AB0092 ANTISYNTHETASE SYNDROME: CLINICAL PROFILE, SEROLOGIC AND TREATMENTS USED IN A COHORT OF PATIENTS FOLLOWED AT THE VIRGEN MACARENA HOSPITAL

Background:The antisynthetase syndrome (SAS) is characterized by the presence of antisynthetase antibodies, anti-JO1, PL7 y PL12 are the most common; and the classic triad of myositis, arthritis, and diffuse interstitial lung disease (ILD)1. Most patients present incomplete forms and the severity of the ILD determines the prognosis of the disease2.Objectives:to analyze epidemiological, clinical and serological characteristics and treatments used in a cohort of patients with SAS.Methods:descriptive study of review of medical records. Data were collected from 15 patients with SAS followed in the Rheumatology and Pneumology consultations of the Virgen Macarena Hospital (Seville) in the last 10 years. The analysis was carried out using the R software.Results:15 patients were included, 8 men and 7 women. The median age was 56 years (33-77). Seven patients (47%) used to smoke. Four patients (27%) met the classical triad. All of them presented ILD and 8 patients (53%) had arthritis and / or myositis. Five (33%) had mechanic’s hands and six of them (40%) presented Raynaud. Seven (47%) suffered from dyspnea before the SAS diagnosis. The median diagnostic delay was 1 month (0-43). Seven (47%) patients had anti-JO1, 1 (7%) anti-PL7, 2 (13%) anti-PL12 and 2 (13%) patients anti-Ro52. Radiological patterns detected by HRCT were: 5 (33%) NINE, 4 (37%) NIU and 6 (40%) others. The initial treatment included mostly (66%) glucocorticoids (GC) and one or more cFAME. In maintenance, mycophenolate was used in 7 patients (47%), cyclosporine 5 (33%), cyclophosphamide in 3 cases (20%), azathioprine in 3 patients (20%) and methotrexate in 3 of them (20%). Four (37%) patients required a combination of DMARDs and 2 cases needed (13%) biological therapy, Rituximab and Tocilizumab. Changes in the mean value of the initial respiratory function tests (FVC1 and DLCO1) and during follow-up (FVC2 and DLCO2) were not relevant (FVC1 81.5% [42-110], FVC2 81% [59-115]; DLCO1 83% [10-112], DLCO2 80.5% [47-108]). Nine patients (60%) remained clinically stable and 3 patients (20%) progressed radiologically. Four patients died from ILD progression.Conclusion:In this study, the incomplete diagnosis of SAS predominated. The most detected antibody was anti-JO1. ILD is present in all cases, with NINE being the most frequent pattern so multidisciplinary management is necessary. Most used treatments were GC and FAMES combined, some cases required biological therapy.References:[1]Irazoque F, et al. Epidemiology, etiology and classification. Reumatol Clin. 2009;5:2-5.[2]Johnson C, et al. Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type. Respir Med. 2014; 108(10):1542-8.Disclosure of Interests:None declared

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Rituximab in the Treatment of Interstitial Lung Disease Associated with Antisynthetase Syndrome: A Multicenter Retrospective Case Review

The Journal of Rheumatology ◽

10.3899/jrheum.170541 ◽

2018 ◽

Vol 45 (6) ◽

pp. 841-850 ◽

Cited By ~ 31

Author(s):

Tracy J. Doyle ◽

Namrata Dhillon ◽

Rachna Madan ◽

Fernanda Cabral ◽

Elaine A. Fletcher ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Interstitial Pneumonia ◽

Clinical Investigation ◽

Medical Center ◽

Usual Interstitial Pneumonia ◽

Antisynthetase Syndrome ◽

Cryptogenic Organizing Pneumonia ◽

Antisynthetase Antibodies

Objective.To assess clinical outcomes including imaging findings on computed tomography (CT), pulmonary function testing (PFT), and glucocorticoid (GC) use in patients with the antisynthetase syndrome (AS) and interstitial lung disease (ILD) treated with rituximab (RTX).Methods.We retrospectively identified all patients at 2 institutions with AS-ILD who were treated with RTX. Baseline demographics, PFT, and chest CT were assessed before and after RTX. Two radiologists independently evaluated CT using a standardized scoring system.Results.Twenty-five subjects at the Brigham and Women’s Hospital (n = 13) and University of Pittsburgh Medical Center (n = 12) were included. Antisynthetase antibodies were identified in all patients (16 Jo1, 6 PL-12, 3 PL-7). In 21 cases (84%), the principal indication for RTX use was recurrent or progressive ILD, owing to failure of other agents. Comparing pre- and post-RTX pulmonary variables at 12 months, CT score and forced vital capacity were stable or improved in 88% and 79% of subjects, respectively. Total lung capacity (%) increased from 56 ± 13 to 64 ± 13 and GC dose decreased from 18 ± 9 to 12 ± 12 mg/day. Although DLCO (%) declined slightly at 1 year, it increased from 42 ± 17 to 70 ± 20 at 3 years. The most common imaging patterns on CT were nonspecific interstitial pneumonia (NSIP; n = 13) and usual interstitial pneumonia/fibrotic NSIP (n = 5), of which 5 had concurrent elements of cryptogenic organizing pneumonia.Conclusion.Stability or improvement in pulmonary function or severity of ILD on CT was seen in most patients. Use of RTX was well tolerated in the majority of patients. RTX may play a therapeutic role in patients with AS-ILD, and further clinical investigation is warranted.

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AB0047 CLINICAL AND RADIOLOGICAL CHARACTERISTICS AND TREATMENTS OF A COHORT OF PATIENTS WITH RHEUMATOID ARTHRITIS AND DIFFUSE INTERSTICIAL PULMONARY DISEASE FOLLOWED AT THE VIRGEN MACARENA UNIVERSITY HOSPITAL

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1413 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1056.1-1056

Author(s):

P. Muñoz Reinoso ◽

F. J. Toyos Sáenz de Miera ◽

D. Ruiz-Montesinos ◽

I. García Hernández ◽

P. Guerrero Zamora ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Respir Crit ◽

Usual Interstitial Pneumonia ◽

Respiratory Function Tests ◽

Mean Value ◽

University Hospital ◽

Radiological Pattern ◽

The Mean

Background:Diffuse interstitial lung disease (ILD) is an extra-articular manifestation of rheumatoid arthritis (RA)1,2. The most common pattern is the usual interstitial pneumonia (UIP), conditioning a worse prognosis3.Objectives:To describe epidemiological, clinical, and radiological characteristics and treatment used in a cohort of patients diagnosed with RA and ILD.Methods:Retrospective descriptive study, including patients with a diagnosis of RA and ILD, reviewed in the Rheumatology and Pneumology consultations of the Virgen Macarena University Hospital, from 2010 to 2019. Data obtained from medical records are analyzed. SPSS statistical software is used.Results:26 patients were included, 18 women (69.2%). Median age at diagnosis of ILD was 62 years (53-73). Twelve patients (46.6%) used to smoke. The mean time from RA diagnosis to ILD diagnosis was 79 months (8-264). The RF was positive in 91.3% cases (21) and 87% of them (20) were ACPA positive. Fourteen patients (53.8%) had erosions and 3 (11.5%) had an associated Sjögren’s Syndrome. When ILD was diagnosed, the RA activity by DAS28PCR was moderate (3.28; 2.34-3.28) and 13 patients (54.1%) suffered from dyspnea. The mean value of FVC and DLCO in the first assessment was 84% (63-108) and 71.7% (64-86), respectively. The most frequent radiological pattern of ILD was NINE in 15 patients (57.7%), 6 of them (23.1%) had UIP and 5 (19.3%) presented other patterns. Prior to ILD diagnosis, 24 (92.3%) patients received oral glucocorticoids, 18 (69.2%) cases started treatment with c-DMARD and 11 (42.3%) of them with b-DMARD; the most widely used were methotrexate (MTX) in 17 patients (65.4%) and anti-TNFα in 10 (38.5%). After diagnosis, treatment was changed to 12 patients (46.6%); the most used DMARD was leflunomide, in 11 (42.3%), MTX was maintained in 7 patients (26.9%); the number of anti-TNFα used decreased to 4 cases (15.4%), using instead drugs such as rituximab 5 (19.2%), abatacept 3 (11.5%), baricitinib 2 (7.7%) and anti-IL6 2 (7, 7%). During follow-up, 11 cases (57.9%) remained radiologically stable. A slight deterioration in DLCO was observed (66%; 51-80) and there was one death due to lung disease (UIP).Conclusion:In this study, the most frequent radiological pattern was NINE. Half of the patients used to smoke. At the diagnosis of ILD (at ILD diagnosis), dyspnea was the most relevant clinical symptom, with a slight deterioration in the? respiratory function tests. This represented a change in the therapeutic strategy.References:[1]Olson AL, Swigris JJ, Sprunger DB, et al. Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med. 2011;183:372-78.[2]Fragoulis GE, Nikiphorou E, Larsen J, Korsten P and Conway R. Methotrexate-Associated Pneumonitis and Rheumatoid Arthritis-Interstitial Lung Disease: Current Concepts for the Diagnosis and Treatment. Front. Med. 2019;6:238.[3]Tanaka N, Kim JS, Newell JD, et al. Rheumatoid arthritis-related lung diseases: CT findings. Radiology. 2004;232:81-91.Disclosure of Interests:None declared

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Treatment in Antisynthetase Syndrome-Associated Interstitial Lung Disease

Current Treatment Options in Rheumatology ◽

10.1007/s40674-021-00177-y ◽

2021 ◽

Author(s):

JA Huapaya ◽

KD Wiley ◽

SK Danoff

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Antisynthetase Syndrome

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Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services

BMJ Open Respiratory Research ◽

10.1136/bmjresp-2020-000829 ◽

2021 ◽

Vol 8 (1) ◽

pp. e000829

Author(s):

Shaney L Barratt ◽

Havra H Adamali ◽

Caroline Cotton ◽

Ben Mulhearn ◽

Hina Iftikhar ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Trna Synthetase ◽

Musculoskeletal Symptoms ◽

Antisynthetase Syndrome ◽

Cytoplasmic Staining ◽

Phenotypic Differences ◽

Lung Physiology

IntroductionAntisynthetase syndrome (ASyS) is a rare autoimmune connective tissue disease (CTD), associated with autoantibodies targeting tRNA synthetase enzymes, that can present to respiratory (interstitial lung disease (ILD)) or rheumatology (myositis, inflammatory arthritis and systemic features) services. The therapeutic management of CTD-associated ILD and idiopathic pulmonary fibrosis (IPF) differs widely, thus accurate diagnosis is essential.MethodsWe undertook a retrospective, multicentre observational cohort study designed to (1) evaluate differences between ASyS-associated ILD with IPF, (2) phenotypic differences in patients with ASyS-ILD presenting to respiratory versus rheumatology services, (3) differences in outcomes between ASySassociated with Jo-1 versus non-Jo-1 autoantibodies and (4) compare long-term outcomes between these groups.ResultsWe identified 76 patients with ASyS-ILD and 78 with IPF. Patients with ASyS were younger at presentation (57 vs 77 years, p<0.001) with a female predominance (57% vs 33%, p=0.006) compared with IPF. Cytoplasmic staining on indirect immunofluorescence was a differentiating factor between ASyS and IPF (71% vs 0%, p<0.0001). Patients with ASyS presenting initially to respiratory services (n=52) had a higher prevalence of ASyS non-Jo-1 antibodies and significantly fewer musculoskeletal symptoms/biochemical evidence of myositis, compared with those presenting to rheumatology services (p<0.05), although lung physiology was similar in both groups. There were no differences in high-resolution CT appearances or outcomes in those with Jo-1 versus non-Jo-1 ASyS-ILD.ConclusionsExtended autoimmune serology is needed to evaluate for ASyS autoantibodies in patients presenting with ILD, particularly in younger female patients. Musculoskeletal involvement is common in ASyS (typically Jo-1 autoantibodies) presenting to rheumatology but the burden of ILD is similar to those presenting to respiratory medicine.

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