SYSTEMIC SCLEROSIS-LUNGS

SUMMARY

1. Main cause of morbidity/mortality in SSC, lung death is due to pulmonary HTN - arterial intimal proliferation & interstitial fibrosis.

2. Order pulmonary function tests (PFTs) with spirometry regularly (every 6-12 months).

3. Cyclophosphamide may be used for interstitial lung disease with active alveolitis.

4. Pulmonary HTN is often treated with drugs that cause vasodilation of the pulmonary vasculature such as prostaglandins.

5. Lung transplant may be required.

Reference(s)

Wilkinson, I. (2017). Oxford handbook of clinical medicine. Oxford: Oxford University Press.
Hannaman, R. A., Bullock, L., Hatchell, C. A., & Yoffe, M. (2016). Internal medicine review core curriculum, 2017-2018. CO Springs, CO: MedStudy.
Therapeutic Guidelines. Melbourne: Therapeutic Guidelines Limited. https://www.tg.org.au [Accessed 2021].