Survival analysis of 118 patients with systemic sclerosis

Abstract. Life-table analysis based on the survival statistics of 118 Hungarian patients with systemic sclerosis suggests that the presence of skin hypo- and/or hyperpigmentation indicates a poor prognosis. Furthermore, truncal skin involvement, lung, cardiac, and renal manifestations, older age at the onset of disease, anaemia, hand deformity, and elevated erythrocyte sedimentation rate values, predict an unfavourable prognosis, whilst patients with exposure to chemicals shows a relatively favourable disease outcome.     

Clinical significance of subcutaneous calcinosis in patients with systemic sclerosis. Does diltiazem induce its regression?

OBJECTIVE—To establish whether diltiazem reduces subcutaneous calcinosis (SCC) in patients with systemic sclerosis (SSc), and whether this calcinosis is related to other signs or symptoms.
METHODS—47 patients with SSc were evaluated and divided into two groups according to the presence or absence of SCC.
RESULTS—Among the 12 patients with SCC who were treated with diltiazem and had sequential hand radiographs (differential time between the two radiographs: 7.8±4 years), there was a slight radiological improvement in three patients only. More patients with SCC had anticentromere antibodies than patients without (p=0.003), fewer had anti-Scl 70 antibodies (p=0.01), more had telangiectasia and giant capillaries (p=0.04 and 0.048 respectively), and SCC patients had significantly fewer capillaries at the nailfold (p=0.03).
CONCLUSION—These results do not clearly indicate that diltiazem is effective in calcinosis associated with SSc. Among the patients with SSc, those who also had SCC exhibited a distinctive autoimmune profile and more severe cutaneous capillary injury than those without SCC.

Keywords: systemic sclerosis; scleroderma; calcinosis; diltiazem     

Health‐related quality of life in systemic sclerosis as measured by the short form 36: Relationship with clinical and biologic markers

Objective

To evaluate health‐related quality of life (HRQOL) in patients with systemic sclerosis (SSc) using the Short Form 36 (SF‐36) and to correlate SF‐36 scores with clinical and biologic markers.

Methods

The SF‐36 was administered to 24 controls and 24 SSc patients. SSc patients also were evaluated for subset (limited SSc [lSSc] and diffuse SSc [dSSc]), age, disease duration, angiotensin‐converting enzyme (ACE) levels, autoantibodies, and skin and internal organ involvement.

Results

The physical summary score (PSS) was lower in SSc patients than in controls (P < 0.05), whereas the mental summary score (MSS) was higher in dSSc than in lSSc patients (P < 0.05). Five of 8 single SF‐36 domain scores were lower in SSc patients than in controls (P < 0.05). Vitality was higher in dSSc than in controls (P < 0.001). In SSc, elder age correlated with lower PSS; low ACE levels and high skin score correlated with higher general mental health and role limitations due to physical problems, respectively (P < 0.05). Patients with heart involvement had higher scores in general health perceptions (P < 0.05).

Conclusion

The SF‐36 shows that HRQOL is impaired in patients with SSc. Higher scores in MSS and vitality in patients with dSSc and correlations of high SF‐36 scores with specific organ involvement suggest that SSc patients with severe disease are more able to cope with HRQOL modification.

     

Associations of breast cancer development in patients with systemic sclerosis: an exploratory study

The goal of this study was to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who develop breast cancer and to identify associations for this relationship. From 769 patients followed at the scleroderma center of our institution over the past 16 years, 24 patients developed a diagnosis of breast cancer. The demographics and clinical characteristics of these patients will be compared to those of a randomly selected group of scleroderma patients without a diagnosis of cancer. A further analysis will compare the patients who developed their breast cancer before the diagnosis of systemic sclerosis to those diagnosed after. Twenty-four patients developed 25 breast cancers with 13 patients diagnosed before the diagnosis of systemic sclerosis and 11 after. Compared to 48 randomly selected systemic sclerosis patients without a diagnosis of cancer, the patients with breast cancer were diagnosed with systemic sclerosis at an older age (53.5 ± 15.2) as compared to those without (42.4 ± 12.5) (p = 0.002). A relatively equal amount of patients had the diffuse and limited form of systemic sclerosis, while pulmonary fibrosis (p = 0.015) and the lack of antinuclear antibody (ANA) positivity (p = 0.02) were more commonly seen in patients with breast cancer. Patients who developed breast cancer before the diagnosis of systemic sclerosis were older at systemic sclerosis diagnosis (61.6 ± 11.3) compared to those after (43.9 ± 13.5) (p = 0.03). An older age at diagnosis of systemic sclerosis, a lack of ANA positivity, and the presence of pulmonary fibrosis were more commonly seen in patients with systemic sclerosis who have a diagnosis of breast cancer.