Download 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
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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
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
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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
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
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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 flarerule 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