Scleroderma

Dr. Lawrence Phillips is the Assistant Professor of Medicine, Division of Cardiology and Director of Nuclear Cardiology at the NYU Langone Medical Center. At the recently concluded Caribbean Autoimmune Diseases Summit, Dr. Phillips presented on the clinical manifestations of heart diseases in autoimmune disorders.

He started off with some interesting statistics about diseases of the heart in Trinidad & Tobago, citing that it was the leading cause of death accounting for almost 25% of deaths in 2005, virtually unchanged from the previous two years. This was followed by an overview of the role of the heart and essentially its context within the larger cardiovascular system and the role played within autoimmune diseases.

This was followed by an overview of Atherosclerosis, an inflammatory process which starts with abnormal fatty deposits in the inner layers of large or medium-sized arteries, which can lead to hardening and narrowing of the arteries and blockages of the blood supply especially to the heart. Some of the associated symptoms include chest pain, shortness of breath, and the sensation of an irregular heart beat. Abdominal discomfort and nausea may also occur, though less common. There was also a noticeable gender affect as women often present atypically.

Dr. Phillips indicated that chronic inflammation is going to increase the development and instability of Coronary Artery Disease (CAD) and any autoimmune disease associated with chronic inflammation was more likely to put patients at a higher risk of heart attacks. For instance, he showed that the presence of chronic inflammation in Rheumatoid Arthritis (RA) may enhance the development of Atherosclerosis. Data collected through his research showed that there was a heart related mortality increase of 50% in RA patients, compared with that of the general population.

In those with Systemic Lupus Erythematosus (SLE), there was a tendency toward accelerated Atherosclerosis, along with a gender distribution towards women and relation to ischemic heart disease (IHD) or myocardial ischaemia - a disease characterized by reduced blood supply of the heart muscle, usually due to coronary artery disease or atherosclerosis of the coronary arteries.

Undoubtedly of great interest to attendees at Dr. Phillips’ presentation was autoimmune disease treatments. He indicated that there was an increased risk of IHD with;

• Glucocorticoid exposure: where supraphysiologic doses of steroids can increase rates of myocardial infarction and has been associated with worsening of the traditional risk factors for coronary heart disease (CHD)
• COX-2 Inhibitors: where the use of rofecoxib has been associated with increased cardiovascular risk
• Nonsteroidal Antiinflammatory Drugs (NSAIDs): which has been shown to have adverse effects on hypertension.
  

It wasn’t all bad news though, as the following treatments presented with potential decreased risks;

• Methotrexate/TNF Inhibitors: which have been shown to control inflammation, and decrease activity of the disease state
• Hydroxychloroquine: which may benefit lipid profiles, decrease the risk of developing diabetes, and provide an alternate therapy to glucocorticoid therapy.
  

Dr. Phillips was quite optimistic about the eventual successes of ongoing and active areas of research into heart heart disease, which shall yield increased understanding of heart disease and new and improved treatments and therapies.

Dr. Phillips went on further to discuss the importance of traditional risk factor modification. A study by the Cleveland Clinic though inclusive, has demonstrated that aggressive risk factor modification, may do more than just slow atherosclerosis - it may actually cause the disease to improve. Furthermore, evidence has shown that achieving very low, Low-density lipoprotein (LDL) cholesterol levels should be a goal for those with CAD, and finally, that in patients with proven CAD, the target systolic blood pressure ought to be 120 mm Hg or lower. See the ATP III Guidelines for further reference information.

Some of the traditional risk factors for CAD highlighted by Dr. Phillips included;

• Hypertension
• Tobacco abuse
• Diabetes
• Age
• High cholesterol levels
• Obesity 
• Lack of physical activity
  

It was subsequently noted that physical exercise and activity was of critical importance, particularly as it has been shown to reduce the risk of CHD.

In rounding out his presentation, Dr. Phillips dove into the some of complications which may present in those with an autoimmune disease - the first of which was Valvular Heart Disease (VHD), which is characterized by damage to or a defect in one of the four heart valves: the mitral, aortic, tricuspid or pulmonary. Valvular heart disease resulting from rheumatic fever is referred to as "rheumatic heart disease".

Rheumatic fever is an autoimmune inflammatory disease that occurs following a Streptococcus pyogenes infection, such as strep throat or scarlet fever and typically occurs in adolescent children, with only 20% of first-time attacks occurring in adults. Some of its major manifestations include migratory arthritis, carditis and valvulitis, and central nervous system involvement. Valve (usually mitral) dysfunction occurs over the course of many years, due to scarring, calcification, and fibrosis or scarring.

Rheumatic Mitral Stenosis is another complication, which is characterized by the mitral valve not opening fully, restricting blood flow. Some of symptoms, Dr. Phillips noted, include the shortness of breath, fatigue, orthopnea, hemoptysis, and palpitations. Treatments can include diuretics, beta blockers, atrial fibrillation management, and anticoagulation.

The last complication offered was Libman-Sacks Endocarditis, the most characteristic cardiac manifestation of Lupus. It has been described in medical literature as mulberrylike clusters of verrucae on the ventricular surface of the posterior mitral leaflet - lesions, of sorts, attributed to thickening due accumulation of “autoimmune response”

Dr. Phillips closed off his presentation by indicating that those most at risk for heart blockage or failure had Rheumatoid Arthritis, Chagas Disease, Lyme Disease, and Neonatal SLE. The major take away point, was that risk factor modification was important for ALL people, and not just those with autoimmune diseases. Finally, that many autoimmune diseases can have direct affects on the heart (such as structural complications), but they can indirectly increase the risk for ischemic heart disease.