Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
Jump up ^ Johanneson, Bo; Lima, Guadalupe; von Salomé, Jenny; Alarcón-Segovia, Donato; Alarcón-Riquelme, Marta E.; Collaborative Group on the Genetics of SLE, The BIOMED II Collaboration on the Genetics of SLE and Sjögrens syndrome (2002-11-01). "A major susceptibility locus for systemic lupus erythemathosus maps to chromosome 1q31". American Journal of Human Genetics. 71 (5): 1060–1071. doi:10.1086/344289. ISSN 0002-9297. PMC 385085. PMID 12373647.
Libman-Sacks endocarditis is the most characteristic cardiac manifestation of lupus. It is characterized by clusters of verrucae on the ventricular surface of the mitral valve. These lesions consist of accumulation of immune complexes, platelets, and mononuclear cells. This can lead to heart failure, valvular dysfunction, emboli, and secondary infective endocarditis. Diagnosis is best made via echocardiography, which may reveal the characteristic valvular masses (arrows). IVS = interventricular septum; LA = left atrium; LV = left ventricle.
The global rates of SLE are approximately 20–70 per 100,000 people. In females, the rate is highest between 45 and 64 years of age. The lowest overall rate exists in Iceland and Japan. The highest rates exist in the US and France. However, there is not sufficient evidence to conclude why SLE is less common in some countries compared to others; it could be the environmental variability in these countries. For example, different countries receive different levels of sunlight, and exposure to UV rays affects dermatological symptoms of SLE. Certain studies hypothesize that a genetic connection exists between race and lupus which affects disease prevalence. If this is true, the racial composition of countries affects disease, and will cause the incidence in a country to change as the racial makeup changes. In order to understand if this is true, countries with largely homogenous and racially stable populations should be studied to better understand incidence. Rates of disease in the developing world are unclear.
The discovery of the LE cell led to further research and this resulted in more definitive tests for lupus. Building on the knowledge that those with SLE had auto-antibodies that would attach themselves to the nuclei of normal cells, causing the immune system to send white blood cells to fight off these "invaders", a test was developed to look for the anti-nuclear antibody (ANA) rather than the LE cell specifically. This ANA test was easier to perform and led not only to a definitive diagnosis of lupus but also many other related diseases. This discovery led to the understanding of what are now known as autoimmune diseases.
Common initial and chronic complaints include fever, malaise, joint pains, muscle pains, and fatigue. Because these symptoms are so often seen in association with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms (see below), however, they are considered suggestive.
Most patients with systemic lupus erythematosus (unless they’re otherwise advised by their rheumatologist) should be taking an oral antimalarial drug — medications originally used to prevent a malaria infection, but that have been found to help with lupus symptoms, says Dr. Kramer. The antimalarial hydroxychloroquine helps prevent lupus flares, minimizes joint inflammation, and controls fever, fatigue, pleurisy (inflammation of the sac surrounding the lungs), and pericarditis (inflammation of the lining around the heart). The drug is also “the backbone of therapy” for most skin rashes associated with lupus, says Kramer. Mouth sores may also be alleviated with this drug. Chloroquine and quinacrine are other antimalarials drugs used to treat lupus. (3)
Since SLE patients can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of systemic lupus. To help doctors improve the accuracy of the diagnosis of SLE, 11 criteria were established by the American Rheumatism Association. These 11 criteria are closely related to the symptoms discussed above. Some people suspected of having SLE may never develop enough criteria for a definite diagnosis. Other people accumulate enough criteria only after months or years of observation. When a person has four or more of these criteria, the diagnosis of SLE is strongly suggested. Nevertheless, the diagnosis of SLE may be made in some settings in people with only a few of these classical criteria, and treatment may sometimes be instituted at this stage. Of these people with minimal criteria, some may later develop other criteria, but many never do.
For instance, a dermatologist for cutaneous lupus (skin disease), a cardiologist for heart disease, a nephrologist for kidney disease, a neurologist for brain and nervous system disease, or a gastroenterologist for gastrointestinal tract disease. A woman with lupus who is considering a pregnancy needs an obstetrician who specializes in high-risk pregnancies.
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