Related Experiment Videos
[Evaluation of survival in mixed connective tissue disease (MCTD)]
Edit Bodolay1, János Gaál, Judit Végh
1Debreceni Egyetem, Orvos- és Egészségtudományi Centrum Altalános Orvostudományi Kar, Országos Immunológiai Koordinációs Intézet. bodolai@iiibel.dote.hu
Introduction:
179 patients with mixed connective tissue disease (MCTD) were follow-up, and the cause of death was analyzed in 12 died patients.
Patients And Methods:
The survival of 179 patients with MCTD was evaluated by using Kaplan-Meier's method. Clinically and immunological data of the patients were analyzed between 1 and 25 years follow-up period (mean: 13.1 +/- 5.5 years).
Results:
The five-year survival rate was 96.4%, 10-year survival rate was 93.9%, and the 15-year survival rate was 89.6%. The cause of death was pulmonary hypertension in 5 patients, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in 3 cases, infection in 3 cases (hepatitis C virus induced hepatic coma in 2 patients, Staphylococcus sepsis in one patient), and on one patient myocarditis. The pulmonary hypertension was the most serious prognostic factor.
Conclusion:
In patients with MCTD the pulmonary hypertension with endothelial cells proliferation and microangiopathy developed very quickly, and there was progressive and therapy resistant statement. The secondary virus and bacterial infections may develop in the patients who were followed-up the long term period. Their survival rate was better than the data in the literature. This fact may cause genetic-demographic factors, and the sequential follow-up of the patients.
Insights
Patients with mixed connective tissue disease (MCTD) have a good prognosis, with 15-year survival rates approaching 90%. Pulmonary hypertension is the leading cause of death, emphasizing its role as a critical prognostic factor in MCTD patient outcomes.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Context:
- Mixed Connective Tissue Disease (MCTD) is a complex autoimmune disorder.
- Long-term survival data and causes of mortality in MCTD patients are crucial for clinical management.
- Understanding prognostic factors is essential for improving patient outcomes.
Purpose:
- To analyze the survival rates and causes of death in a cohort of 179 patients with MCTD.
- To identify the most significant prognostic factors influencing mortality in MCTD.
- To compare the observed survival rates with existing literature data.
Summary:
- Kaplan-Meier survival analysis of 179 MCTD patients over a mean follow-up of 13.1 years revealed 5-, 10-, and 15-year survival rates of 96.4%, 93.9%, and 89.6%, respectively.
- Pulmonary hypertension was the primary cause of death in 5 patients, followed by thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (3 cases) and infections (3 cases).
- Pulmonary hypertension, characterized by rapid development, endothelial proliferation, and microangiopathy, emerged as the most critical prognostic factor.
Impact:
- The study highlights the significant impact of pulmonary hypertension on MCTD mortality.
- Findings suggest that improved survival rates in this cohort may be attributed to genetic-demographic factors and diligent patient follow-up.
- This research provides valuable insights for clinicians managing MCTD patients, particularly regarding early detection and management of pulmonary hypertension and infections.