Lupus

By Peter Galvin, MD

Systemic lupus erythematosus (SLE or just lupus) is a chronic systemic autoimmune disease characterized by inflammation and immune-related injury to multiple organ systems, including the skin, mucus membranes, musculoskeletal, hematologic (blood), and kidney systems. It affects about 3.4 million people worldwide, and of these individuals, three million are female. It is newly diagnosed in 400,000 people worldwide each year. According to a 2023 review that included 112 studies, Poland, the U.S., Barbados, and China had the highest incidence of lupus. In 2018, the estimated incidence in the U.S. was five to 12 per 100,000 person-years, and the incidence among females vs males was 8.7 vs 1.2 per 100,000 person-years. Black females had an almost three times higher rate as opposed to white females.

Lupus involves inflammation associated with elevated levels of interferon I and autoantibodies to nuclear antigens. Development of clinical disease is thought to require exposure to environmental risk factors such as UV light, cigarette smoking, Epstein-Barr virus, or silica from occupational exposure such as painting, foundry work, or sandblasting. There is also a genetic predisposition to lupus. Lupus affects multiple organs. While there are no formal diagnostic criteria, a positive antinuclear antibody (ANA) titer of at least 1:80 is required for SLE classification. The higher the ANA titer is (for example 1:320), the more likely a lupus diagnosis is. Lupus may present in many different forms, but symptoms typically include fever, alopecia (hair loss), rash (see photos – note the typical “butterfly” facial rash in top photo), oral ulcers, and joint pain and swelling. Lab abnormalities may include the aforementioned elevated ANA, anemia, thrombocytopenia (low platelet count), leukopenia (low white blood cell count), hypocomplementemia (low clotting factors), and serum and urine markers of kidney injury (lupus nephritis) such as proteinuria, hematuria, and elevated serum creatinine.

Lupus may affect multiple organ systems including the skin, joints, blood systems, lungs, kidneys, and may have neuropsychiatric complications which may include headaches, psychosis, mood disorders, cognitive impairment, and seizures. It may also cause gastrointestinal issues such as malabsorption, hepatitis, and pancreatitis. Some patients may develop antiphospholipid antibodies which may cause blood clots, strokes, and pregnancy issues. Treatment of lupus is based on immune system suppression and treatment of complications such as kidney failure, stroke, heart attack, and infections. The mainstay of treatment is hydroxychloroquine. Biologic agents may be needed as well as immunosuppressive drugs for moderate to severe cases. Steroids (such as prednisone or methylprednisolone) may be used but doses are always kept to a minimum dosage because of the often-severe side effects of these drugs.

Multiple studies have all shown that people with SLE have higher rates of disability and death than the general population, often despite treatment. Fortunately, however, developments in anti-lupus drugs, particularly biologic agents that utilize the patient’s own immune system, are showing promise in treating this nasty disease.

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