Clinical Characteristic and Causes of Death of Patients with Scleroderma at Maharat Nakhon Ratchasima Hospital

Abstract

Background and Objective : Scleroderma is one of the complicated disease with high mortality rate. Most of the patients should be follow up at tertiary care hospital. Outcome of Scleroderma care in Maharat Nakhon Ratchasima Hospital was never analyzed before, We want to analyze in aspect of percentage of death, cause of death, factors which increase risk of death of Systemic sclerosis patients in Maharat Nakhon Ratchasima Hospital, tertiary  care Hospital in South- Northeastern part of  Thailand for improve outcome of treatment.

Method : Patients  fullfilling the American College of Rheumatology (ACR) criteria for diagnosis of Systemic sclerosis were retrospective review from Medical record between 2009 and 2011.

Results : A total of 96 patients were included, Sex ratio female to male was 3:1 and the mean age was 54±12.9 years. The mean disease duration until the study performed was 38.19±37.1 months. 63 % were Diffuse Cutaneous Systemic sclerosis (dcSsc). Internal organ involvement were Pulmonary Fibrosis (13.5%) ,Cardiac involvement (6.2%), Scleroderma Renal Crisis (4.2%) and Pulmonary Arterial Hypertension (4.2%). The patients with regular follow-up were 76%. Almost of the patients use National Health Insurance System about 90.6%. The death rate was 43.8% and only 35.4% could identified the caused of death.  The principal causes were infections (48%), non-ischemic cardiogenic cause (17%), cardiac and cerebrovascular disease (9%),and cancer (9%). The factors correlated with death were Diffuse Cutaneous  Systemic Sclerosis (dcSsc) HR 7.3 [95%CI 2.838-22.567] and duration of disease more than 4 years HR 3.2 [95%CI 1.178-9.217].

Conclusions : Our patients with Scleroderma had a high mortality rate. The most common cause of death was infection. Early detection and early management of this disease and its complication need to be considered.

Key Words : Systemic sclerosis, Death