Lupus 101 Rachel Kaiser MD MPH Arthritis & Rheumatism Associates Lupus Foundation Summit Johns Hopkins University September 13, 2014
Jan 05, 2016
Lupus 101Rachel Kaiser MD MPH
Arthritis & Rheumatism AssociatesLupus Foundation Summit
Johns Hopkins UniversitySeptember 13, 2014
Lupus 101What 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-selfImmune system attacks various organs in the
bodyChronicSystemic
Affects many organ systems (e.g. kidney, skin)
Lupus: a Difficult DiagnosisSymptoms
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
FatigueJoint pain/swelling >90%Rashes (worse in the sun) >80%Ulcers in the mouth/noseHair lossChest pain worse w/ breathingKidney, brain involvementRaynaud’s (fingers changing color/numbness
in cold)
RashesMalar, 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 involvementCertain types require different treatmentsA kidney biopsy will help us know what kind
you haveSymptoms
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 SeizuresAcute confusion/psychosisStrokeMovement disorderCognitive dysfunction (brain fog, memory
issues)Myelitis
GastrointestinalHepatitis (liver inflammation)Peritoneal inflammationPancreatitis (often from medications)
SLE subtypesSystemicCutaneous (skin only)Drug-induced
Older blood pressure medications (e.g. hydralazine)
TNF inhibitors Drugs for acne (e.g. minocycline)
Neonatal
Diseases associated with SLEAntiphospholipid antibody syndrome (APS)
Miscarriages, blood clotsSjögren’s Syndrome
Dry eyes, mouthThyroid diseaseFibromyalgia
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 ageDiffers 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 LupusRheumatologists make a diagnosis of lupus
by: Carefully listening to your historyExamining youOrdering/reviewing lab testsExcluding 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 testLupus is characterized by the production of
antibodies against the selfOther causes of a +ANA
Chronic infections Other autoimmune diseases. Up to 20% of healthy young women
Diagnosing LupusIf 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 Lupus4 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 treatmentMake you feel betterPrevent long term complications
Organ damage (e.g. kidneys)MortalityDisability (e.g. job loss, stay in school)
Minimize potential side effects from medications
What causes a flare of lupus?UV light exposure
Wear sunscreen, hatInfection
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, rashesHelps prevent flares Improves survivalMay help prevent blood clotsPrevents nephritis flaresImproves pregnancy outcomes
Treatments – Moderate to Severe
PrednisoneMycophenolate mofetil AzathioprineCyclophosphamide Belimumab
Steroids (e.g. prednisone)Pros: work quickly for acute issues/flares
Cons: side effects if long-term useWeight gainOsteoporosisAvascular necrosis DiabetesCataracts, glaucomaPancreatitisInfectionsPoor wound healingSalt, water retentionPsychiatric symptoms
Routine medical care Monitor for development of new symptoms Health maintenance
CholesterolBlood pressureGynecologic care (e.g. safe contraception)VaccinationsBone health
Screening for side effects of medicationsLab tests
Prevent flares Counseling on wearing sunscreen
Why do I need a primary care doctor?Increased risk of early cardiovascular disease
CholesterolBlood pressure monitoring
VaccinationsCoordination of care between specialists
Obstetrics and Gynecology in lupusBirth control options may be limited
Patients with anti-phosphospholipid antibodies or the syndrome itself may not use estrogen-containing birth control
PAP smears – yearlyIncreased risk of cervical dysplasia
Pregnancy Symptoms need to be quiescent and controlled for a
prolonged period before trying to conceiveToxic medications need to be held if possible or
changed to medications that may be safer in pregnancyPre-conception counseling
Other SpecialistsPulmonology - lungsNephrology - kidneyHematology -bloodDermatology - skinOphthalmology - eyesNeurology – brain/nervesGastroenterology - gut
Prognosis better than everEarlier diagnosisBetter awarenessImproved lab testsBetter treatments that help minimize long-term
steroid exposureMortality:
Early deaths: active SLE +/- infectionsLate deaths: cardiovascular disease
Five year survival increased~40% 1950>90% after 1980
Be your own advocateTempting 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
infectionKnow 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