Abstract
ObjectiveTo investigate health-related quality of life (HRQOL) in patients affected by early systemic sclerosis (eSSc) and to compare it with that of patients with undifferentiated connective tissue disease (UCTD).MethodsAt baseline, 31 eSSc and 35 UCTD patients underwent clinical evaluation, laboratory investigations, nailfold videocapillaroscopy, echocardiography, and lung function tests. All patients and 40 controls, matched for sex and age completed the Short Form-36 (SF-36) questionnaire and the Health Assessment Questionnaire Disability Index (HAQ-DI).ResultsSF-36 scores were significantly lower in eSSc and UCTD patients than in healthy controls as regards the following domains: physical component score (PCS), mental component score (MCS), physical functioning, role-physical, bodily pain, general health and mental health. PCS was negatively correlated to the HAQ-DI (rho −0.59; p = 0.0004) and ESR >20 mm/h (rho −0.58; p = 0.0006) in eSSc patients. No statistically significant correlation was found between PCS, MCS and HAQ-DI in UCTD patients. Age, sex, disease duration, history of arthritis, low levels of either C3 or C4, a low DLCO (carbon monoxide lung diffusion) and inversion of the E/A ratio were not correlated to PCS and MCS in either eSSc or UCTD patients.ConclusionMany eSSc or UCTD patients perceive they have an impaired quality of life in both physical and mental domains. This condition has to be taken into account by the clinicians involved in the care of these patients.
Highlights
Systemic sclerosis (SSc) is an autoimmune rheumatic disease of unknown etiology characterized by microvascular injury and deposition of collagen and other constituents of extracellular matrix in target organs [1]
The aim of the present study was to investigate Health-related quality of life (HRQOL) in patients affected by early systemic sclerosis (eSSc) and to compare it with that of patients with undifferentiated connective tissue disease (UCTD) presenting only Raynaud’s phenomenon (RaP) and no other symptoms and that of healthy controls to determine whether the burden of secondary RaP and diagnosis of either eSSc or UCTD affects quality of life
Inclusion/exclusion criteria Patients consecutively admitted to the outpatient clinic of the Rheumatology Unit of the Second University of Naples for the evaluation of RaP from January 1st 2007 to December 31st 2011 were enrolled in the study if they fulfilled the following criteria: 1) Koenig et al.’s criteria for early SSc [10] (i.e., RaP with a marker autoantibody and/or a capillaroscopic scleroderma pattern without any clinical manifestation other than RaP, puffy fingers and/or present or past arthralgia/arthritis), or 2) Doria et al.’s criteria [11] for UCTD
Summary
Systemic sclerosis (SSc) is an autoimmune rheumatic disease of unknown etiology characterized by microvascular injury and deposition of collagen and other constituents of extracellular matrix in target organs [1] It is associated with a reduced life expectancy [2], significant functional disability [3,4,5] and depressive symptoms [6]. Koenig et al [10] reported that a high percentage of patients with Raynaud’s phenomenon (RaP) and an SSc marker autoantibody and/or typical SSc capillaroscopic findings and no manifestation other than puffy fingers and/or arthritis, develop definite SSc over time and proposed this condition be labeled “early (e)SSc” These patients, by definition, have no clinical manifestation other than RaP. The aim of the present study was to investigate HRQOL in patients affected by eSSc and to compare it with that of patients with undifferentiated connective tissue disease (UCTD) presenting only RaP and no other symptoms and that of healthy controls to determine whether the burden of secondary RaP and diagnosis of either eSSc or UCTD affects quality of life
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