Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)

Abstract

We found a significantly increased mortality in patients with SSc, particularly in the subset of patients with diffuse skin sclerosis and in young patients. The excess mortality was due to an increase in both the SSc-related mortality and the unrelated mortality. However, all other subsets also had a significantly increased risk of death, mainly due to an increased risk of death due to causes unrelated to SSc, cancer among others. The fact that the unrelated mortality in SSc was found to be 2-fold increased suggests that the excess mortality that was classified as unrelated should more appropriately be termed indirectly SSc related.

Malignancy in scleroderma patients from south west England: a population-based cohort study

“…[10] A Danish study by Jacobsen et al reported malignancy as the primary cause of death in 19% of scleroderma patients, where cancers of the lung (43%), haematological system (13%) and breast (10%) were predominantly implicated. [13] After excluding lung malignancies which were not observed in our study, these observations relate to the most frequently affected sites of malignancy as observed within our scleroderma cohort.…”

Association of C‐reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group

“…There are 2 subtypes of SSc, i.e., diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc), according to the extent of cutaneous involvement (2); dcSSc has more extensive skin involvement, including the trunk or proximal extremities, and is associated with more internal organ involvement such as significant interstitial lung disease (ILD), cardiomyopathy, and scleroderma renal crisis (SRC) and with increased mortality (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”

Mortality and causes of death in primary Sjögren's syndrome: A prospective cohort study

“…Other rheumatic connective tissue diseases such as RA (5), SLE (6), and scleroderma (7) have been shown to be associated with increased mortality, especially with cardiovascular death. Only a few studies have investigated mortality in SS, a disease that is similar to SLE in some characteristics such as autoantibody production, but different in other respects such as the frequency of severe organ involvement (infrequent in SS).…”