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Background and Objective: Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE‐ILD from the OrphaLung and French SLE networks during 2005–2020 aimed to describe the characteristics of patients with SLE‐ILD and analyse factors associated with prognosis. Methods: We analysed data for 89 patients with SLE‐ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27–47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE. Results: Forty two (47.2%) patients were positive for anti‐ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren's syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0–14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non‐specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow‐up of 86.5 [39.5–161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5‐year and 10‐year transplantation‐free survival were 96% (92–100) and 87% (78–97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud's phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression. Conclusion: ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE‐ILD frequently have another connective tissue disease. [ABSTRACT FROM AUTHOR]

Cardiac output (CO) is one of the primary prognostic factors evaluated during the follow-up of patients treated for pulmonary hypertension (PH). It is recommended that it be measured using the thermodilution technique during right heart catheterization. The difficulty to perform iterative invasive measurements on the same individual led us to consider a non-invasive option. The aims of the present study were to assess the agreement between CO values obtained using bioreactance (Starling™ SV) and thermodilution, and to evaluate the ability of the bioreactance monitor to detect patients whose CO decreased by more than 15% during follow-up and, accordingly, its usefulness for patient monitoring. A prospective cohort study evaluating the performance of the Starling™ SV monitor was conducted in patients with clinically stable PH. Sixty patients referred for hemodynamic assessment were included. CO was measured using both the thermodilution technique and bioreactance during two follow-up visits. A total of 60 PH patients were included. All datasets were available at the baseline visit (V0) and 50 of them were usable during the follow-up visit (V1). Median [IQR] CO was 4.20 l/min [3.60–4.70] when assessed by bioreactance, and 5.30 l/min [4.57–6.20] by thermodilution (p<0.001). The Spearman correlation coefficient was 0.51 [0.36–0.64], and the average deviation on Bland-Altman plot was -1.25 l/min (95% CI [-1.48–1.01], p<0.001). The ability of the monitor to detect a variation in CO of more than 15% between two follow-up measurements, when such variation existed using thermodilution, was insufficient for clinical practice (AUC = 0.54, 95% CI [0.33–0.75]). [ABSTRACT FROM AUTHOR]

Objective: Patients with systemic sclerosis and both pulmonary hypertension and interstitial lung disease (SSc–PH‐ILD) generally carry a worse prognosis than patients with SSc and pulmonary arterial hypertension (SSc‐PAH) without ILD. There is no evidence of the efficacy of PAH therapies in SSc–PH‐ILD. We undertook this study to compare survival of and response to treatment in patients with SSc–PH‐ILD and those with SSc‐PAH. Methods: We analyzed 128 patients (66 with SSc–PH‐ILD and 62 with SSc‐PAH) from 15 centers, in whom PH was diagnosed by right‐sided heart catheterization; they were prospectively included in the PH registry. All patients received PAH‐specific therapy. Computed tomography of the chest was used to confirm or exclude ILD. Results: At baseline, patients with SSc–PH‐ILD had less severe hemodynamic impairment than those with SSc‐PAH (pulmonary vascular resistance 5.7 Wood units versus 8.7 Wood units; P = 0.0005) and lower diffusing capacity for carbon monoxide (median 25% [interquartile range (IQR) 18%, 35%] versus 40% [IQR 31%, 51%]; P = 0.0005). Additionally, patients with SSc–PH‐ILD had increased mortality (8.1% at 1 year, 21.2% at 2 years, and 41.5% at 3 years) compared to those with SSc‐PAH (4.1%, 8.7%, and 21.4%, respectively; P = 0.04). Upon treatment with PAH‐targeted therapy, no improvement in the 6‐minute walk distance was observed in either group. Improvement in the World Health Organization functional class was observed less frequently in patients with SSc–ILD‐PH compared to those with SSc‐PAH (13.6% versus 33.3%; P = 0.02). Hemodynamics improved similarly in both groups. Conclusion: ILD confers a worse prognosis to SSc‐PH. Response to PAH‐specific therapy is clinically poor in SSc–PH‐ILD but was not found to be hemodynamically different from the response observed in SSc‐PAH. [ABSTRACT FROM AUTHOR]

Multiple clinical conditions are combined with genetic mutations to contribute to the development of pulmonary vascular remodelling in hereditary haemorrhagic telangiectasia. A systematic aetiological evaluation is required for these patients. https://bit.ly/34V7HPy., Competing Interests: Conflict of interest: V. Margelidon-Cozzolino reports non-financial support for travel to medical meetings from Actelion and Vitalaire outside the submitted work. Conflict of interest: V. Cottin reports advisory board and lecture fees, and travel support from Actelion; grants, consultancy and lecture fees, and travel support from Boehringer Ingelheim and Roche; advisory board and data safety monitoring board (DSMB) fees from Bayer/MSD and Galapagos; advisory board and lecture fees from Novartis; lecture fees from Sanofi; DSMB and steering committee fees from Promedior; and DSMB fees from Celgene and Galecto, all outside the submitted work. Conflict of interest: S. Dupuis-Girod has nothing to disclose. Conflict of interest: J. Traclet reports travel to medical meetings supported by Roche, Boehringer Ingelheim, CSL Behring and Actelion, and lecture fees from Actelion, outside the submitted work. Conflict of interest: K. Ahmad reports lecture fees and invitations to medical meetings from Actelion and from Roche, and invitations to medical meetings from Boehringer Ingelheim, outside the submitted work. Conflict of interest: J.F. Mornex reports consultancy fees from LFB Biomedicaments and CSL Behring, lecture fees and travel support from Actelion, consultancy fees from Adene, travel support from Agiradom and Amgen, travel support and lecture fees from Boehringer Ingelheim and Roche, and travel support from Novartis, Pfizer and Vitalaire, outside the submitted work. Conflict of interest: S. Turquier reports lecture fees and a congress invitation from Actelion, and a congress invitation from Vitalaire, outside the submitted work., (Copyright ©ERS 2021.)