AB0676 One year progression of interstitial lung disease in connective tissue diseases. A descriptive study in a single tertiary center.

Abstract

BackgroundInterstitial lung disease (ILD) in connective tissue diseases (CTD) is an important cause of morbidity and mortalitiy.ObjectivesTo evaluate ILD in CTD (systemic sclerosis, myositis, Sjögren syndrome, rheumatoid arthritis, mixed connective tissue disease), sarcoidosis and interstitial pneumonia with autoimmune features and its progression in 12 months evaluated through high resolution computed tomography (HRCT) and pulmonary function test (PFT).MethodsA retrospective single tertiary center cohort study in CTD-ILD outpatients seen between 2012 and 2021. Clinical, serological data, PFT and HRCT results were collected. ILD patterns were classified into: usual interstitial pneumonia (UIP), inconsistent UIP, nonspecific interstitial pneumonia (NSIP), fibrosing NSIP, organizing pneumonia, interstitial lymphoid pneumonia and associated to sarcoidosis. Progression of ILD was defined as:->10% decline in FVC in PFT.->15% decline in DLCO in PFT.-Progression of fibrosis in HRCT.IBM SPSS v23 was used for statistical analysis.Results51 patients were collected. Baseline characteristics are shown in Table 1. Figure 1 shows ILD progression in 1 year.Table 1.Baseline characteristics.Sociodemographic characteristicsClinical features/affection– n (%)Total51Raynaud23 (45,1%)Female-n (%)42 (82,4%)Skin23 (45,1%)Age- years (mean) IQR)56 (27-82)Ocular13 (25,5%)Arthritis16 (31,4%)Myositis7 (13,7%)Renal1 (2%)Esophagus7 (13,7%)Hemathological3 (5,9%)PHT7 (13,7%)Smoking status- n (%)Symptoms at ILD diagnosis- N (41) %Current9 (17,6%)Cough31,4%Former13 (25,5%)Toracic pain11,8%Never29 (56,9%)Dyspnea47,1%Comorbidities- n (%)Pattern HRCT- n (%)Diabetes mellitus5 (9,8%)UIP11 (21,6%)Ischaemic cardiopathy3 (5,9%)Fib-NSIP5 (9,8%)Hypertension15 (29,4%)UIPincons13 (25,5%)POCD3 (5,9%)NSIP15 (29,4%)OP2 (3,9%)LIP2 (3,9%)Sarcoidosis3 (5,9%)Type of disease associated to ILD - n (%)Immunosupression- n (%)Systemic Sclerosis14(27,5%)Methotrexate20(39,2%)Sjögren’s Syndrome7 (13,7%)Mycophenolate mofetil16 (31,4%)Sarcoidosis3 (5,9%)Hydroxychloroquine19(37,3%Myositis8 (15,7%)Cyclophosphamide4 (7,8%)SLE1 (2%)Etanercept6 (11,8%)MCTD3 (5,9%)TNF inhibitors4 (7,8%)RA12(23,5%)Tocilizumab2 (3,9%)IPAF4 (7,8%)Azathioprine10 (19,6%)Leflunomide3 (5,9%)MP pulses15 (29,4%)Rituximab12 (23,5%)Abatacept5 (9,8%)Tacrolimus5 (9,8%)Antibodies- n (%)Anti-myositis6 (11,8%)Anti-sclerosis15(29,4%)Anti- Ro25 (49%)Anti- RNP3 (5,9%)ANA35(68,6%)RF21(41,2%)Anti- Synt6 (11,8%)Note. POCD = Pulmonary Obstructive Chronic Disease SLE = Systemic Lupus Erythematosus MCTD = Mixed Connective Tissue Disease RA = Rheumatoid Arthritis IPAF = Insterstitial Pneumonia with Autoimmune Features Anti RNP = Anti RiboNucleoProtein ANA = Antinuclear Antibodies RF = Rheumatoid Factor Anti Synt =Anti-synthetasePHT= Pulmonary Hypertension UIP = Usual Interstitial Pneumonia Incons-UIP = Inconsistent Usual Interstitial PneumoniaNSIP = Nonspecific Interstitial PneumoniaFib-NSIP = Fibrosing Nonspecific Interstitial PneumoniaOP = Organizing PneumoniaLIP = Lymphoid Interstitial PneumoniaTNF inhibitor = Tumor Necrosis Factor inhibitorMP = MetyhprednisoloneFigure 1.During follow up, 1 patient with sarcoidosis died of COVID19 bilateral pneumonia.ConclusionIn our series most patients were middle aged women. Anti-Ro antibodies and smoking status (former or current) were common among patients. Common clinical features were Raynaud (45%), skin affection (45%) and arthritis (40%). 47% of the patients expressed dyspnea at ILD diagnosis. 29,4% were treated with MP pulses, 23,5% with rituximab, 31,4% with mycofenolate mophetil. Fibrosing pattern in HRCT (UIP and fib-NSIP) was the most prevalent. 20% of the patients had progressive fibrosis under PFT criteria and 18% under HCRT.More studies of ILD-CTD are necessary to identify factors for progression and response to treatment and throw out more conclusions of prediction and prognosis of disease.Disclosure of InterestsNone declared