Transcript Lupus 101
Rachel Kaiser MD MPH Arthritis & Rheumatism Associates Lupus Foundation Summit Johns Hopkins University September 13, 2014 Lupus 101 What is lupus? Why is lupus hard to diagnose? What causes lupus? Who gets lupus? Is there a test for lupus? How is lupus treated? What causes flares? What is lupus? Autoimmune disease Self vs. non-self Immune system attacks various organs in the body Chronic Systemic Affects many organ systems (e.g. kidney, skin) Lupus: a Difficult Diagnosis Symptoms Develop slowly Vague (e.g. fatigue, rash) Other potential causes (e.g. thyroid disease, infection) Two lupus patients can have very different symptoms Determining which symptoms and lab tests add up to a diagnosis of lupus can be difficult Lupus symptoms Fatigue Joint pain/swelling >90% Rashes (worse in the sun) >80% Ulcers in the mouth/nose Hair loss Chest pain worse w/ breathing Kidney, brain involvement Raynaud’s (fingers changing color/numbness in cold) Rashes Malar, photosensitivity Rashes - discoid Rashes – Subacute Cutaneous Lupus (SCLE) Hair loss, mouth ulcers Arthritis Raynaud’s Inflammation around heart, lungs Kidney – “nephritis” Several different types of kidney involvement Certain types require different treatments A kidney biopsy will help us know what kind you have Symptoms Sudden onset of swollen legs, sometimes in the setting of flaring lupus (e.g. rash, increased fatigue) Protein/blood can be detected in a routine urine sample Neuropsychiatric Seizures Acute confusion/psychosis Stroke Movement disorder Cognitive dysfunction (brain fog, memory issues) Myelitis Gastrointestinal Hepatitis (liver inflammation) Peritoneal inflammation Pancreatitis (often from medications) SLE subtypes Systemic Cutaneous (skin only) Drug-induced Older blood pressure medications (e.g. hydralazine) TNF inhibitors Drugs for acne (e.g. minocycline) Neonatal Diseases associated with SLE Antiphospholipid antibody syndrome (APS) Miscarriages, blood clots Sjögren’s Syndrome Dry eyes, mouth Thyroid disease Fibromyalgia Diffuse pain, sleep disorder, exhaustion What causes it? Immune system Genetics: >50 genes identified + Environment (e.g. viruses, drug-induced) Who gets lupus? Women of childbearing age Ages 15-45 (mean 31) More women than men: 10-15:1 Can affect both men and women of any age Differs by ethnicity More common and severe among Asians, African Americans and Hispanics than Caucasians Family members of lupus patients Siblings 2-5% Monozygotic (identical) twins 24% Diagnosing Lupus Rheumatologists make a diagnosis of lupus by: Carefully listening to your history Examining you Ordering/reviewing lab tests Excluding other causes of your symptoms and lab tests Is there a test for lupus? No one lab test to diagnose lupus +ANA (anti-nuclear antibody) – blood test Lupus is characterized by the production of antibodies against the self Other causes of a +ANA Chronic infections Other autoimmune diseases. Up to 20% of healthy young women Diagnosing Lupus If your history and exam suggest lupus, your rheumatologist will order further, more specific tests in addition to the ANA that can help make the diagnosis. E.g. dsDNA, anti-smith In and of themselves, these tests are still insufficient to establish a definitive diagnosis (because they are neither perfectly specific for lupus nor do they identify every lupus case). Classification criteria for Lupus 4 or more (at least one clinical and one immunologic) Clinical Rash Mouth/nose ulcers Nonscarring hair loss Swollen joints Inflammation around heart or lungs Kidney involvement Neurologic involvement Hemolytic anemia (low blood count) Leukopenia (low white count, these cells respond to infections) Thrombocytopenia (low platelets – these cells clot the blood) Immunologic ANA Low complements (C3,C4) dsDNA, anti-smith, anti-phospholipid antibodies, direct Coombs Goals of lupus treatment Make you feel better Prevent long term complications Organ damage (e.g. kidneys) Mortality Disability (e.g. job loss, stay in school) Minimize potential side effects from medications What causes a flare of lupus? UV light exposure Wear sunscreen, hat Infection Obtain appropriate vaccinations (e.g. yearly flu shot) Surgeries Plan medications appropriately around elective procedures Stress Adequate rest, stress reduction Smoking Need to quit Treatments – All Lupus Daily oral medicine called hydroxychloroquine Helps hand pain, rashes Helps prevent flares Improves survival May help prevent blood clots Prevents nephritis flares Improves pregnancy outcomes Treatments – Moderate to Severe Prednisone Mycophenolate mofetil Azathioprine Cyclophosphamide Belimumab Steroids (e.g. prednisone) Pros: work quickly for acute issues/flares Cons: side effects if long-term use Weight gain Osteoporosis Avascular necrosis Diabetes Cataracts, glaucoma Pancreatitis Infections Poor wound healing Salt, water retention Psychiatric symptoms Routine medical care Monitor for development of new symptoms Health maintenance Cholesterol Blood pressure Gynecologic care (e.g. safe contraception) Vaccinations Bone health Screening for side effects of medications Lab tests Prevent flares Counseling on wearing sunscreen Why do I need a primary care doctor? Increased risk of early cardiovascular disease Cholesterol Blood pressure monitoring Vaccinations Coordination of care between specialists Obstetrics and Gynecology in lupus Birth control options may be limited Patients with anti-phosphospholipid antibodies or the syndrome itself may not use estrogen-containing birth control PAP smears – yearly Increased risk of cervical dysplasia Pregnancy Symptoms need to be quiescent and controlled for a prolonged period before trying to conceive Toxic medications need to be held if possible or changed to medications that may be safer in pregnancy Pre-conception counseling Other Specialists Pulmonology - lungs Nephrology - kidney Hematology -blood Dermatology - skin Ophthalmology - eyes Neurology – brain/nerves Gastroenterology - gut Prognosis better than ever Earlier diagnosis Better awareness Improved lab tests Better treatments that help minimize long-term steroid exposure Mortality: Early deaths: active SLE +/- infections Late deaths: cardiovascular disease Five year survival increased ~40% 1950 >90% after 1980 Be your own advocate Tempting for patients (and their doctors) to attribute all new symptoms to lupus Take new symptoms seriously Fever is rarely a symptom of lupus flarerule out infection Know your medications Current Past Side effects/allergies/intolerances Keep copies of your own records (lab tests, xrays, echocardiograms/heart tests, skin biopsies, kidney biopsies, notes from prior rheumatologists) Thank you! Arthritis and Rheumatism Associates