ABSTRACT
Antecedentes: La enfermedad pulmonar intersticial (EPI) es una complicación frecuente en la esclerosis sistémica (ES) progresiva, presente en el 25-90% de los pacientes. Objetivo: Evaluar si los niveles séricos de propéptido aminoterminal de procolágeno tipos i y iii (PINP y PIIINP) se correlacionan con la gravedad de la EPI en mujeres mexicanas con ES. Métodos: En 33 pacientes con ES se evaluaron las características de la enfermedad, anticuerpos antitopoisomerasa (topo i), pruebas de función pulmonar y tomografía computarizada de alta resolución (TCAR). Diecinueve pacientes tenían ES + EPI y 14 no presentaban afectación pulmonar (ES sin EPI). Se compararon con 45 controles sanos. Se evaluaron PINP y PIIINP en los 3 grupos. Resultados: El grupo ES tuvo mayores niveles de PINP y PIIINP que el control (p = 0,001 y p < 0,001, respectivamente). Las pacientes ES + EPI habían presentado la enfermedad más años que las ES sin EPI (p = 0,005), tenían mayor puntuación en el índice modificado de Rodnan (p < 0,001), puntuación alta en el índice de evaluación de discapacidad (p < 0,001), mayores niveles de antitopoisomerasa i (p < 0,001), PINP (49,28 ± 28,63 vs. 32,12 ± 18,58 μg/l, p = 0,05), y PIIINP (4,33 ± 1,03 vs. 2,67 ± 1,26 μg/l, p < 0,001). La gravedad de la EPI en TACAR se correlacionó con los niveles de PINP (r = 0,388, p = 0,03) y PIIINP (p = 0,594, p < 0,001). En el análisis ajustado, la gravedad de la EPI se asoció con la duración de la enfermedad (p = 0,037) y con los niveles de PIIINP (p = 0,038) y de antitopoisomerasa i (p = 0,045). Conclusiones: El PINP y el PIIINP son marcadores útiles para la ES + EPI grave. Esto apoya su uso clínico para el seguimiento de esta complicación
Background: Interstitial lung disease (ILD) is a frequent complication in progressive systemic sclerosis (SSc), being present in 25% to 90% of cases. Objectives: To evaluate whether serum levels of procollagen type i and iii aminoterminal propeptide (PINP and PIIINP) correlate with severity and patterns of ILD in Mexican women with SSc. Methods: Thirty three SSc patients were assessed for disease characteristics and anti-topoisomerase antibodies (topo i), and also underwent pulmonary function tests and high-resolution computed tomography (HRCT). Nineteen patients had ILD + SSc, and 14 had no lung involvement (no ILD-SSc); data were compared with those from 45 healthy controls. PINP and PIIINP were assessed in all 3 groups. Results: Patients with SSc had higher PINP and PIIINP vs controls (P = .001, P < .001, respectively). Compared to no ILD-SSc patients, those with ILD + SSc had longer disease duration in years (P = .005), higher modified Rodnan skin score (P < .001), higher Health Assessment Questionnaire-Disability-Index scores (P < .001), higher topo i U/mL (P < .001), PINP (49.28 ± 28.63 vs. 32.12 ± 18.58 g/L, P = .05), and PIIINP (4.33 ± 1.03 vs. 2.67 ± 1.26 g/L, P < .001) levels. ILD severity based on total HRCT correlated with PINP (r = .388, P = .03) and PIIINP (P = .594, P < .001). On adjusted analysis, ILD severity was associated with disease duration (P = .037), PIIINP (P = .038), and topo i (P = .045). Conclusions: PINP and PIIINP are useful markers for severe ILD + SSc, suggesting they could play a role in the follow-up of this complication in SSc
Subject(s)
Adult , Female , Humans , Lung Diseases, Interstitial/diagnosis , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Pulmonary Fibrosis/diagnosis , Procollagen , Collagen Type I/blood , Collagen Type III/blood , Epidemiological Monitoring/trends , Lung/physiology , Hypertension, Pulmonary/diagnosis , Biomarkers , Tomography, X-Ray Computed , Respiratory Function Tests , Severity of Illness Index , Quality of Life , Prognosis , Cross-Sectional Studies , Mexico/epidemiologyABSTRACT
BACKGROUND: Interstitial lung disease (ILD) is a frequent complication in progressive systemic sclerosis (SSc), being present in 25% to 90% of cases. OBJECTIVES: To evaluate whether serum levels of procollagen typei and iii aminoterminal propeptide (PINP and PIIINP) correlate with severity and patterns of ILD in Mexican women with SSc. METHODS: Thirty three SSc patients were assessed for disease characteristics and anti-topoisomerase antibodies (topoi), and also underwent pulmonary function tests and high-resolution computed tomography (HRCT). Nineteen patients had ILD+SSc, and 14 had no lung involvement (no ILD-SSc); data were compared with those from 45 healthy controls. PINP and PIIINP were assessed in all 3 groups. RESULTS: Patients with SSc had higher PINP and PIIINP vs controls (P=.001, P<.001, respectively). Compared to no ILD-SSc patients, those with ILD+SSc had longer disease duration in years (P=.005), higher modified Rodnan skin score (P<.001), higher Health Assessment Questionnaire-Disability-Index scores (P<.001), higher topoi U/mL (P<.001), PINP (49.28±28.63 vs. 32.12±18.58µg/L, P=.05), and PIIINP (4.33±1.03 vs. 2.67±1.26µg/L, P<.001) levels. ILD severity based on total HRCT correlated with PINP (r=.388, P=.03) and PIIINP (P=.594, P<.001). On adjusted analysis, ILD severity was associated with disease duration (P=.037), PIIINP (P=.038), and topoi (P=.045). CONCLUSIONS: PINP and PIIINP are useful markers for severe ILD+SSc, suggesting they could play a role in the follow-up of this complication in SSc.
