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SSc Mortality Causes — SCE Rheumatology MCQ

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EasySystemic SclerosisSSc Mortality CausesSCE Rheumatology

A 48-year-old woman with systemic sclerosis asks which complications account for the greatest proportion of disease-related mortality. Which pair is most important?

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Correct answer: DInterstitial lung disease and pulmonary arterial hypertension

Interstitial lung disease and pulmonary arterial hypertension are the leading causes of systemic-sclerosis-related mortality. This cardiopulmonary burden underpins routine surveillance with pulmonary function testing, thoracic imaging where appropriate, echocardiography and further pulmonary hypertension assessment when indicated. Primary cardiac involvement is also an important disease-related cause of death. Scleroderma renal crisis remains life-threatening, but it is no longer the predominant cause of mortality following the introduction of prompt ACE-inhibitor treatment. Malignancy and infection are important causes of non-SSc-related death, so option C does not answer the question as framed. Gastrointestinal disease, arthritis, calcinosis, digital vasculopathy and osteoporosis cause substantial morbidity but do not collectively account for the largest proportion of SSc-related deaths.

Reference: Good SD, Lee JY, Johnson RE, Volkmann ER. A scoping review of the epidemiology of systemic sclerosis and its organ manifestations: 2018-2024. Current Opinion in Rheumatology. 2024. https://pubmed.ncbi.nlm.nih.gov/20551155/