POS0834 TREATMENT AND DISEASE SEVERITY OF SSc-ASSOCIATED PULMONARY HYPERTENSION WITH AND WITHOUT INTERSTITIAL LUNG DISEASE: A LARGE COHORT STUDY OF THE GERMAN NETWORK FOR SYSTEMIC SCLEROSIS

Background: In patients with systemic sclerosis (SSc), pulmonary hypertension (PH) represents a major risk factor for morbidity and mortality. Treatment of patients with PH and interstitial lung disease (ILD) can be particularily challenging. Objectives: Analysis of drug therapy for PH depending on the presence of ILD in patients with SSc included in the multicenter German DNSS registry. Comparison of disease parameters between PH patients with or without ILD. Methods: Descriptive statistic analysis from the Red Cap database of the German DNSS registry for SSc patients. For the analysis of drug therapy, the subgroup of patients who did not have PH at baseline visit and developed it only during follow-up was analyzed. Comparison of disease parameters (worst during follow up) between PH patient with or without ILD with T-test for continous variables was performed. Results: As of 01/16/2023, 6003 patients were included in the DNSS registry. 384 (11.6%of 3314 evaluable patients) developed PH during follow-up. 271 of the PH patients had concomitant interstitial lung disease (70.6%). Table 1 contains the baseline characteristics of these 384 patients. Only age differed significantly (by 4.70 years, p-value=0.001). In the PH with ILD group 57,6 % of patients already had ILD at first visit, the rest developed ILD during follow up. Average follow-up time was longer in the PH with ILD group. The proportion of evaluable patients who ever received a specific PAH drug was: -For ERA (endothelin receptor antagonists) 51.4% for PH + ILD vs 30.9% for PH without ILD. -For PDE5i (phosphodiesterase 5 inhibitors) 43.3% vs 29.9%, respectively Figure 1 shows the frequency of patients receiving monotherapy, dual therapy, triple therapy, and more intensive combination therapies ever comparing patients with and without ILD. PH patients without ILD were more likely to receive monotherapy, whereas patients with ILD were more likely to receive combination therapy. Comparing disease parameters (mean) between patients with or without ILD, patients with ILD had a significantly higher NT-pro-BNP (brain natriuretic peptide), a significantly higher uric acid, a numerically higher PAPsys and a significantly lower left ventricular ejection fraction on echocardiography, a significantly lower forced vital capacity (FVC, 70,6% vs 87,2%) and a significantly lower DLCO (diffusing capacity for carbon monoxide, 44,4% vs 56,9%). Thus, although ILD patients had significantly more severe restrictive lung disease, restrictive impairment was only mild to moderate in most ILD cases. Values for right heart catheter were available only in fewer patients (54 PH with ILD vs 23 PH without ILD), and PAP mean did not differ, while cardiac output was numerically lower in patients with ILD without reaching statistical significance. Conclusion: In spite of current treatment recommendations, PH patients with ILD rather receive more intensive PH treatment under real world conditions, and they seem to have more severe pulmovascular disease. Further analysis are planned to evaluate impact of treatment on survival and change of treatment patterns over time. Table 1. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: Marc Schmalzing BMS, Novartis, Abbvie, AstraZeneca, Chugai/Roche, Janssen-Cilag, Gilead, Boeringer, Mylan, Onkowissen.de, Galapagos, EUSA-Pharma, Roche, Hexal/Sandoz, Gilead, AbbVie, Janssen, Boehringer, Onkowissen.de, EUSA Pharma, Novartis, AstraZeneca, Medac, Lilly, Galapagos, UCB, Chugai, Novartis, Pia Moinzadeh: None declared, Jithmi Weliwitage: None declared, Gabriela Riemekasten: None declared, Norbert Blank Boehringer, Janssen, BMS, EUSA, Novartis, Pfizer, Roche, Sanofi, SOBI, SOBI, Novartis, Jörg Henes Janssen, Chugai, GSK, Boehringer, Novartis, Ulf Müller-Ladner: None declared, Margitta Worm ALK, Arzneimittel GmbH, Milan, Mice GmbH, Viatris, Allergopharma, Aimmune Therapeutics, Novartis, Sanofi-Aventis, Genzyme Europe, Leo Pharma, DBV Technologies, Kymab, Lilly, Gernot Keyszer Abbvie, Astra Zeneca, Biogen, Boehringer, Chugai, Lilly, Novartis, Otsuka, Pfizer, Roche, Sanofi, UCB, Abbvie, Biogen, Boehringer, Lilly, Novartis, Roche, Sanofi, Aaron Juche: None declared, Alexander Kreuter: None declared, Jan Ehrchen Bristol Myers, Squibb, Boehringer, Sobi, Astra Zeneca, Biogen, Janssen, La Roche Posey, Mylan, Galderma, Novartis, Sobi, Boehringer, Laura Susok: None declared, Claudia Günther BI, Boehringer, GSK, Gabriele Zeidler Pfizer, Janssen, Amgen, Lilly, Bristol-Myers Squibb, Boehringer, Ilona Jandova Boehringer, Tim Schmeiser: None declared, Christiane Pfeiffer Actelion - Nurse Training about PH and DU, Actelion, Pfizer, Boehringer, Ina Kötter Boehringer, Amgen, GSK, Jörg Distler AbbVie, Active Biotech, ARXX, Astra Zeneca, Boehringer, Anamar, Bayer, Calliditas Therapeutics, Galapagos, Genentech, GSK, Inventiva, Janssen, Novartis, Pfizer, Roch and UCB, AbbVie, Active Biotech, ARXX, Astra Zeneca, Boehringer, Anamar, Bayer, Calliditas Therapeutics, Galapagos, Genentech, GSK, Inventiva, Janssen, Novartis, Pfizer, Roch and UCB, 4D Science, Scientific Lead of Fibro Cure, AbbVie, Active Biotech, ARXX, Astra Zeneca, Boehringer, Anamar, Bayer, Calliditas Therapeutics, Galapagos, Genentech, GSK, Inventiva, Janssen, Novartis, Pfizer, Roch and UCB, Anamar, ARXX, Argenx, BMS, Bayer, Boehringer Cantargia, Celgene, CSL Behring, Galapagos, GSK, Inventiva, Kiniksa, Lassen, Sanofi, RedX, UCB, Nicolas Hunzelmann Boehringer, Sanofi, Roche, Janssen, Boehringer.