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Methods: Patients with a definite CS, prospectively followed in a single centre, were included. SC was diagnosed using the following procedures: electrocardiogram, myocardial echography, MRI and scintigraphy. The diagnosis of CS was defined by the evidence of myocardial abnormalities to at least two different procedures. Patients with a CS were analyzed regarding their clinical, diagnostic, therapeutic and outcome features.
Results: Out of a cohort of 502 patients with a systemic sarcoidosis, 126 patients (25%) presented a CS: mean age 41 ± 12 years; male gender 59%; Caucasian 70%; Black 28% and Asiatic 2%. Extracardiac sarcoidosis involvement included: chest 79%, central nervous system (CNS) 44%, peripheral lymph nodes 31% and skin 25%. Diagnosis:Dyspnea was present in 19% of patients. ECG abnormalities were present in 64% of patients including conduction (54%) and rhythm (63%) disturbances. The echocardiography (n = 125) showed pathological signs for 88 patients (70%): hypokinesia (33%), low left ventricular ejection fraction (22%), interventricular septum thickness (10%). Myocardial scintigraphy (n = 112) was pathological for 81 patients (72%) with localized (61%) and diffuse (12%) defects. Cardiac MRI (n = 64) showed abnormal signals and gadolinium uptake in 37 patients (58%). Morphological and electrocardiographic troubles were both present in 80 patients (63.5%) and 46 patients (36.5%) had isolated morphological abnormalities. The highest diagnostic accuracy was reached between MRI and echocardiography (67%), followed by MRI and scintigraphy (63%) and echocardiography and scintigraphy (56%).Treatment and outcome: A first line treatment with steroids was initiated for 111 patients (88%), among whom 48 patients (43%) received an additional immunosuppressive drug (ISD) including methotrexate (71%), cyclophosphamide (23%), mycophenolate mofetil (6%). After a mean follow-up of 74.8 ± 63 months, 122 patients were evaluable: 89 patients (73%) recovered, 28 patients (23%) were stable, and 5 patients (4%) worsened. After multivariate analysis, two factors were associated with the absence of CS recovery: CNS involvement (OR = 3.2; 95%CI: 1.3-7.5; P = 0.0025) and a left heart insufficiency (OR = 2.5; 95%CI = 1-6; P = 0.05). Thirteen patients (10%) died and only two deaths (1.6%) were imputable to CS. Conclusion : Our results show that CS has a good prognosis under steroids alone or in association with an ISD. Up to 70% of patients may recover and the CS related death rate is less than 2%. The absence of a stable recovery is associated with the severity of systemic sarcoidosis, i.e. left heart insufficiency and CNS involvement.
Disclosure: D. Sène, None; C. Chapelon-Abric, None; D. Saadoun, None; A. Mathian, None; N. Costedoat-Chalumeau, None; J. Haroche, None; D. Li Thi Huong-Boutin, None; J. -. C. Piette, None; P. P. Cacoub, None; Z. Amoura, None.