Search

Background: Systemic sclerosis (SSc) associated interstitial lung disease (ILD) is a common complication of SSc, with a high mortality, despite current available treatments. Rituximab has shown some promising, although varied, results for the treatment of SSc-ILD., Aims: To determine whether rituximab stabilised or improved pulmonary function at 12 months, in patients with SSc-ILD., Methods: A retrospective analysis of patients with SSc-ILD who progressed despite conventional therapy and received rituximab between 2008 and 2019 was performed at two tertiary centres. Baseline percentage forced vital capacity (FVC) and percentage diffusing capacity of carbon monoxide (DLCO) were compared with 1-year post the first dose of rituximab. Mean and median change in FVC (%) and DLCO (%) were calculated. For those with available data, the FVC (%) and DLCO (%) 2 years and 1 year prior to rituximab were compared with the change 12-months post-rituximab., Results: Thirteen patients were included in the analysis. All patients demonstrated stability in their pulmonary function testing at 1-year post-rituximab. The mean FVC (%) was 57.18 (±16.93 standard deviation (SD)) prior to rituximab and 59.75 (±18.83 SD) 12-month post-rituximab, demonstrating an increase of 2.57 (±4.70 SD; P-value 0.07). The mean DLCO (%) increased from 37.10 (±18.41 SD) prior to rituximab to 38.03 (±19.83) post-rituximab. The mean change in DLCO (%) was 0.93 (±5.05 SD; P-value 0.53). In the 2 years preceding rituximab, the mean FVC (%) and DLCO (%) declined by 9.25 and 9.66 respectively., Conclusion: This case series suggests that rituximab might stabilise pulmonary function tests, and delay deterioration in patients with progressive SSc-ILD. These findings add to the growing body of evidence suggesting a role for rituximab in the treatment of SSc-ILD., (© 2022 Royal Australasian College of Physicians.)

Background: Screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is now standard care in this disease. The existing Australian Scleroderma Interest Group algorithm (ASIGSTANDARD ) is based on transthoracic echocardiography (TTE) and pulmonary function tests (PFT). Recently, ASIG has derived and validated a new screening algorithm (ASIGPROPOSED ) that incorporates N-terminal pro-B-type natriuretic peptide level together with PFT in order to decrease reliance on TTE, which has some limitations. Right heart catheterisation (RHC) remains the gold standard for the diagnosis of PAH in patients who screen 'positive'., Aim: To compare the cost of PAH screening in SSc with ASIGSTANDARD and ASIGPROPOSED algorithms., Methods: We applied both ASIGSTANDARD and ASIGPROPOSED algorithms to 643 screen-naïve SSc patients from the Australian Scleroderma Cohort Study (ASCS), assuming a PAH prevalence of 10%. We compared the costs of screening, the number of TTE required and both the total number of RHC required and the number of RHC needed to diagnose one case of PAH, and costs, according to each algorithm. We then extrapolated the costs to the estimated total Australian SSc population., Results: In screen-naïve patients from the ASCS, ASIGPROPOSED resulted in 64% fewer TTE and 10% fewer RHC compared with ASIGSTANDARD , with $1936 (15%) saved for each case of PAH diagnosed. When the costs were extrapolated to the entire Australian SSc population, there was an estimated screening cost saving of $946 000 per annum with ASIGPROPOSED , with a cost saving of $851 400 in each subsequent year of screening., Conclusions: ASIGPROPOSED substantially reduces the number of TTE and RHC required and results in substantial cost savings in SSc-PAH screening compared with ASIGSTANDARD ., (© 2015 Royal Australasian College of Physicians.)

Background: We sought to determine the prevalence of pulmonary complications and especially pulmonary arterial hypertension (PAH) in an Australian scleroderma population., Methods: Between July 2005 and June 2007, physicians in Western Australia were asked to refer patients with scleroderma specifically for pulmonary hypertension screening. All patients were assessed for PAH and other respiratory conditions using echocardiography, lung function testing and clinical assessments. Right heart catheterization was carried out in patients with evidence of increased right ventricular systolic pressure., Results: Of the 184 patients analysed, 44 had possible PAH on echocardiography. Right heart catheterization confirmed the diagnosis in 24 (13%). Diffuse interstitial lung disease was found in 32 patients representing a point prevalence of 17.4%. The severity of PAH at diagnosis varied according to whether the patients were referred for screening (group A) or for diagnostic (group B) purposes. The 6-min-walk test distance and median pulmonary vascular resistance were significantly worse in group B versus group A (324 vs 402 m; P= 0.02 and 884 dynes/s per cm(-5) vs 486 dynes/s per cm(-5); P < 0.01, respectively)., Conclusion: Screening may result in earlier diagnosis of PAH with, in general more mild disease. This is important, given that early treatment for PAH while patients are less symptomatic is associated with improved exercise tolerance and pulmonary haemodynamics: indices indicative of disease progression and clinical worsening.