[Evaluation of survival in mixed connective tissue disease (MCTD)].
BACKGROUND
179 patients with mixed connective tissue disease (MCTD) were follow-up, and the cause of death was analyzed in 12 died patients.


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.


CONCLUSIONS
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.
Bodolay
Edit
E
Debreceni Egyetem, Orvos- és Egészségtudományi Centrum Altalános Orvostudományi Kar, Országos Immunológiai Koordinációs Intézet. bodolai@iiibel.dote.hu
Gaál
János
J
Végh
Judit
J
Soltész
Pál
P
Szodoray
Péter
P
Lakos
Gabriella
G
Nemes
Zoltán
Z
Dezsö
Balázs
B
Szegedi
Gyula
G
D000328:Adult; D000368:Aged; D017153:Antibodies, Anticardiolipin / Q000097:blood; D002423:Cause of Death; D004727:Endothelium / Q000166:cytology / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D006976:Hypertension, Pulmonary / Q000209:etiology; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000276:immunology / Q000401:mortality*; D011379:Prognosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology; D016019:Survival Analysis; D015996:Survival Rate; D013997:Time Factors
D017153:Antibodies, Anticardiolipin