Clinical course, prognosis, and causes of death in mixed connective tissue disease

Agota Hajas, Peter Szodoray, Britt Nakken, J. Gaál, Eva Zold, Renata Laczik, Nora Demeter, G. Nagy, Z. Szekanecz, M. Zeher, G. Szegedi, E. Bodolay

Research output: Contribution to journalArticle

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Abstract

Objective. To study the survival rate and prognostic indicators of mixed connective tissue disease (MCTD) in a Hungarian population. Methods. Two hundred eighty patients with MCTD diagnosed between 1979 and 2011 were followed prospectively. Clinical features, autoantibodies, and mortality data were assessed. Prognostic factors for survival were investigated and survival was calculated from the time of the diagnosis by Kaplan-Meier method. Results. A total of 22 of 280 patients died: the causes of death were pulmonary arterial hypertension (PAH) in 9 patients, thrombotic thrombocytopenic purpura in 3, infections in 3, and cardiovascular events in 7. The 5, 10, and 15-year survival rates after the diagnosis was established were 98%, 96%, and 88%, respectively. The deceased patients were younger at the diagnosis of MCTD compared to patients who survived (35.5 ± 10.4 vs 41.8 ± 10.7 yrs; p <0.03), while there was no difference in the duration of the disease (p = 0.835). Our cohort study showed that the presence of cardiovascular events (p <0.0001), esophageal hypomotility (p = 0.04), serositis (p <0.001), secondary antiphospholipid syndrome (p = 0.039), and malignancy (p <0.001) was significantly higher in the deceased patients with MCTD. The presence of anticardiolipin (p = 0.019), anti-β2-glycoprotein I (p = 0.002), and antiendothelial cell antibodies (p = 0.002) increased the risk of mortality. Conclusion. Overall, PAH remained the leading cause of death in patients with MCTD. The prevalence of cardiovascular morbidity and mortality, malignancy, and thrombotic events increased during the disease course of MCTD. The presence of antiphospholipid antibodies raised the risk of mortality.

Original languageEnglish
Pages (from-to)1134-1142
Number of pages9
JournalJournal of Rheumatology
Volume40
Issue number7
DOIs
Publication statusPublished - Jul 2013

Fingerprint

Mixed Connective Tissue Disease
Cause of Death
Mortality
Pulmonary Hypertension
Survival Rate
Serositis
Thrombotic Thrombocytopenic Purpura
Antiphospholipid Antibodies
Antiphospholipid Syndrome
Survival
Autoantibodies
Neoplasms
Glycoproteins
Cohort Studies
Morbidity
Infection

Keywords

  • Antiphospholipid Syndrome
  • Cardiovascular Events
  • Clinical Course
  • Mixed Connective Tissue Disease
  • Prognosis
  • Survival

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

Cite this

Clinical course, prognosis, and causes of death in mixed connective tissue disease. / Hajas, Agota; Szodoray, Peter; Nakken, Britt; Gaál, J.; Zold, Eva; Laczik, Renata; Demeter, Nora; Nagy, G.; Szekanecz, Z.; Zeher, M.; Szegedi, G.; Bodolay, E.

In: Journal of Rheumatology, Vol. 40, No. 7, 07.2013, p. 1134-1142.

Research output: Contribution to journalArticle

Hajas, Agota ; Szodoray, Peter ; Nakken, Britt ; Gaál, J. ; Zold, Eva ; Laczik, Renata ; Demeter, Nora ; Nagy, G. ; Szekanecz, Z. ; Zeher, M. ; Szegedi, G. ; Bodolay, E. / Clinical course, prognosis, and causes of death in mixed connective tissue disease. In: Journal of Rheumatology. 2013 ; Vol. 40, No. 7. pp. 1134-1142.
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AU - Hajas, Agota

AU - Szodoray, Peter

AU - Nakken, Britt

AU - Gaál, J.

AU - Zold, Eva

AU - Laczik, Renata

AU - Demeter, Nora

AU - Nagy, G.

AU - Szekanecz, Z.

AU - Zeher, M.

AU - Szegedi, G.

AU - Bodolay, E.

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N2 - Objective. To study the survival rate and prognostic indicators of mixed connective tissue disease (MCTD) in a Hungarian population. Methods. Two hundred eighty patients with MCTD diagnosed between 1979 and 2011 were followed prospectively. Clinical features, autoantibodies, and mortality data were assessed. Prognostic factors for survival were investigated and survival was calculated from the time of the diagnosis by Kaplan-Meier method. Results. A total of 22 of 280 patients died: the causes of death were pulmonary arterial hypertension (PAH) in 9 patients, thrombotic thrombocytopenic purpura in 3, infections in 3, and cardiovascular events in 7. The 5, 10, and 15-year survival rates after the diagnosis was established were 98%, 96%, and 88%, respectively. The deceased patients were younger at the diagnosis of MCTD compared to patients who survived (35.5 ± 10.4 vs 41.8 ± 10.7 yrs; p <0.03), while there was no difference in the duration of the disease (p = 0.835). Our cohort study showed that the presence of cardiovascular events (p <0.0001), esophageal hypomotility (p = 0.04), serositis (p <0.001), secondary antiphospholipid syndrome (p = 0.039), and malignancy (p <0.001) was significantly higher in the deceased patients with MCTD. The presence of anticardiolipin (p = 0.019), anti-β2-glycoprotein I (p = 0.002), and antiendothelial cell antibodies (p = 0.002) increased the risk of mortality. Conclusion. Overall, PAH remained the leading cause of death in patients with MCTD. The prevalence of cardiovascular morbidity and mortality, malignancy, and thrombotic events increased during the disease course of MCTD. The presence of antiphospholipid antibodies raised the risk of mortality.

AB - Objective. To study the survival rate and prognostic indicators of mixed connective tissue disease (MCTD) in a Hungarian population. Methods. Two hundred eighty patients with MCTD diagnosed between 1979 and 2011 were followed prospectively. Clinical features, autoantibodies, and mortality data were assessed. Prognostic factors for survival were investigated and survival was calculated from the time of the diagnosis by Kaplan-Meier method. Results. A total of 22 of 280 patients died: the causes of death were pulmonary arterial hypertension (PAH) in 9 patients, thrombotic thrombocytopenic purpura in 3, infections in 3, and cardiovascular events in 7. The 5, 10, and 15-year survival rates after the diagnosis was established were 98%, 96%, and 88%, respectively. The deceased patients were younger at the diagnosis of MCTD compared to patients who survived (35.5 ± 10.4 vs 41.8 ± 10.7 yrs; p <0.03), while there was no difference in the duration of the disease (p = 0.835). Our cohort study showed that the presence of cardiovascular events (p <0.0001), esophageal hypomotility (p = 0.04), serositis (p <0.001), secondary antiphospholipid syndrome (p = 0.039), and malignancy (p <0.001) was significantly higher in the deceased patients with MCTD. The presence of anticardiolipin (p = 0.019), anti-β2-glycoprotein I (p = 0.002), and antiendothelial cell antibodies (p = 0.002) increased the risk of mortality. Conclusion. Overall, PAH remained the leading cause of death in patients with MCTD. The prevalence of cardiovascular morbidity and mortality, malignancy, and thrombotic events increased during the disease course of MCTD. The presence of antiphospholipid antibodies raised the risk of mortality.

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KW - Mixed Connective Tissue Disease

KW - Prognosis

KW - Survival

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