TY - JOUR
T1 - Vascular complications in systemic sclerosis
T2 - a prospective cohort study
AU - Mecoli, Christopher A.
AU - Shah, Ami A.
AU - Boin, Francesco
AU - Wigley, Fredrick M.
AU - Hummers, Laura K.
N1 - Funding Information:
Funding Research reported in this publication was supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health under Award Number T32AR048522. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. C.M. is a Jerome L. Greene Foundation Scholar and the Foundation has provided support for his work. This work was also supported by NIH grants 5K23AR52742-5 from NIAMS and P50-HL084946-01 from NHLBI. This work was also supported by the Scleroderma Research Foundation, Martha McCrory professorship, The John Staurulakis Endowed Scholar in Rheumatology (LKH), and Chresanthe Staurulakis Memorial Fund for Scleroderma Research.
Publisher Copyright:
© 2018, International League of Associations for Rheumatology (ILAR).
PY - 2018/9/1
Y1 - 2018/9/1
N2 - Two major complications in scleroderma patients that cause substantial morbidity and mortality are ischemic digital lesions (DL) and pulmonary hypertension (PH). The clinician’s ability to predict which patients will develop these complications is imperfect. We conducted a prospective observational cohort study of 300 patients with scleroderma who were followed for at least a 5-year period. At baseline, patients lacked evidence of PH and were without a current DL. At each 6-month visit, the patient was examined for signs/symptoms of PH and/or a DL. The primary outcomes were (1) PH defined as a mean pulmonary artery pressure ≥ 25 mmHg by right heart catheterization and (2) ≥ 1 DL defined as new onset of severe vascular compromise. Thirty patients (10%) developed PH (11 group 1/PAH, 4 group II, 15 group III) and 69 developed DL. The average time from enrollment until diagnosis of PH was 3.2 ± 2 years. In multivariable analyses, patients who developed PH were more likely to have diffuse disease (HR 3.2, p = 0.004), a forced vital capacity (FVC)/diffusing capacity of the lungs for carbon monoxide (DLCO) ratio > 1.6 (HR 1.7, p = 0.008), and elevated RVSP (HR = 1.07, p = 0.007). Patients who developed PAH were more likely to have a FVC/DLCO ratio > 1.6 (HR = 5.8, p = 0.014), and patients who developed group III PH were less likely to have an elevated FVC (HR = 0.92, p = 0.001). Patients were more likely to develop a DL if they had a history of prior DL (HR = 7.0, p < 0.001), or were men (HR = 2.3, p = 0.007). In a prevalent cohort of scleroderma patients, individuals who develop PH or DL have simple to measure clinical features that can predict these complications years before they occur.
AB - Two major complications in scleroderma patients that cause substantial morbidity and mortality are ischemic digital lesions (DL) and pulmonary hypertension (PH). The clinician’s ability to predict which patients will develop these complications is imperfect. We conducted a prospective observational cohort study of 300 patients with scleroderma who were followed for at least a 5-year period. At baseline, patients lacked evidence of PH and were without a current DL. At each 6-month visit, the patient was examined for signs/symptoms of PH and/or a DL. The primary outcomes were (1) PH defined as a mean pulmonary artery pressure ≥ 25 mmHg by right heart catheterization and (2) ≥ 1 DL defined as new onset of severe vascular compromise. Thirty patients (10%) developed PH (11 group 1/PAH, 4 group II, 15 group III) and 69 developed DL. The average time from enrollment until diagnosis of PH was 3.2 ± 2 years. In multivariable analyses, patients who developed PH were more likely to have diffuse disease (HR 3.2, p = 0.004), a forced vital capacity (FVC)/diffusing capacity of the lungs for carbon monoxide (DLCO) ratio > 1.6 (HR 1.7, p = 0.008), and elevated RVSP (HR = 1.07, p = 0.007). Patients who developed PAH were more likely to have a FVC/DLCO ratio > 1.6 (HR = 5.8, p = 0.014), and patients who developed group III PH were less likely to have an elevated FVC (HR = 0.92, p = 0.001). Patients were more likely to develop a DL if they had a history of prior DL (HR = 7.0, p < 0.001), or were men (HR = 2.3, p = 0.007). In a prevalent cohort of scleroderma patients, individuals who develop PH or DL have simple to measure clinical features that can predict these complications years before they occur.
KW - Cohort studies
KW - Pulmonary hypertension
KW - Scleroderma
KW - Wounds and injuries
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U2 - 10.1007/s10067-018-4148-5
DO - 10.1007/s10067-018-4148-5
M3 - Article
C2 - 29804150
AN - SCOPUS:85047420991
SN - 0770-3198
VL - 37
SP - 2429
EP - 2437
JO - Clinical rheumatology
JF - Clinical rheumatology
IS - 9
ER -