This study was undertaken to assess the quality of life in eSSc patients compared to that in UCTD and controls. We found that HRQOL was impaired in both eSSc and UCTD patients.
It would have been interesting to assess HRQOL in patients with primary RaP, in whom an impaired HRQOL has been recently pointed out by either SF-36 or EUROQoL 5 item questionnaire (EQ-5D) . However, few patients with such a condition are referred to our Rheumatologic tertiary centre.
Our results show that eSSc patients, who, by definition, experience only Raynaud’s phenomenon but no other SSc-specific clinical manifestation, perceive an impaired physical and mental quality of life, as happens in other subgroups of SSc patients [7, 21]. Here we show that disability and an increase in ESR levels are negatively associated with physical health status. In these patients the only functional impairment was the RaP and/or the presence of puffy hands (this latter condition in only 3 eSSc patients).
Since HRQOL has been reported to be impaired in patients with primary RaP, one could postulate that the impairment in HRQOL in SSc and UCTD patients might be ascribed to RaP itself. Such assumption, however, would not totally justify this condition, especially considering that these patients were taking the maximum tolerated dose of vasodilators prescribed to decrease the frequency and duration of RaP attacks and that most of them stated to have a good control of their RaP condition.
Given the low burden of disability registered, we can postulate that the clinical measures commonly assessed in eSSc and UCTD do not completely account for the poor quality of life perceived by these patients and this could be explained in different ways.
First of all, a great difference between the assessment of the disease severity and HRQOL has to be taken into account: in fact, the former is made through a pre-specified combination of clinical, laboratory and instrumental exams which might overlook some aspects of the disease influencing the health status, that is investigated, as known, by self-report questionnaires.
Moreover, a recent study of HRQOL in SSc patients suggested that the most important determinants of physical and mental health are the patient’s cognitive representations of the illness . Arat et al.  observed that the fear of clinical consequences and the tendency to attribute each physical complaint to SSc (“illness identity”), are the main contributors to physical health, whereas the emotional responses to personal representation of the disease are the main contributors to mental health. In addition to illness perception, the use of various strategies to cope better with the disease is related to physical and mental health .
Given the above observations, it is conceivable that in eSSc and UCTD patients similar psychological mechanisms might contribute, together with the disease –related symptoms to the worse quality of life. Unfortunately, one of the shortcomings of our study is that we did not include instruments that measure psychosocial factors, and so no conclusion about the relationships between psychosocial factors and HRQOL can be drawn.
ANA positivity in patients with RaP has long been known to be a factor predictive of the development of a CTD [23, 24]. In that regard, the absence of any significant difference between patients with (eSSc) and without (UCTD) marker autoantibody would suggest that the presence of an autoimmune RaP might be sufficient to affect HRQOL.
This study has some limitations. First of all, the number of patients enrolled is relatively low so that a larger sample is required to confirm these results. Moreover, as already stated it would have been intriguing to assess the HRQOL also in patients affected by a primary RaP, but unfortunately few patients with such a condition are referred to our centre. Finally, our data do not allow us to identify other determinants of the reduced quality of life in both mental and physical domains, apart from those described.
In conclusion, we assessed HRQOL in eSSc and UCTD patients and looked for associations between clinical features and health status perception. Although the correlations observed do not reflect a causal relationship, our results might be useful to identify targets of intervention. Our data would suggest that also these patients experience an impaired quality of life in both physical and mental domains. This condition has to be taken into account by the clinicians involved in their care. In the absence of a therapy able to definitively block injury to lungs, joints and other internal organs, further studies are needed to completely identify the factors that contribute to the poor health status in eSSc and UCTD patients.