Scleroderma Heart Disease

1/12/02 (Oddis)

RE: A 44 year old African American male previously diagnosed with scleroderma, presenting with  SOB, progressive DOE, and orthopnea.

Question: is long-term therapy with either calcium channel blockers or ACE inhibitors indicated for sclerodermal heart disease?

Fulltext Available in MDConsult using Journal Search and the search term: 97082360

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Unique Identifier: 97082360 / PMID: 8923602

Authors: Pope JE. Institution University of Western Ontario, London, Canada.

Title: Treatment of systemic sclerosis. [Review] [95 refs]

Source: Rheumatic Diseases Clinics of North America. 22(4):893-907, 1996 Nov.

Abstract: Guidelines for the conduct of clinical trials in progressive systemic sclerosis have been recommended to determine drug efficacy better. To date, the results of disease-modifying drugs in scleroderma have been disappointing. The treatment of esophagitis has been revolutionized by omeprazole. Raynaud's phenomenon can be treated with calcium channel blockers and iloprost. Scleroderma renal crisis can be treated with aggressive blood pressure control using angiotensin converting enzyme inhibitors. The best treatment for rapidly progressive scleroderma lung is still unknown. Future treatments in scleroderma should be tested with the use of recommended guidelines. [References: 95]

ulltext Available in MDConsult using Journal Search and the search term: 97082357

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Unique Identifier: 97082357 / PMID: 8923599

Authors: Deswal A. Follansbee WP. Institution University of Pittsburgh School of Medicine, Pennsylvania, USA.

Title: Cardiac involvement in scleroderma. [Review] [87 refs]

Source: Rheumatic Diseases Clinics of North America. 22(4):841-60, 1996 Nov.

Abstract: In summary, cardiac involvement in systemic sclerosis can be manifested as myocardial disease, pericardial disease, conduction system disease , or arrhythmias. Clinical cardiac involvement is a poor prognostic factor. Asymptomatic cardiac abnormalities are frequent, and all cardiac abnormalities are seen more often in diffuse scleroderma. Unlike other organs, the role of vascular involvement is unclear. At present, treatment of cardiac scleroderma is essentially symptomatic and empiric. The role of vasodilation and immunosuppression needs further exploration. [References: 87]

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Unique Identifier: 90227836 / PMID: 2183960

Authors: Kahan A. Devaux JY. Amor B. Menkes CJ. Weber S. Venot A. Strauch G. Institution Department of Rheumatology, Rene Descartes University, School of Medicine, Hopital Cochin, Paris, France.

Title: The effect of captopril on thallium 201 myocardial perfusion in systemic sclerosis.

Source: Clinical Pharmacology & Therapeutics. 47(4):483-9, 1990 Apr.

Abstract: In systemic sclerosis, abnormalities of myocard ial perfusion are common and may be caused by a disturbance of the coronary microcirculation. We evaluated the long-term effect of captopril (75 to 150 mg per day) on thallium 201 myocardial perfusion in 12 normotensive patients with systemic sclerosis. Captopril significantly decreased the mean (+/- SD) number of segments with thallium 201 myocard ial perfusion defects (6.5 +/- 1.9 at baseline and 4.4 +/- 2.7 after 1 year of treatment with captopril; p less than 0.02) and increased the mean global thallium score (9.6 +/- 1.7 at baseline and 11.4 +/- 2.1 after captopril; p less than 0.05). In a control group of eight normotensive patients with systemic sclerosis who did not receive captopril, no significant modification in thallium results occurred. Side effects with captopril included hypotension (six patients), taste disturbances (one patient), and skin rash (one patient). These side effects subsided when the dosage was reduced. These findings demonstrate that captopril improves thallium 201 myocardial perfusion in patients with systemic sclerosis and may therefore have a beneficial effect on scleroderma myocardial disease.

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Unique Identifier: 86203495 / PMID: 3486363

Authors: Kahan A. Devaux JY. Amor B. Menkes CJ. Weber S. Nitenberg A. Venot A. Guerin F. Degeorges M. Roucayrol JC.

Title: Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis.

Source: New England Journal of Medicine. 314(22):1397-402, 1986 May 29.

Abstract: Heart disease in patients with progressive systemic sclerosis may be due in part to myocardial ischemia caused by a disturbance of the coronary microcirculation. To determine whether abnormalities of myocardial perfusion in this disorder are potentially reversible, we evaluated the effect of the coronary vasodilator nifedipine on myocard ial perfusion assessed by thallium-201 scanning in 20 patients. Thallium-201 single-photon-emission computerized tomography was performed under control conditions and 90 minutes after 20 mg of oral nifedipine. The mean (+/- SD) number of left ventricular segments with perfusion defects decreased from 5.3 +/- 2.0 to 3.3 +/- 2.2 after nifedipine (P = 0.0003). Perfusion abnormalities were quantified by a perfusion score (0 to 2.0) assigned to each left ventricular segment and by a global perfusion score (0 to 18) for the entire left ventricle. The mean perfusion score in segments with resting defects increased from 0.97 +/- 0.24 to 1.26 +/- 0.44 after nifedipine (P less than 0.00001). The mean global perfusion score increased from 11.2 +/- 1.7 to 12.8 +/- 2.4 after nifedipine (P = 0.003). The global perfusion score increased by at least 2.0 in 10 patients and decreased by at least 2.0 in only 1. These observations reveal short-term improvement in thallium-201 myocardial perfusion with nifedipine in patients with progressive systemic sclerosis. The results are consistent with a potentially reversible abnormality of coronary vasomotion in this disorder, but the long-term therapeutic effects of nifedipine remain to be determined.

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Unique Identifier: 86155818 / PMID: 3953616

Authors: Ellis WW. Baer AN. Robertson RM. Pincus T. Kronenberg MW.

Title: Left ventricular dysfunction induced by cold exposure in patients with systemic sclerosis.

Source: American Journal of Medicine. 80(3):385-92, 1986 Mar.

Abstract: Raynaud's phenomenon and cardiac abnormalities are frequent in patients with systemic sclerosis. Radionuclide ventriculograms were obtained in 16 patients with Raynaud's phenomenon and systemic sclerosis or the related CREST syndrome and in 11 normal volunteers in order to evaluate changes in left ventricular function that might be induced by exposure to cold. Left ventricular regional wall motion abnormalities developed in nine of 16 patients during cooling compared with only one of 11 control subjects, despite a comparable rise in mean arterial pressure (p less than 0.02). The abnormalities occurred in seven of 11 patients with systemic sclerosis, one of four with CREST syndrome, and one with Raynaud's disease . To test the potential protective effect of nifedipine, radionuclide ventriculograms were then obtained during cooling after sublingual nifedipine (20 mg). Only five of 13 patients had wall motion abnormalities, and the severity of the abnormalities was significantly less than during the first cooling period (p = 0.03). Five of eight patients who had cold-induced wall motion abnormalities during the first cooling period had none after nifedipine, whereas two other patients demonstrated small abnormalities only during the second cooling period after treatment with nifedipine. It is concluded that cold induces segmental myocardial dysfunction in patients with systemic sclerosis and that nifedipine may blunt the severity of this abnormal response.