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Lupus Systemic Lupus Erythematosus Sarah Mouser SLE A chronic multisystem inflammatory disorder that can affect various parts of the body esp the joints, kidney, skin, and blood. It‟s a disease of flares and remissions Autoimmune disease-attacks itself Etiology 70%-90% occur in women, but can occur in men. More common in African Americans & Asians More than 16,000 new cases a year 5 million people worldwide 1.5 million in America CAUSES No gene or gene group has been proven to cause Lupus Appears in certain families Unknown cause Some environmental factors such as: UV rays, sulfa rays, some sun-sensitizing tetracyclinesBactrim, penicillin-Amoxicillin Can be exaccerbated by emotional stress, cold, virus, exhaustion, Sex hormone Estrogen plays a part in it. Forms of Lupus SLE (Systemic Lupus Erythematosus) CLE (Cutaneous Lupus Erythematosus) Drug-Induced Lupus Erythematosus SLE Most common Inflammation of the kidneys Increased bp in the lungs Inflammation of blood vessels in brain Hardening of arteries CLE Limited to the skin Raised, scaly and red, but not itchy, known as discoid rash bec of disk shaped lesions Butterfly rash Drug-Induced Lupus Most often caused by hydralazine (used to treat HTN), procainamide (irregular heart rhythm), isoniazide (used to treat tuberculosis) More common in men SLE -alterations in the immune system -Antinuclear antibodies, antibodies to DNA, and antihistones are all strongly associated with SLE. -abnormalities in both B cells and T cells -appearance of B cells thought to cause increase in production of antibodies to self -visceral lesions mediated by type III hypersensitivity General Symptoms -extreme fatigue -malaise -fever -painful or swollen joints -known as the „great imitator‟ because it has symptoms similar to RA, blood disorders, fibromyalgia, diabetes, thyroid problems, Lyme disease, and many heart, lung, and muscle and bone disorders. Prognosis -no cure for disease -80-90% can expect to live a normal life span -many people die from overwhelming infection and kidney failure Overall 5 yr survival rate with extended therapeutic options are 95% Diagnosis -difficult to diagnosis because it mimics -evolves over time -have abnormal lab tests -not one specific test 4 out of 11 of the criteria Criteria -face rash -scaly, discoid rash -sun-related rash -mouth sores -joint pain in more than 2 joints -swelling around heart or lungs -kidney disease -seizures -low blood count -positive anti-nuclear antibody test -other positive blood tests Overlapping Syndromes -5%-30% have overlapping symptoms -likelihood of an overlap disease-15% -Overlap Disease Approximate Rate of Occurrence Rheumatoid Arthritis 1% Polymyositis-Dermatomyositis 2% Mixed Connective Tissue Disease 3% Scleroderma 4% Sjogren's syndrome 5% Myositis-muscle pain due to fatigue and high levels of cortisone Polymyositis-Dermatomyositis-muscle weakness due to muscle inflammation Scleradoma-thickened skin Mixed connective tissue disorder-lupus, polymyositis-dermatomyositis, and scleroderma. Sjogren‟s syndrome-dry eyes and mouth-caused by an accumulation of lymphocytes Cardiovascular -very hard to know -pericardial effusion -myocarditis-inflammation-at risk due to immunosuppressives -increased risk for CAD, HTN from kidney disease or corticosteroid use, elevated cholesterol levels from corticosteroid use, type 2 diabetes from corticosteroid use -lupus-related anemia b/c body is not producing enough red blood cells, antibodies destroy the healthy blood cells-hemolytic anemia cont -valvular disease -thrombophlebitis -serositis-inflammation of sacs around organs -vasculitis -Raynaud‟s phenomenon- fingers turn white and/or blue when cold GI system - dysphagia -nausea -vomiting -pancreatitis -elevated liver function tests -sometimes due to treatments such as NSAIDS, other times due to muscles not moving wastes through body Pulmonary System -cough -dyspnea -pleurisy -pneumonitis MS system -joint deformities -arthralgia-joint pain -morning stiffness -lupus arthritis, which can cause muscle atrophy Skin -discoid lesions on sun-exposed skin -photosensitivity -butterfly rash -build-up of calcium deposits under the skinCalcinosis -inflammation damages blood vessels in the skin-Cutaneous vasculitis lesions -alopecia-hair loss -ulcers in mouth-mucosal ulcerations -petechia Calcinosis Hematologic System -lupus-related anemia b/c body is not producing enough red blood cells, antibodies destroy the healthy blood cells-hemolytic anemia -thrombosis -thrombocytopenia -leukopenia -neutropenia Splenomegaly Nervous System -neuropathy -stroke -seizures -cognitive function described as “lupus fog” due to feelings of confusion, fatigue, memory loss, & difficulty expressing thoughts -lupus headache-twice as many headaches as general population Renal System -lupus nephritis-inflammation of nephrons which can lead to ESRD -first sign edema of feet and hands, urine frothy -urinary tract infections -lupus cystitis-inflammation of lining of the bladder LIVING WITH LUPUS -exercise is encouraged to strengthen bones and tone muscles -especially walking, swimming, bicycling, -limit if experiencing bone and/or joint pain -extreme fatigue (unknown why) -poor coping strategies, feelings of helplessness, lack of exercise -proper amount of rest CONT -increased sensitivity to light -infections can occur more often due to immunosuppressive -most common infections are involved with the respiratory (lungs and heart) system, skin, and urinary tract also Candia (yeast) infections, and shingles -30% of people get fibromyalgia -depression Treatments Corticosteroids, Antimalarial-Plaquenil, Aspirin, Immunosuppressive, Anticoagulants Treated by rheumatologist Treatment based on age, symptoms, health, and lifestyle. INVESTIGATIONS -Monoclonal antibodies-interfere with action of cells involved with immune response -hormones -stem-cell transplant Famous People with Lupus Seal-singer had non-SLE as a child Flannery O‟Connor-author October is official Lupus Awareness Month
