Cardiac Sarcoidosis

12/16/02 (Chun)

Question: What are the diagnostic criteria for cardiac sarcoidosis?

 Link Directly to Fulltext Article at Science Direct

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Unique Identifier:11703997

Authors: Yazaki Y. Isobe M. Hiroe M. Morimoto S. Hiramitsu S. Nakano T. Izumi T. Sekiguchi M. The Central Japan Heart Study Group.

Institution: First Department of Internal Medicine, Shinshyu University, Matsumoto, Japan. yoshiy@athena.ocn.ne.jp

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

Source: American Journal of Cardiology. 88(9):1006-10, 2001 Nov 1.

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 > or = 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 (> or = 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. CAS Registry/EC Number 0 (Glucocorticoids, Synthetic). 53-03-2 (Prednisone).

Link Directly to Fulltext article in Ovid

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Unique Identifier:11584179

Authors: Rizzato G.

Institution: Ambulatorio della Sarcoidosi, Vergani Dept, Niguarda Hospital, Via Juvara 9, 20129 Milan, Italy.rizzatog@tin.it

Title: Extrapulmonary presentation of sarcoidosis. [Review] [14 refs]

Source: Current Opinion in Pulmonary Medicine. 7(5):295-7, 2001 Sep.

Abstract: Sarcoidosis is a systemic disease that usually has a pulmonary presentation. Extrapulmonary presentation of the disease is often unrecognized because the clinical signs ( eg, peripheral lymph nodes) and more important symptoms ( eg, renal stones or uveitis) do not usually indicate systemic disease. In three different long-term studies of patients with sarcoidosis, 1.5% of the patients presented with uveitis, 11.7% presented with peripheral lymph nodes, and 3.6% presented with renal stones; a total of 16.6% of the patients had extrapulmonary presentation of sarcoidosis. Two of the three studies were retrospective. Many patients may have skin, parotid gland, spleen, liver, central nervous system, bone, or heart presentation; 16.6% is only the tip of an iceberg. A well-conducted, prospective, long-term study could indicate that more than 30% of patients with sarcoidosis have extrapulmonary presentations of the disease. [References: 14]

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Unique Identifier:9764954

Authors: Lynch JP 3rd. Sharma OP. Baughman RP.

Institution: Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor 48109-0360, USA.

Title: Extrapulmonary sarcoidosis. [Review] [261 refs]

Source: Seminars in Respiratory Infections. 13(3):229-54, 1998 Sep.

Abstract: The clinical manifestations of sarcoidosis are extremely heterogeneous and overlap with a wide gamut of infectious and noninfectious granulomatous disorders. Prognosis of sarcoidosis is highly variable. Spontaneous remissions occur in nearly two thirds of patients, but chronic, progressive disease may result in severe sequelae. Fatalities occur in 1% to 4% of patients. Pulmonary manifestations typically dominate, but any organ can be affected. Skin, eye, and peripheral lymph nodes are each involved in 20% to 30% of patients. Clinically significant involvement of spleen, liver, bone, heart, kidney, or central nervous system occurs in 2% to 6% of patients. Asymptomatic involvement of these organs is far more common. We review the salient extrapulmonary features of sarcoidosis, and compare and contrast specific features that may mimic other etiologies. [References: 261]

 Link Directly to Fulltext Article at Science Direct

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Unique Identifier:9723651

Authors: Yazaki Y. Isobe M. Hiramitsu S. Morimoto S. Hiroe M. Omichi C. Nakano T. Saeki M. Izumi T. Sekiguchi M.

Institution: First Department of Internal Medicine, Shinshu University, Matsumoto, Japan.

Title: Comparison of clinical features and prognosis of cardiac sarcoidosis and idiopathic dilated cardiomyopathy.

Source: American Journal of Cardiology. 82(4):537-40, 1998 Aug 15.

Abstract: In the present study, clinical findings of 15 patients with cardiac sarcoidosis presenting as dilated cardiomyopathy were compared with those of 30 consecutive patients with idiopathic dilated cardiomyopathy. The sarcoidosis patients had different clinical features, including female predominance, a high incidence of grave conduction disturbance and abnormal wall thickness, uneven wall motion abnormalities, and perfusion defects preferentially affecting the anteroseptal and apical regions, and poor prognosis compared with those with idiopathic dilated cardiomyopathy.

 Fulltext Available in MDConsult using Journal Search and the search term: 9413660

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Unique Identifier:9413660

Authors: Sharma OP.

Institution: Department of Medicine, University of Southern California, School of Medicine, Los Angeles, USA.

Title: Cardiac and neurologic dysfunction in sarcoidosis. [Review] [108 refs]

Source: Clinics in Chest Medicine. 18(4):813-25, 1997 Dec.

Abstract: Clinically apparent involvement of the heart and nervous system occurs in a relatively small number of patients with sarcoidosis. The diagnosis of myocardial and neurological sarcoidosis is difficult because anatomic presence of granulomas without clinical dysfunction is an important feature of sarcoidosis. The chest radiography is abnormal in 8 of every 10 patients with myocardial or neurosarcoidosis. Serum angiotensin-converting enzyme and gallium uptake studies may provide some indication of the extent and severity of the granulomatous process. Corticosteriods are the mainstay of therapy but chloroquine or hydroxychloriquine, methotrexate, and azathioprine are also effective. Prognosis of myocardial and neurological sarcoidosis is poor. [References: 108]

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Unique Identifier:8950063

Authors: Sekiguchi M. Yazaki Y. Isobe M. Hiroe M.

Institution: 1st Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto City, Japan.

Title: Cardiac sarcoidosis: diagnostic, prognostic, and therapeutic considerations. [Review] [288 refs]

Source: Cardiovascular Drugs & Therapy. 10(5):495-510, 1996 Nov.

Abstract: Cardiac involvement in patients with sarcoidosis is an important consideration for those who are concerned with this strange disease. Sarcoidosis is not an acute malignant disease but may be noticed at the time of sudden, expected death as fatal myocardial sarcoidosis at autopsy. Even with modern advances in our ability to diagnose heart disease, cardiac sarcoidosis is still often overlooked because of its subclinical disease progression. In view of this, an extensive review of previously published literature and of our own case analyses has been carried out because of the authors' long-term experience with performing Konno's endomyocardial biopsy, which was originally developed in 1962 at the author's institution. However, the sensitivity of endomyocardial biopsy in detecting sarcoid granuloma is low (20-30%), and, instead, various kinds of nongranulomatous pathologies are often seen. During the course of our research it was found that there might exist a racial difference in cardiac sarcoidosis. Cardiac death was much more frequent in Japanese patients. The possibility that heart disease in sarcoidosis is caused by cor pulmonale due to advanced pulmonary fibrosis should be reevaluated because only a limited amount of background data is available. The author's review clarified the fact that cardiac sarcoidosis is caused by myocardial or pericardial involvement, resulting in various kinds of bradyarrhythmias or tachyarrhythmias and/or congestive heart failure. Electrocardiographic (ECG) and Holter monitor readings provide a simple and effective method for early detection of this disease. The incidence of ECG abnormalities in a total of 963 sarcoidosis patients was 22.1%, which was more frequent than that of the sex- and age-matched healthy control subjects (17.9%; p < 0.025). Echocardiography and radionuclide studies also provide useful clinical information. Careful follow-up and early corticosteroid administration followed by small maintenance doses may prevent the progression of the disease and improve prognosis. Owing to the progress in antiarrhythmic drugs and pacemaker implantation, the primary cause of death in cardiac sarcoidosis has changed from sudden death (1976 report) to congestive heart failure (1985 report). [References: 288] CAS Registry/EC Number 0 (Adrenal Cortex Hormones).

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Unique Identifier:8893389

Authors: Nelson JE. Kirschner PA. Teirstein AS.

Institution: Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029-6574, USA.

Title: Sarcoidosis presenting as heart disease. [Review] [48 refs]

Source: Sarcoidosis Vasculitis & Diffuse Lung Diseases. 13(2):178-82, 1996 Sep.

Abstract: It is unusual for sarcoidosis to present with isolated cardiac involvement. We report 3 patients in whom conduction disturbances preceded other clinical or radiographic evidence of sarcoidosis, in 2 cases by several years. The literature of sarcoidosis manifesting solely with cardiac disease is reviewed. Our report is intended to emphasize consideration of sarcoidosis in the diagnostic evaluation of cardiac disease without another obvious cause, particularly in young patients with conduction disturbances, arrhythmias, or cardiomyopathy. We suggest diagnostic strategies and a therapeutic approach. [References: 48] CAS Registry/EC Number 0 (Glucocorticoids, Synthetic).