Reumatología Clínica (English Edition) Reumatología Clínica (English Edition)
Original Article
Undifferentiated Connective Tissue Disease and Interstitial Lung Disease: Trying to Define Patterns
Enfermedad indiferenciada del tejido conectivo y enfermedad pulmonar intersticial: intentando definir patrones
María Laura Albertia,, , , Francisco Paulina, Heidegger Mateos Toledob, Martín Eduardo Fernándeza, Fabián Matías Caroa, Jorge Rojas-Serranob, Mayra Edith Mejíab
a Hospital María Ferrer, Ciudad Autónoma de Buenos Aires, Argentina
b Instituto Nacional de Enfermedades Respiratorias (INER), Distrito Federal, Mexico
Received 28 June 2016, Accepted 30 October 2016
Abstract
Objectives

To identify clinical or immunological features in patients with undifferentiated connective tissue disease (UCTD) associated interstitial lung disease (ILD), in order to group them and recognize different functional and high resolution computed tomography (HRCT) behavior.

Methods

Retrospective cohort study. Patients meeting Kinder criteria for UCTD were included. We defined the following predictive variables: ‘highly specific’ connective tissue disease (CTD) manifestations (Raynaud's phenomenon, dry eyes or arthritis), high antinuclear antibody (ANA) titer (above 1:320), and ‘specific’ ANA staining patterns (centromere, cytoplasmic and nucleolar patterns). We evaluated the following outcomes: change in the percentage of the predicted forced vital capacity (FVC%) during the follow-up period, and HRCT pattern.

Results

Sixty-six patients were included. Twenty-nine (43.94%) showed at least one ‘highly specific’ CTD manifestation, 16 (28.57%) had a ‘specific’ ANA staining pattern and 29 (43.94%) high ANA titer. Patients with ‘highly specific’ CTD manifestations were younger (mean [SD] 52 years [14.58] vs 62.08 years [9.46], P<.001), were more likely men (10.34% vs 48.65%, P<.001) and showed a smaller decline of the FVC% (median [interquartile range] 1% [−1 to 10] vs −6% [−16 to −4], P<.006). In the multivariate analysis, the presence of highly specific manifestations was associated with improvement in the FVC% (B coefficient of 13.25 [95% confidence interval, 2.41 to 24.09]). No association was observed in relation to the HRCT pattern.

Conclusion

The presence of ‘highly specific’ CTD manifestations was associated with female sex, younger age and better functional behavior. These findings highlight the impact of the clinical features in the outcome of patients with UCTD ILD.

Resumen
Objetivos

Agrupar a los pacientes con enfermedad pulmonar intersticial (EPI) asociada a enfermedad indiferenciada del tejido conectivo (EITC) según la presencia o no de ciertas manifestaciones clínicas o inmunológicas, esperando encontrar diferentes expresiones tomográficas o funcionales.

Métodos

Estudio de cohortes retrospectivas. Se incluyeron pacientes que cumplían criterios de Kinder para EITC. Se consideraron variables predictoras: manifestaciones «altamente específicas de enfermedad del tejido conectivo (ETC)» (Raynaud, xeroftalmia o artritis), títulos altos de anticuerpos antinucleares (ANA) (mayores a 1:320) y patrones específicos de ANA (centromérico, citoplásmico y nucleolar). El cambio en la capacidad vital forzada % (CVF%) en el tiempo y el patrón en TCAR fueron las variables de resultado estudiadas.

Resultados

Se incluyeron 66 pacientes. Veintinueve presentaron al menos una manifestación «altamente específica de ETC» (43,94%), 16 ANA específico (28,57%) y 29 ANA alto título (43,94%). Aquellos con manifestaciones «altamente específicas de ETC» presentaron menor frecuencia de sexo masculino (10,34% vs 48,65%, p<0,001), menor edad en años (media 52 [DE14,58] vs 62,08 [9,46], p<0,001) y menor mediana de declinación de CVF% (1[RIC −1 a 10] vs −6 [RIC −16 a −4], p<0,006). En el análisis de regresión lineal múltiple la presencia de manifestaciones «altamente específicas de ETC» se asoció con mejoría en CVF% (coeficiente B de 13,25 [IC 95% 2,41 a 24,09]). No encontramos asociaciones en cuanto al patrón en TACAR.

Conclusiones

La presencia de manifestaciones «altamente específicas de ETC» se asoció con sexo femenino, menor edad al inicio y una evolución más favorable en cuanto a la CVF%, lo cual evidencia el impacto de las manifestaciones clínicas en la evolución de estos pacientes.

Keywords
Interstitial lung diseases, Connective tissue diseases, Autoimmune diseases, Antinuclear antibodies, Raynaud phenomenon
Palabras clave
Enfermedad pulmonar intersticial, Enfermedad del tejido conectivo, Enfermedades autoinmunes, Anticuerpos antinucleares, Fenómeno de Raynaud

Article

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