA) kit method, while YKL-40 expression in muscle tissues was analyzed using an immunohistochemical technique. Disease status was assessed using the International Myositis Assessment and Clinical Studies Group (IMACS) set scores. The patients’ mean age was 44.8 ± 11.8 years, and median disease duration was 1.5 (0.0–4.0) years. These patients were predominantly female (82.8%) and Caucasian (73.4%). Most patients had stable disease. The median YKL-40 serum level was significantly higher in patients with ASSD when compared to the healthy individuals: 538.4 (363.4–853.1) pg/mL versus 270.0 (201.8–451.9) pg/mL, respectively; P 0.050), except tumor necrosis factor alpha (TNF-α) serum levels (Spearman’s correlation, rho = 0.382; P = 0.007). YKL-40 was highly expressed by inflammatory cells found in muscle biopsy specimens. High YKL-40 serum levels were observed in patients with ASSD and correlated positively with TNF-α serum levels. Moreover, YKL-40 was expressed by the inflammatory cells of the muscle tissue.
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High YKL-40 serum levels and its expression in the muscle tissues of patients with Antisynthetase Syndrome
'Springer Science and Business Media LLC', 2021Co-Authors: Renata Casseb De Souza Carboni, Gustavo Luiz Behrens Pinto, Samuel Katsuyuki ShinjoAbstract:Abstract Background The protein chitinase-3-like-1 (YKL-40) is rarely analyzed in patients with myositis. Therefore, we aimed to evaluate YKL-40 serum levels; correlate them with laboratory and clinical parameters, disease status, and treatment schemes; and analyze the YKL-40 expression in the muscle tissues of patients with Antisynthetase Syndrome (ASSD). Methods This cross-sectional single-center study included 64 adult patients with ASSD who were age-, gender-, and ethnicity-matched to 64 healthy control individuals. Their YKL-40 serum levels were analyzed using the Enzyme-Linked Immunosorbent Assay (ELISA) kit method, while YKL-40 expression in muscle tissues was analyzed using an immunohistochemical technique. Disease status was assessed using the International Myositis Assessment and Clinical Studies Group (IMACS) set scores. Results The patients’ mean age was 44.8 ± 11.8 years, and median disease duration was 1.5 (0.0–4.0) years. These patients were predominantly female (82.8%) and Caucasian (73.4%). Most patients had stable disease. The median YKL-40 serum level was significantly higher in patients with ASSD when compared to the healthy individuals: 538.4 (363.4–853.1) pg/mL versus 270.0 (201.8–451.9) pg/mL, respectively; P 0.050), except tumor necrosis factor alpha (TNF-α) serum levels (Spearman’s correlation, rho = 0.382; P = 0.007). YKL-40 was highly expressed by inflammatory cells found in muscle biopsy specimens. Conclusions High YKL-40 serum levels were observed in patients with ASSD and correlated positively with TNF-α serum levels. Moreover, YKL-40 was expressed by the inflammatory cells of the muscle tissue
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the aerobic capacity in patients with Antisynthetase Syndrome and dermatomyositis
Advances in rheumatology (London England), 2020Co-Authors: Alexandre Moura Dos Santos, Rafael Giovani Misse, Isabela Bruna Pires Borges, Samuel Katsuyuki ShinjoAbstract:This study was aimed at evaluating the aerobic capacity of patients with Antisynthetase Syndrome (ASS) and dermatomyositis (DM) and analyzing possible relationships between aerobic capacity and disease status, cardiovascular diseases and their risk factors. The study was a cross-sectional, single-center study that assessed the aerobic capacity of 22 women (13 with DM and 9 with ASS) who were matched by age and body mass index to 17 healthy women (control group). The aerobic capacity (oxygen uptake [VO2 peak], anaerobic threshold, respiratory compensation point and time-to-exhaustion) was evaluated using the cardiopulmonary treadmill test. Disease status was assessed using International Myositis Assessment & Clinical Studies Group (IMACS) set scores. The patients had low IMACS parameters that showed low or absent disease activity. The distribution of cardiovascular diseases and their risk factors was similar between the patients and the control group (P > 0.05) at the time of the analysis. The patients with DM and the control group had similar aerobic capacity. However, the patients with ASS exhibited significantly reduced aerobic capacity (relative VO2 peak, anaerobic threshold, respiratory compensation point and time to exhaustion) when compared to the control group. In addition, patients with ASS had a lower anaerobic threshold compared to the DM group. There were no significant relationships between the aerobic capacity and disease status, cardiovascular diseases and their risk factors. In contrast to DM patients and healthy individuals, patients with stable ASS have significantly impaired aerobic capacity, which is unlikely to be totally explained by traditional cardiovascular diseases, their risk factors and disease status. Further studies are needed to corroborate our data and to clarify the cause of this reduced aerobic capacity in ASS.
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the clinical manifestations at the onset of Antisynthetase Syndrome a chameleon with multiple faces
Reumatismo, 2020Co-Authors: A C C D Baccaro, Renata Casseb De Souza Carboni, G Behrens L Pinto, Samuel Katsuyuki ShinjoAbstract:The Antisynthetase Syndrome (ASS) is clinically characterized by fever, myositis, interstitial lung disease, joint involvement, mechanic's hands, or Raynaud's phenomenon, and the presence of Antisynthetase autoantibodies. These clinical manifestations may not occur simultaneously. Therefore, the aim of this study was to analyze the sequence in which these clinical manifestations can develop at the onset of ASS. This retrospective, single-center cohort study enrolled 55 ASS patients. Their mean age at the onset of ASS symptoms was 42.3±11.8 years. There was a predominance of female patients (75.9%) and white patients (72.7%). At initial presentation, 41.8% of the patients had fever, 43.6% had joint symptoms, 38.2% had myositis, 36.4% had interstitial lung disease, 18.2% had Raynaud's phenomenon, and 16.4% had mechanic's hands. Subsequent clinical symptoms emerged at varying time points. In two out of 55 cases, joint, muscle, and lung manifestations developed simultaneously. The median time between the onset of symptoms and the complete ASS clinical manifestation was 19.9 (4.0-60.2) months; whereas, the timeframe between the onset of symptoms and the ASS diagnosis was 29.0 (11.0-63.0) months. The confounding misdiagnoses interfering with the initial diagnosis were polymyositis (52.7%), dermatomyositis (29.1%), nonspecific interstitial pneumopathy (23.6%), rheumatoid arthritis (18.2%), and others (10.9%). Clinical features at the onset of ASS are highly variable. Consequently, confounding factors can lead to significant delays for the final and definitive diagnosis of ASS. Therefore, ASS should be considered a differential diagnosis in patients with initial symptoms of joint, lung, and/or muscle involvements, as well as fever, mechanic's hands, and/or Raynaud's phenomenon manifestations.
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a prospective cross sectional study of serum il 17a in Antisynthetase Syndrome
Clinical Rheumatology, 2020Co-Authors: Gustavo Luiz Behrens Pinto, Renata Casseb De Souza Carboni, Fernando Henrique Carlos De Souza, Samuel Katsuyuki ShinjoAbstract:The pro-inflammatory interleukin (IL)-17A serum has been characterized in several systemic autoimmune diseases, but not in Antisynthetase Syndrome (ASS). Therefore, the present study aims firstly to assess the serum level of the IL-17A in patients with ASS, comparing with healthy individuals, and secondly to analyze prospectively this IL in patients with refractory ASS undergoing rituximab treatment. A cross-sectional, single-center study that included 64 patients with ASS who were age-, gender-, and ethnicity-matched to 64 healthy individuals. Disease status was measured by the International Myositis Assessment and Clinical Studies Group (IMACS) set scores. Secondarily, the patients with refractory disease treated with rituximab were prospectively followed for 12 months. The IL-17A was assessed by the ELISA method. The mean age of the patients was 44.8 ± 11.8 years, with a predominance of female gender and Caucasian. The median serum IL-17A level was higher in ASS patients compared with healthy individuals: 9.7 (9.1–10.4) vs. 7.7 (5.7–9.0) pg/mL, respectively, and P < 0.001. However, the demographical, clinical, and laboratory data indicates that disease status did not correlate with serum levels of the IL-17A in ASS patients. Prospectively, 16 patients received rituximab, and there was a drop of IL-17A serum level over the first year of treatment in these patients: from 9.7 (9.1–10.6) to 9.0 (8.2–9.7) pg/mL (P = 0.01). Our study demonstrated that patients with ASS have increased serum levels of the IL-17A compared with healthy controls. In addition, the patients with refractory ASS treated with rituximab showed a reduction of the serum levels of the IL-17A.
Lorenzo Cavagna - One of the best experts on this subject based on the ideXlab platform.
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comment on idiopathic inflammatory myopathies and Antisynthetase Syndrome contribution of Antisynthetase antibodies to improve current classification criteria by greco et al
Annals of the Rheumatic Diseases, 2020Co-Authors: Johannes Knitza, Lorenzo Cavagna, Georg Schett, Jorg H W DistlerAbstract:With great interest, we read the letter titled ‘Idiopathic inflammatory myopathies and Antisynthetase Syndrome: contribution of Antisynthetase antibodies to improve current classification criteria’ by Greco et al 1 published in the Annals of the Rheumatic Diseases . The authors analysed if the detection of anti-aminoacyl transfer RNA synthetase (ARS) autoantibodies other than anti-Jo1 could improve the European League Against Rheumatism/Amercian College of Rheumatology (EULAR/ACR) classification criteria2 for adult and juvenile idiopathic inflammatory …
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Antisynthetase Syndrome or what else different perspectives indicate the need for new classification criteria
Annals of the Rheumatic Diseases, 2017Co-Authors: Lorenzo Cavagna, Santos Castaneda, Carlo Alberto Scire, Miguel A GonzalezgayAbstract:We read with great interest the extended report by Lilleker et al 1 on the EuroMyositis registry. Our attention was particularly addressed to Antisynthetase Syndrome (ASSD) because in the last years we and all members of the AENEAS (American and European Network of Antisynthetase Syndrome) collaborative group strongly contributed2–7 to increase the knowledge on this peculiar disease. We think that a comparison between our cohorts could be of interest and useful for clinicians, even if the lack of some data in the EuroMyositis paper does not allow us to perform statistical analysis. Both groups collected a very large number of patients (AENEAS collaborative group 813 cases, EuroMyositis 512 cases). The comparison of available …
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serum jo 1 autoantibody and isolated arthritis in the Antisynthetase Syndrome review of the literature and report of the experience of aeneas collaborative group
Clinical Reviews in Allergy & Immunology, 2017Co-Authors: Lorenzo Cavagna, Santos Castaneda, Carlo Alberto Scire, L Nuno, Marcello Govoni, Francisco Javier Lopez Longo, Franco Franceschini, R Neri, Walter Alberto Sifuentes Giraldo, Roberto CaporaliAbstract:Anti-Jo-1 is the most frequently detectable antibody in the Antisynthetase Syndrome (ASSD), an autoimmune disease characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). Recently, we organized an international collaborative group called American and European NEtwork of Antisynthetase Syndrome (AENEAS) for the study of this rare and fascinating disease. The group collected and published one of the largest series of ASSD patients ever described and with one of the longer follow-up ever reported. The number of participating centers is steadily increasing, as well as the available cohort. In the first paper, we showed that arthritis, myositis, and ILD may be frequently the only feature at disease onset, raising problems to reach a correct diagnosis of this Syndrome. Nevertheless, we first observed that the ex novo appearance of further manifestations is common during the follow-up, strengthening the importance of a correct diagnosis. In our cohort, the 24 % of the 243 patients up to now collected had isolated arthritis as a presenting feature. These patients represent the most intriguing group in terms of differential diagnosis and clinical time course. Furthermore, data on this aspect are scanty, the reason that lead us to evaluate these aspects in our cohort of patients, reviewing also available literature. In fact, the most relevant aspect is that ASSD is rarely suspected in this setting of patients, in particular in case of poliarticular involvement, positive rheumatoid factor (RF), or anti-cyclic citrullinated peptide antibodies (ACPA) or evidence of joint erosions at plain radiographs. These findings were not rare in our cohort, and they have been also described in other series. Furthermore, manifestations such as Raynaud’s phenomenon, mechanic’s hands, and fever that may lead to the suspect of ASSD are observed only in a third of cases. If we consider the high rate of clinical picture progression in these patients, we feel that ASSD should be carefully considered in all patients presenting with isolated arthritis, even in those with erosive, RF, and ACPA-positive arthritis.
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fri0291 clinical spectrum time course in non anti jo 1 positive Antisynthetase Syndrome
Annals of the Rheumatic Diseases, 2016Co-Authors: Lorenzo Cavagna, Oyvind Molberg, Santos Castaneda, M A Gonzalezgay, H Andersson, F Franceschini, Ilaria Cavazzana, F Lopez J Longo, Silvia Balduzzi, Carlomaurizio MontecuccoAbstract:Background The clinical phenotype of the Antisynthetase Syndrome (AS) has been related with the underlying autoantibody specificity. The typical disease triad (arthritis, myositis, and interstitial lung disease -ILD) is common in anti Jo-1 + AS, whereas isolated ILD is associated with anti PL-7 and PL-12 Ab. The majority of anti Jo-1 + patients without all triad findings at the onset (incomplete ASSD) will develop lacking features during the followup Objectives To assess the triad clinical spectrum time course in non anti Jo-1 + AS Methods The study cohort included patients + for anti-EJ, OJ, PL-7, or PL-12 Ab with at least 1 triad finding. Clinical features were retrospectively collected and analyzed Results we included 65 non anti Jo-1 + AS (7 anti EJ, 4 anti OJ, 30 anti PL-12, 24 anti PL-7; 49 females, 16 males). In median, age at disease onset was 53 years (IQR 42–64), diagnostic delay 9 months (IQR 3–23.5), followup 60 months (IQR 20.5–113). Patients9 characteristics are reported in Table 1. During the followup, 22/61 patients with incomplete AS developed new triad findings, in median 12 months (IQR 6–27) after disease onset. The frequency of incomplete AS developing new triad findings and the length of followup were significantly reduced in non anti Jo-1 + patients with respect to our corresponding anti Jo-1 + cohort (respectively: 22/61 vs 138/220, p Conclusions In non anti Jo-1 + patients the ex novo occurrence of classic triad finding is common, but less frequent than in anti Jo-1 + patients. The different length of followup may explain at least partly this difference References Cavagna L, et al. Medicine (Baltimore) 2015;94:e1144 Disclosure of Interest None declared
I Marie - One of the best experts on this subject based on the ideXlab platform.
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functional outcome and prognostic factors in anti jo1 patients with Antisynthetase Syndrome
Arthritis Research & Therapy, 2013Co-Authors: I Marie, P Cherin, J F Menard, Eric Hachulla, P Y Hatron, Olivier Vittecoq, E Diot, F Jouen, S DominiqueAbstract:The aims of this present study were firstly to assess the outcome, including functional course, in anti-Jo1 positive patients with Antisynthetase Syndrome (ASS), and secondly to determine predictive parameters of poor outcome in these patients. The medical records of 86 consecutive anti-Jo1 patients with ASS were reviewed in 4 academic centers. 13 patients (15.1%) achieved remission of ASS, whereas 55 (63.9%) improved and 18 (20.9%) deteriorated in their clinical status. Both steroid and cytotoxic drugs could be discontinued in only 4.7% of patients. ASS was associated with decreased quality of life at long-term follow-up: only 69.2% of patients considered to be in remission experienced a return to previous normal activities; and 24.7% of other patients with non-remitting ASS still had a marked reduction of activities (as shown by the disability scale of the Health Assessment Questionnaire). Decreased quality of life was further due to calcinosis cutis (8.1%) and adverse effects of steroid therapy (36%). Factors associated with ASS deterioration were older age, pulmonary and esophageal involvement, calcinosis cutis and cancer. Higher anti-Jo1 levels were further associated with disease severity in ASS patients. The present study shows high morbidity related to ASS. Furthermore, we suggest that patients with predictive factors of ASS deterioration may require more aggressive therapy. Our findings also suggest that in anti-Jo1 patients with severe esophageal manifestations, combined high dose steroids and intravenous immunoglobulins might be proposed as the first line therapy. Finally, as cancer occurred in 14% of anti-Jo1 patients, our findings underscore that the search for cancer should be performed in these patients.
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clinical manifestations and outcome of anti pl7 positive patients with Antisynthetase Syndrome
European Journal of Internal Medicine, 2013Co-Authors: I Marie, Eric Hachulla, Severine Josse, P Y Hatron, Olivier Decaux, E Diot, Cedric Landron, P Roblot, S Jouneau, Olivier VittecoqAbstract:BACKGROUND: The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with Antisynthetase Syndrome (ASS). METHODS: The medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. RESULTS: Anti-PL7 patients exhibited polymyositis (n=14) and dermatomyositis (n=1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n=5), was identified concomitantly with ASS (n=8) and occurred after ASS diagnosis (n=1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n=1), progressive onset of lung signs (n=11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n=2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO<45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP). CONCLUSION: Our series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO<45% at ILD diagnosis and UIP pattern on HRCT-scan.
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interstitial lung disease in anti jo 1 patients with Antisynthetase Syndrome
Arthritis Care and Research, 2013Co-Authors: I Marie, P Cherin, E Hachulla, S Dominique, A Janvresse, Severine Josse, P Y Hatron, Luc Mouthon, Olivier Vittecoq, J F MenardAbstract:Objective To assess the outcome of interstitial lung disease (ILD) in anti–Jo-1 patients with Antisynthetase Syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti–Jo-1 patients with and without ILD. Methods Ninety-one anti–Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans. Results Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 12), progressive onset of lung signs (n = 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n = 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age ≥55 years. Furthermore, anti–Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels. Conclusion Our findings confirm that ILD is a frequent complication in anti–Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti–Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.
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rituximab therapy for refractory interstitial lung disease related to Antisynthetase Syndrome
Respiratory Medicine, 2012Co-Authors: I Marie, S Dominique, A Janvresse, H Levesque, J F MenardAbstract:Summary Objective To report our experience using rituximab as therapy for refractory Antisynthetase Syndrome (ASS)-associated interstitial lung disease. Methods We retrospectively evaluated the medical records of 7 ASS patients with refractory interstitial lung disease, which had previously failed to respond to prednisone and/or other cytotoxic drugs. All 7 patients received rituximab therapy, i.e.: 1 g at days 0 and 14 and at 6-month follow-up. Data on pulmonary symptoms, pulmonary function tests and high resolution computed tomography (HRCT) scan of the lungs were collected: 1) before rituximab initiation; and 2) at 6-month and one-year follow-up after the first infusion of rituximab. Results At one-year follow-up, ASS patients had resolution ( n = 2) or improvement of pulmonary clinical manifestations. Patients also exhibited significant improvement of interstitial lung disease parameters: 1) on pulmonary function tests: FVC ( p = 0.03) and DLCO ( p = 2 × 10 −5 ); 2) and HRCT-scan of the lungs. Due to clinical resolution/improvement of interstitial lung disease, the median daily dose of oral prednisone could be reduced in these 7 ASS patients at one-year follow-up, compared with baseline (20 mg/day vs. 9 mg/day; p = 0.015). Conclusion Our findings suggest that rituximab may be a helpful therapy for refractory interstitial lung disease in patients with ASS.
J F Menard - One of the best experts on this subject based on the ideXlab platform.
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functional outcome and prognostic factors in anti jo1 patients with Antisynthetase Syndrome
Arthritis Research & Therapy, 2013Co-Authors: I Marie, P Cherin, J F Menard, Eric Hachulla, P Y Hatron, Olivier Vittecoq, E Diot, F Jouen, S DominiqueAbstract:The aims of this present study were firstly to assess the outcome, including functional course, in anti-Jo1 positive patients with Antisynthetase Syndrome (ASS), and secondly to determine predictive parameters of poor outcome in these patients. The medical records of 86 consecutive anti-Jo1 patients with ASS were reviewed in 4 academic centers. 13 patients (15.1%) achieved remission of ASS, whereas 55 (63.9%) improved and 18 (20.9%) deteriorated in their clinical status. Both steroid and cytotoxic drugs could be discontinued in only 4.7% of patients. ASS was associated with decreased quality of life at long-term follow-up: only 69.2% of patients considered to be in remission experienced a return to previous normal activities; and 24.7% of other patients with non-remitting ASS still had a marked reduction of activities (as shown by the disability scale of the Health Assessment Questionnaire). Decreased quality of life was further due to calcinosis cutis (8.1%) and adverse effects of steroid therapy (36%). Factors associated with ASS deterioration were older age, pulmonary and esophageal involvement, calcinosis cutis and cancer. Higher anti-Jo1 levels were further associated with disease severity in ASS patients. The present study shows high morbidity related to ASS. Furthermore, we suggest that patients with predictive factors of ASS deterioration may require more aggressive therapy. Our findings also suggest that in anti-Jo1 patients with severe esophageal manifestations, combined high dose steroids and intravenous immunoglobulins might be proposed as the first line therapy. Finally, as cancer occurred in 14% of anti-Jo1 patients, our findings underscore that the search for cancer should be performed in these patients.
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interstitial lung disease in anti jo 1 patients with Antisynthetase Syndrome
Arthritis Care and Research, 2013Co-Authors: I Marie, P Cherin, E Hachulla, S Dominique, A Janvresse, Severine Josse, P Y Hatron, Luc Mouthon, Olivier Vittecoq, J F MenardAbstract:Objective To assess the outcome of interstitial lung disease (ILD) in anti–Jo-1 patients with Antisynthetase Syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti–Jo-1 patients with and without ILD. Methods Ninety-one anti–Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans. Results Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 12), progressive onset of lung signs (n = 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n = 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age ≥55 years. Furthermore, anti–Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels. Conclusion Our findings confirm that ILD is a frequent complication in anti–Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti–Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.
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rituximab therapy for refractory interstitial lung disease related to Antisynthetase Syndrome
Respiratory Medicine, 2012Co-Authors: I Marie, S Dominique, A Janvresse, H Levesque, J F MenardAbstract:Summary Objective To report our experience using rituximab as therapy for refractory Antisynthetase Syndrome (ASS)-associated interstitial lung disease. Methods We retrospectively evaluated the medical records of 7 ASS patients with refractory interstitial lung disease, which had previously failed to respond to prednisone and/or other cytotoxic drugs. All 7 patients received rituximab therapy, i.e.: 1 g at days 0 and 14 and at 6-month follow-up. Data on pulmonary symptoms, pulmonary function tests and high resolution computed tomography (HRCT) scan of the lungs were collected: 1) before rituximab initiation; and 2) at 6-month and one-year follow-up after the first infusion of rituximab. Results At one-year follow-up, ASS patients had resolution ( n = 2) or improvement of pulmonary clinical manifestations. Patients also exhibited significant improvement of interstitial lung disease parameters: 1) on pulmonary function tests: FVC ( p = 0.03) and DLCO ( p = 2 × 10 −5 ); 2) and HRCT-scan of the lungs. Due to clinical resolution/improvement of interstitial lung disease, the median daily dose of oral prednisone could be reduced in these 7 ASS patients at one-year follow-up, compared with baseline (20 mg/day vs. 9 mg/day; p = 0.015). Conclusion Our findings suggest that rituximab may be a helpful therapy for refractory interstitial lung disease in patients with ASS.