Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone

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Abstract

Cardiac involvement is an important prognostic factor in sarcoidosis, but reliable indicators of mortality risk in cardiac sarcoidosis are unstudied in a large number of patients. To determine the significant predictors of mortality and to assess the efficacy of corticosteroids, we analyzed clinical findings, treatment, and prognosis in 95 Japanese patients with cardiac sarcoidosis. Twenty of these 95 patients had cardiac sarcoidosis proven by autopsy; none of these patients had received corticosteroids. We assessed 12 clinical variables as possible predictors of mortality by Cox proportional hazards model in 75 steroid-treated patients. During the mean follow-up of 68 months, 29 patients (73%) died of congestive heart failure and 11 (27%) experienced sudden death. Kaplan-Meier survival curves showed 5-year survival rates of 75% in the steroid-treated patients and of 89% in patients with a left ventricular ejection fraction ≥50%, whereas there was only 10% 5-year survival rate in autopsy subjects. There was no significant difference in survival curves of patients treated with a high initial dose (>30 mg) and a low initial dose (≥30 mg) of prednisone. Multivariate analysis identified New York Heart Association functional class (hazard ratio 7.72 per class I increase, p = 0.0008), left ventricular end-diastolic diameter (hazard ratio 2.60/10 mm increase, p = 0.02), and sustained ventricular tachycardia (hazard ratio 7.20, p = 0.03) as independent predictors of mortality. In conclusion, the severity of heart failure was one of the most significant independent predictors of mortality for cardiac sarcoidosis. Starting corticosteroids before the occurrence of systolic dysfunction resulted in an excellent clinical outcome. A high initial dose of prednisone may not be essential for treatment of cardiac sarcoidosis.

Section snippets

Patient selection

Patients who were diagnosed with cardiac sarcoidosis at our institution and collaborating hospitals between 1984 and 1996 were analyzed retrospectively. Clinical details and follow-up information for patients from collaborating centers were obtained by questionnaire, and if needed, by telephone or letter. Clinical data included electrocardiograms, echocardiograms, radionuclide examinations, hemodynamics, contrast left ventriculograms, and endomyocardial biopsies. We obtained records concerning

Clinical characteristics of the study population

Age and sex distribution of all patients is shown in Figure 1. Cardiac sarcoidosis occurred most frequently in middle-aged women. Patient characteristics are listed in Table 1. Subjects with autopsy-proven sarcoidosis had significantly reduced systolic performance, severe heart failure symptoms, a higher incidence of complete atrioventricular block and sustained ventricular tachycardia, and an enlarged left ventricle compared with the steroid-treated patients. During follow-up, 40 died from

Recent clinical profile in cardiac sarcoidosis

In the present study, cardiac sarcoidosis most frequently occurred in women between 50 and 65 years of age. This same tendency has been noted in other Japanese studies.11, 12 In Western countries, there is no definite male or female predominance in the occurrence of the syndrome and differences in mean age; cardiac involvement in sarcoidosis is more predominant among young persons.3, 13, 14, 15 A racial difference should be considered in cardiac sarcoidosis.

In a previous report by Roberts et al,

Acknowledgements

The investigators thank the physicians at the following institutions who provides patient data: Gifu University: Hisayoshi Fujiwara, Kenji Nagashima, Hiroyasu Itoh; Hokaido University: Nagara Tamaki; Kyoto University: Masatoshi Fujita; Komatsujima Red Cross Hospital: Yoshikazu Hiasa; Keio University: Satoshi Ogawa; Asahikawa Medical College: Kenjiro Kikuchi; Tenri Yorozu Sodanshyo Hospital: Hiromitsu Gen; Kanazawa Medical University: Eiji Murakami; Toho University Ohashi Hospital: Michiro

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This study was supported in part by a research grant from the Intractable Disease Division, Public Health Bureau, Ministry of Health Welfare, Tokyo, Japan.

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