Background Systemic sclerosis (SSc) is a connective tissue disease characterized by fibrosis of the skin and internal organs, pronounced alterations in the microvasculature and frequent cellular and humoral immunity abnormalities (1). In clinical practice, patients with SSc occasionally complain of pain in the extremities, such as dysesthesia, burning pain, and autonomic nervous symptoms (1,2).Few studies in SSc include assessments of pain. To date, there are no studies assessing the prevalence of neuropathic pain in SSc (2).
Objectives To study the prevalence of neuropathic pain (NP) in the SSc patients and associations between SSc clinical variables and pain in all patients with SSc and in limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets
Methods A total of 42 patients (36 females, 6 males) with a mean age 53.7±15.3 years, diagnosed according to American Rheumatism Association criteria for SSc were evaluated. Patients were classified into SSc subsets (lcSSc ordcSSc) according to the criteria for SSc by Leroy et al. Skin involvement was assessed with the modified Rodnan skin thickness score (MRSS) ranging from 0 to 51. The severity of Raynaud’s phenomenon was assessed with the number of patient-reported episodes in the past week. The severity disease was assessed by systemic sclerosis severity scale (SsSS) range from 0 to 36. The assessment of Hand Function was evaluated by Hand Mobility in SSc (HAMIS), range from 0 to 27 and the quality of life by Heath assessment questionnaire (HAQ). The neuropathic pain was evaluated by the DN4 neuropathic pain questionnaire.
Using the DN4 neuropathic pain questionnaire, we divided patients into two groups: first group – patients with non- NP (0-3 points), second group - patients with NP (4-8 points).
Results In SSc patients 67% (28 patients) had signs of NP by DN4. The prevalence of NP was higher in the patients who haddiffusecutaneous subsets. Patients with NP had longer disease duration (9.3±6.7years versus 3.8±2.3y, p<0.05) and a higher disease severity (15.3±9.2 versus 5.7±4.1 p<0,05). The modified Rodnan skin thickness score was higher in NP patients group (19.2±6.7 versus 8.6±4.6, p<0.01). The NP patients group had higher functional hand disability (12.6±9.6 versus 3.1±2.5 p<0.05). We found no significant differences between the two groups in quality of life andseverity of Raynaud’s phenomenon.
NP presented with numbness 94%, tingling 92%, electric shocks 83%, painful cold 81%, burning 77% and itching 56% in the second group. In neurological examination 79% had hypoesthesia to pinprick and 72% had hypoesthesia to touch.
Conclusions The pain syndrome in SSc is complex, because has multiples sources, with the neuropathic component prevailing in a 67% patients. SSc duration, skin thickness, diffuse cutaneous subsets and a disease severity had an impact in neuropathic severity, while severity of Raynaud’s phenomenon raynaud’s and HAQ is not linked to neuropathic pain.
More attention to pain and how to best manage it is needed in SSc.
Valentini G. The assessment of the patient with systemic sclerosis. Autoimmun Rev 2003;2:370–6.
Sokka T. Assessment of pain in patients with rheumatic diseases.Best Pract Res Clin Rheumatol 2003;17:427–49
Disclosure of Interest None Declared
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