The emerging association between bronchiectasis and systemic sclerosis: assessing prevalence and potential causality

Abstract

Background: Bronchiectasis has been observed in association with systemic sclerosis (SSc). Theorised aetiology includes aspiration related to oesophageal dysmotility, immunosuppressant medication use and the direct effect of collagen deposition on airway calibre.Aims: To detail bronchiectasis prevalence in an SSc population who have had a highresolution computed tomography (HRCT) of the thorax. We assessed whether oesophageal dysmotility, demographic variables, SSc duration or subclass were associated with bronchiectasis.Methods: Participants in the Australian Scleroderma Cohort Study (ASCS) with a HRCT were included. The ASCS provided demographic and clinical data. HRCT studies were reviewed for bronchiectasis, oesophageal dilatation and interstitial lung disease (ILD). Traction bronchiectasis associated with ILD was recorded as a separate entity to bronchiectasis. Oesophageal dysmotility was defined by symptoms and/or oesophageal dilatation.Results: Of the 256 participants, 16.4% (n = 42) had bronchiectasis. Logistic regression analysis revealed no significant association between bronchiectasis and oesophageal dysmotility (observed in 95.7%), any demographic variable, SSc duration or subclass. A negative association between bronchiectasis and ILD was observed (P = 0.009; odds ratio 0.322; 95% confidence intervals 0.137-0.756). Conclusion:Those with SSc appear to have an increased risk for bronchiectasis. Since bronchiectasis was not more frequent in participants with a longer duration of SSc, we hypothesise that its development is not related to immunosuppression alone. Oesophageal dysmotility was almost universal in our population such that its effect on bronchiectasis development could not be concluded. A negative association between bronchiectasis and ILD reflects that bronchiectasis occurring alongside ILD was recorded as a separate entity.

Systemic sclerosis: one year in review 2022

“…Results from the Australian Scleroderma Cohort Study reported that 16.4% and 95.7% out of 256 patients had bronchiectasis and oesophageal dysmotility, respectively. A negative association between bronchiectasis and ILD was observed (75). Ostojic et al reported the presence of small airway obstruction in 66.6% of SSc patients.…”

Systemic sclerosis and risk of bronchiectasis: A nationwide longitudinal cohort study

“…Previous studies reporting the prevalence of bronchiectasis in patients with systemic sclerosis were based on small cohorts of systemic sclerosis (the largest study population was 256). In this regard, the prevalence of bronchiectasis in systemic sclerosis was reported to range widely from 3.3-59.1% [9][10][11]. To overcome the limitation of a small study population in previous studies, our study included a large number of individuals with systemic sclerosis (n = 4,485) and revealed that about 5.5% of individuals with systemic sclerosis developed new bronchiectasis during a median 6-year follow-up period.…”

“…Non-cystic brosis bronchiectasis (hereafter referred to as bronchiectasis) is a chronic lung disease characterized radiologically by permanent bronchial dilatation and clinically by the presence of cough, sputum, and recurrent chest infections [8]. A few systemic sclerosis cohort studies have reported that bronchiectasis is a common pulmonary comorbidity in patients with systemic sclerosis, ranging from 3.3-59.1% [9][10][11]. However, it is not well known whether the risk of incident bronchiectasis is higher in patients with systemic sclerosis due to small study populations and the absence of comparable controls [9][10][11].…”

“…A few systemic sclerosis cohort studies have reported that bronchiectasis is a common pulmonary comorbidity in patients with systemic sclerosis, ranging from 3.3-59.1% [9][10][11]. However, it is not well known whether the risk of incident bronchiectasis is higher in patients with systemic sclerosis due to small study populations and the absence of comparable controls [9][10][11]. Furthermore, bronchiectasis itself is known to be associated with increased mortality [12], and an in ammatory process in the lungs could provoke autoimmune responses in rheumatoid arthritis potentially creating a worse prognosis [13,14].…”

Nontuberculous Mycobacteria, Mucociliary Clearance, and Bronchiectasis

“…Connective tissue diseases (CTD) related to autoimmune diseases, such as rheumatoid arthritis, Sjögren syndrome, or systemic sclerosis, have been viewed as risk factors of bronchiectasis [4] because they are frequently concomitant diseases [71][72][73]. The reasons may be associated, in some cases, with the use of immunosuppressive therapies such as mycophenolate mofetil, believed to induce hypogammaglobulinemia [74].…”