The major finding was a bimodal incidence pattern with an increased risk of lymphoma, mainly NHL, early during follow-up, but lung cancer and squamous skin cancer later on.

IntroductionWhile microvascular disease is well described in systemic sclerosis (SSc), it is still unclear whether the occurrence of ischemic macrovascular events and atherosclerosis is enhanced among patients with SSc.MethodsIn this study, 111 SSc patients (74% of prevalent cases in Stockholm County) and 105 age- and sex-comparable population controls were investigated. Previous ischemic arterial events were tabulated. As surrogate measures of atherosclerosis, plaque occurrence and intima-media thickness (IMT) were determined with carotid ultrasound and the ankle-brachial index (ABI) was calculated. Traditional cardiovascular risk factors were recorded and we also measured biomarkers indicating systemic inflammation and endothelial activation/dysfunction.ResultsMean age was 62 ± 12 years for patients and controls. Ischemic arterial events were more common, due to increased occurrence of ischemic heart disease (IHD) and ischemic peripheral vascular disease (IPVD), in the patient group (12% vs. 4%, P = 0.03 and 9% vs. 0%, P = 0.003 respectively). On a group level, there was no difference regarding the occurrence of ischemic cerebrovascular disease, the frequency of plaques, IMT or ABI between SSc patients and controls. Subgroup analyses revealed that patients with anticentromere antibodies (ACA+) had more plaques and more ischemic arterial events compared to other SSc patients (67% vs. 39% and 32% vs. 11%; P = 0.006 and P = 0.01, respectively) and compared to controls (67% vs. 41% and 32% vs. 7%, P = 0.02 and P = 0.0003, respectively). Biomarkers of inflammation/endothelial activation were generally increased among SSc patients.ConclusionsPatients with SSc are at enhanced risk for IHD and IPVD. The ACA+ SSc subgroup was particularly affected with both ischemic arterial events and premature atherosclerosis. The microvascular vulnerability of ACA+ patients is previously well documented. We demonstrate that ACA+ SSc patients have an enhanced risk of macrovascular injury as well. This group should be followed closely and modifiable cardiovascular risk factors should be treated at an early stage.

BackgroundNo previous studies have examined the effect of intensive exercise in systemic sclerosis patients with pulmonary impairment. The objective of this study was to examine the effect of an eight-week intensive aerobic exercise and muscle endurance training program for patients with systemic sclerosis with 50–100% of forced vital capacity.MethodsA single-subject experimental design with repeated systematic measures during a six week A-phase (non-interventional baseline period) and an eight week B-phase (exercise intervention period) was used. Three women and one man with median age 66 years and median disease duration of 3.5 years completed aerobic exercise corresponding to 15 on the Borg RPE scale (strenuous) and muscular endurance training three times/week. Physical capacity (six-minute walk test), aerobic capacity (submaximal treadmill test) and muscle endurance in shoulder and hip flexion (Functional Index 2) were assessed every other week throughout the 14-week study. Activity limitation (Health Assessment Questionnaire), quality of life (Short Form 36), Raynaud, Fatigue and Global Health during the recent week (Visual Analogue Scales) were assessed at weeks 0, 6, 14.ResultsThree participants improved significantly in muscular endurance, and two participants improved significantly or clinically relevant in aerobic capacity. All other variables remained unchanged, except for a trend towards reduced fatigue.ConclusionsThis eight week exercise program was largely successful with positive effects on aerobic capacity and muscle endurance.Trial registrationClinicaltrials.gov Identifier: NCT01813578

Both NTproBNP and hs-cTnI were associated with echocardiographic abnormalities, which were more prevalent in SSc patients than in controls. Our results thus suggest that hs-cTnI could be a potential cardiac biomarker in SSc. Low RV function and signs of pulmonary hypertension (PH) were uniquely found in the SSc group. SSc patients had more valve regurgitation than controls, an observation that warrants more clinical attention.