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Circulation. 2017 Sep 19;136(12):1114-1122. doi: 10.1161/CIRCULATIONAHA.116.027094. Epub 2017 Jul 12.

Long-Term Outcomes and Prognostic Factors of Complications in Takayasu Arteritis: A Multicenter Study of 318 Patients.

Author information

1
From Sorbonne Universités, UPMC Univ Paris 06, Inflammation-Immunopathology-Biotherapy Department, France (C.C., P.C, D.S.); INSERM, Paris, France (C.C., P.C, D.S.); Centre National de la Recherche Scientifique, Paris, France (C.C., P.C, D.S.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires, Paris, France (C.C., Y.F., P.C, D.S.); AP-HP, SBIM, Hôpital Saint-Louis, Université Paris Diderot, France (L.B., M.R.-R.); INSERM, ECSTRA Team, Paris, France (L.B., M.R.-R.); Hôpital Claude Huriez, Service de Médecine Interne, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, CHRU, Université de Lille, France (M.L., E.H.); AP-HP, Service de Médecine Interne, Hôpital Saint-Antoine, UPMC, Paris France (A.M.); Service de Médecine Interne, Hôpital Foch, Suresnes, France (J.-E.K.); Service de Médecine Interne, CHU de Rouen, France (Y.B.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Chirurgie Vasculaire, UPMC, Paris, France (L.C., F.K.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Département d'Imagerie CardioVasculaire et de Radiologie Interventionnelle, UPMC, INSERM-Centre National de la Recherche Scientifique-LIB, Paris, France (P.C.); AP-HP, Hôpital Européen Georges-Pompidou, Service de Médecine Vasculaire, INSERM UMR970, PARCC, Centre de Référence des Maladies Vasculaires Rares, DHU Pathologies Artérielles Rares et Communes, Hôpitaux Universitaires Paris Ouest, Université Paris Descartes, Sorbonne Paris Cité, France (E.M., T.M.).
2
From Sorbonne Universités, UPMC Univ Paris 06, Inflammation-Immunopathology-Biotherapy Department, France (C.C., P.C, D.S.); INSERM, Paris, France (C.C., P.C, D.S.); Centre National de la Recherche Scientifique, Paris, France (C.C., P.C, D.S.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires, Paris, France (C.C., Y.F., P.C, D.S.); AP-HP, SBIM, Hôpital Saint-Louis, Université Paris Diderot, France (L.B., M.R.-R.); INSERM, ECSTRA Team, Paris, France (L.B., M.R.-R.); Hôpital Claude Huriez, Service de Médecine Interne, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, CHRU, Université de Lille, France (M.L., E.H.); AP-HP, Service de Médecine Interne, Hôpital Saint-Antoine, UPMC, Paris France (A.M.); Service de Médecine Interne, Hôpital Foch, Suresnes, France (J.-E.K.); Service de Médecine Interne, CHU de Rouen, France (Y.B.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Chirurgie Vasculaire, UPMC, Paris, France (L.C., F.K.); AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Département d'Imagerie CardioVasculaire et de Radiologie Interventionnelle, UPMC, INSERM-Centre National de la Recherche Scientifique-LIB, Paris, France (P.C.); AP-HP, Hôpital Européen Georges-Pompidou, Service de Médecine Vasculaire, INSERM UMR970, PARCC, Centre de Référence des Maladies Vasculaires Rares, DHU Pathologies Artérielles Rares et Communes, Hôpitaux Universitaires Paris Ouest, Université Paris Descartes, Sorbonne Paris Cité, France (E.M., T.M.). david.saadoun@aphp.fr.

Abstract

BACKGROUND:

Because of the wide variation in the course of Takayasu arteritis (TA), predicting outcome is challenging. We assess long-term outcome and prognosis factors for vascular complications in patients with TA.

METHODS:

A retrospective multicenter study of characteristics and outcomes of 318 patients with TA fulfilling American College of Rheumatology and Ishikawa criteria was analyzed. Factors associated with event-free survival, relapse-free survival, and incidences of vascular complications were assessed. Risk factors for vascular complications were identified in a multivariable model.

RESULTS:

The median age at TA diagnosis was 36 [25-47] years, and 276 patients (86.8%) were women. After a median follow-up of 6.1 years, relapses were observed in 43%, vascular complications in 38%, and death in 5%. Progressive clinical course was observed in 45%, carotidodynia in 10%, and retinopathy in 4%. The 5- and 10-year event-free survival, relapse-free survival, and complication-free survival were 48.2% (42.2; 54.9) and 36.4% (30.3; 43.9), 58.6% (52.7; 65.1) and 47.7% (41.2; 55.1), and 69.9% (64.3; 76.0) and 53.7% (46.8; 61.7), respectively. Progressive disease course (P=0.018) and carotidynia (P=0.036) were independently associated with event-free survival. Male sex (P=0.048), elevated C-reactive protein (P=0.013), and carotidynia (P=0.003) were associated with relapse-free survival. Progressive disease course (P=0.017), thoracic aorta involvement (P=0.009), and retinopathy (P=0.002) were associated with complication-free survival.

CONCLUSIONS:

This nationwide study shows that 50% of patients with TA will relapse and experience a vascular complication ≤10 years from diagnosis. We identified specific characteristics that identified those at highest risk for subsequent vascular complications.

KEYWORDS:

Takayasu arteritis; outcome; prognosis; relapse; vasculitis

[Indexed for MEDLINE]

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