TY - JOUR
T1 - The role of nailfold capillary dropout on mortality in systemic sclerosis.
AU - Tieu, Joanna
AU - Hakendorf, Paul
AU - Woodman, Richard
AU - Patterson, Karen
AU - Walker, Jenny
AU - Roberts-Thomson, Peter
PY - 2018/5
Y1 - 2018/5
N2 - Background: Semi-quantitative wide-field nailfold capillaroscopy (NFC) is a simple technique with proven utility in the early diagnosis of systemic sclerosis (SSc). Its role in prognosis, however, remains uncertain. Aim: To investigate the possible utility of NFC in predicting survival. Methods: Patients with SSc listed on the South Australian Scleroderma Register (SASR) with prior NFC performed at Flinders Medical Centre from 1991 to 2015 were included in this study. Baseline demographic data, diagnosis, scleroderma antibody status and mortality status were also collected for each patient. Results: The cohort consisted of 99 patients with limited cutaneous SSc, 30 patients with diffuse cutaneous SSc and 23 with an overlap scleroderma syndrome. Fifty-six patients died during the period of study (censured end June 2015). Patients with diffuse scleroderma had significantly greater capillary dropout compared with limited and overlap scleroderma (P < 0.001). In univariate analysis, both capillary dropout scores (log-rank χ2 = 8.75, P = 0.003) and antibody status (log-rank χ2 = 13.94, P = 0.003) were associated with mortality. ANOVA showed a significant association between antibody status and capillary dropout (P < 0.001). In Cox regression, adjustment for capillary dropout attenuated the impact of autoantibody group on survival. Conclusions: Nailfold capillary dropout was significantly associated with mortality and the severity of dropout attenuates survival dictated by antibody status. Together these observations support the hypothesis that capillary dropout is on the causal pathway between induction of scleroderma associated autoantibodies and mortality.
AB - Background: Semi-quantitative wide-field nailfold capillaroscopy (NFC) is a simple technique with proven utility in the early diagnosis of systemic sclerosis (SSc). Its role in prognosis, however, remains uncertain. Aim: To investigate the possible utility of NFC in predicting survival. Methods: Patients with SSc listed on the South Australian Scleroderma Register (SASR) with prior NFC performed at Flinders Medical Centre from 1991 to 2015 were included in this study. Baseline demographic data, diagnosis, scleroderma antibody status and mortality status were also collected for each patient. Results: The cohort consisted of 99 patients with limited cutaneous SSc, 30 patients with diffuse cutaneous SSc and 23 with an overlap scleroderma syndrome. Fifty-six patients died during the period of study (censured end June 2015). Patients with diffuse scleroderma had significantly greater capillary dropout compared with limited and overlap scleroderma (P < 0.001). In univariate analysis, both capillary dropout scores (log-rank χ2 = 8.75, P = 0.003) and antibody status (log-rank χ2 = 13.94, P = 0.003) were associated with mortality. ANOVA showed a significant association between antibody status and capillary dropout (P < 0.001). In Cox regression, adjustment for capillary dropout attenuated the impact of autoantibody group on survival. Conclusions: Nailfold capillary dropout was significantly associated with mortality and the severity of dropout attenuates survival dictated by antibody status. Together these observations support the hypothesis that capillary dropout is on the causal pathway between induction of scleroderma associated autoantibodies and mortality.
KW - actuarial survival
KW - nailfold capillaroscopy
KW - nailfold capillary dropout
KW - prognosis
KW - scleroderma
KW - systemic sclerosis
UR - http://www.scopus.com/inward/record.url?scp=85046377336&partnerID=8YFLogxK
U2 - 10.1111/imj.13745
DO - 10.1111/imj.13745
M3 - Article
VL - 48
SP - 517
EP - 523
JO - Internal Medicine Journal
JF - Internal Medicine Journal
SN - 0004-8291
IS - 5
ER -