AB0391 CLINICAL ASSOCIATIONS OF MYOSITIS AND SYSTEMIC SCLEROSIS IMMUNOBLOTS SPECIFICITIES IN AUTOIMMUNE DISEASE-RELATED INTERSTITIAL LUNG DISEASE: DATA OF 203 PATIENTS FROM A NATIONAL REFERRAL CENTER

• Published in: Annals of the rheumatic diseases Vol. 83; no. Suppl 1; p. 1442
• Main Authors: Atienza-Mateo, B., Renuncio-García, M., Serrano-Combarro, A., Irure-Ventura, J., Cifrián-Martínez, J. M., López Hoyos, M., Blanco, R.
• Format: Journal Article
• Language: English
• Published: Kidlington BMJ Publishing Group Ltd and European League Against Rheumatism 01.06.2024; Elsevier B.V; Elsevier Limited
• Subjects: Arthralgia; Arthritis; Autoantibodies; Autoimmune diseases; Carbon monoxide; Comorbidities; Computed tomography; Demography; Diagnosis; Dysphagia; Gastroesophageal reflux; Lung diseases; Lungs; Medical records; Myalgia; Myositis; Patients; Photosensitivity; Pneumonia; Respiratory function; Rheumatoid arthritis; Scientific Abstracts; Scleroderma; Systemic sclerosis; Thrombosis; Ulcers; Comorbidities; Lungs; Autoantibodies
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2024-eular.3324

Background:Interstitial lung diseases (ILDs) may be idiopathic or related to a known cause, such as rheumatic autoimmune diseases (ADs). Approximately one third of patients with ILD have an associated AD [1]. An early and accurate diagnosis of AD-ILD is crucial, to which the determination of autoantibodies (AAbs) contributes significantly. In clinical practice, it is not unusual to find different or multiple AAbs positivities in patients within the same type of AD-ILD. However, they may present different clinical and pathological characteristics.Objectives:To assess the specificity of myositis and systemic sclerosis AAbs present in a cohort of patients with AD-ILD and their correlation with clinical and pathological features.Methods:We collected data of patients assessed in a multidisciplinary referral clinic of ILD at the Marqués de Valdecilla University Hospital (Santander, Spain). Available medical records of the patients with AD-ILD were registered, with special attention to ILD patterns, clinical manifestations and serologic profile of myositis and systemic sclerosis AAbs.Results:We included a total of 203 patients with AD-ILD (demographic and clinical characteristics shown in Table 1). The most frequent ADs were rheumatoid arthritis (n=50) and systemic sclerosis (n=49), followed by interstitial pneumonia with autoimmune features (n=39) and antisynthetase syndrome (n=28) (Figure 1A). The myositis and systemic sclerosis immunoblots were positive in 62 patients, finding a total of 79 specificities (Figure 1B). Anti-Scl70 was significantly associated with systemic sclerosis (p<0.001), non-specific interstitial pneumonia ILD pattern (p=0.007) and clinically with Raynaud’s phenomenon (RP) (p<0.001), diffuse cutaneous involvement (p<0.001), telangiectasias (p=0.008), digital ulcers (p=0.001) and photosensitivity (p=0.009). Anti-PMScl75 was associated with RP (p=0.003) and diffuse cutaneous involvement (p=0.005). Anti-PL7 and anti-Ro52 were negatively associated with arthritis (p=0.017 and 0.023, respectively). Anti-Ku was significantly associated with myalgia (p=0.007). No further significant correlations between immunoblot AAbs and other features were found.Conclusion:The AAbs profile is decisive in all patients with AD-ILD and may be associated with expected or not so typical characteristics. The presence of multipositivities is not uncommon and further research is required to identify possible clinical correlations.REFERENCES:[1] Atienza-Mateo B, et al. J Clin Med. 2020 May 26;9(6):1606. doi: 10.3390/jcm9061606.This work was partially supported by MTVAL22/01 from IDIVAL.Table 1.Demographic and clinical features of 203 patients with AD-ILD.CharacteristicsPatients with AD-ILDAge at ILD diagnosis (years), mean ± SD59.97 ± 12.46Sex (women/men), n (%)104/99 (51.23/48.77)Smoking history, n (%)131 (64.53)PFTs at ILD diagnosisFVC// DLCO (% predicted), mean ± SD83.18 ± 24.09// 46.65 ± 18.47HRCT pattern of ILD, n (%)Definite// probable// indeterminate UIP72 (35.47)// 21 (10.35)// 4 (1.97)Alternative: NSIP// non-NSIP// unclassifiable80 (39.41)// 24 (11.83)// 2 (0.97)Clinical manifestations, n (%)Arthralgia/ arthritis116 (57.14)Myalgia/ myositis33 (16.25)Raynaud’s phenomenon76 (37.44)Diffuse cutaneous sclerosis45 (22.17)Dysphagia/ GERD70 (34.48)Pulmonary hypertension39 (19.22)Digital ulcers19 (9.36)Telangiectasia24 (11.82)Photosensitivity/ rash13 (6.40)Thrombosis20 (9.85)AD: autoimmune disease; DLCO: diffusing capacity of the lung for carbon monoxide; FVC: forced vital capacity; GERD: gastroesophageal reflux disease; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; NSIP: non-specific interstitial pneumonia; PFTs: pulmonary function tests; SD: standard deviation; UIP: usual interstitial pneumonia.Figure 1.Spectrum of rheumatic AD of the 203 patients with AD-ILD (1A) and specificities of myositis and systemic sclerosis positive immunoblots in 62 patients (1B).Acknowledgements:We thank all the subjects that participated in this study.Disclosure of Interests:Belén Atienza-Mateo: None declared, Mónica Renuncio-García: None declared, Ana Serrano-Combarro: None declared, Juan Irure-Ventura: None declared, José M. Cifrián-Martínez: None declared, Marcos López Hoyos: None declared, Ricardo Blanco Abbvie, Pfizer, Roche, lilly, Bristol-Myers, Janssen, Galapagos and MSD, Abbvie, Pfizer, Roche, lilly, Bristol-Myers, Janssen and MSD, Abbvie, MSD, Novartis and Roche.