1. Lupus 101 Rachel Kaiser MD MPH Arthritis & Rheumatism Associates
Lupus Foundation Summit
Johns Hopkins University
September 13, 2014

2. 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?

3. What is lupus? Autoimmune disease Chronic Systemic Self vs. non-self
Immune system attacks various organs in the body
Chronic
Systemic
Affects many organ systems (e.g. kidney, skin)

4. 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

5. 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)

6. Rashes
Malar, photosensitivity

7. Rashes - discoid

8. Rashes – Subacute Cutaneous Lupus (SCLE)

9. Hair loss, mouth ulcers

10. Arthritis

11. Raynaud’s

12. Inflammation around heart, lungs

13. 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

14. Neuropsychiatric Seizures Acute confusion/psychosis Stroke
Movement disorder
Cognitive dysfunction (brain fog, memory issues)
Myelitis

15. Gastrointestinal Hepatitis (liver inflammation)
Peritoneal inflammation
Pancreatitis (often from medications)

16. SLE subtypes Systemic Cutaneous (skin only) Drug-induced Neonatal
Older blood pressure medications (e.g. hydralazine)
TNF inhibitors
Drugs for acne (e.g. minocycline)
Neonatal

17. 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

18. What causes it? Immune system Genetics: >50 genes identified +
Environment (e.g. viruses, drug-induced)

19. Who gets lupus? Women of childbearing age Differs by ethnicity
Ages (mean 31)
More women than men: :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%

20. 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

21. 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

22. 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).

23. Classification criteria for Lupus 4 or more (at least one clinical and one immunologic)
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

24. 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

25. 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

26. 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

27. Treatments – Moderate to Severe
Prednisone
Mycophenolate mofetil
Azathioprine
Cyclophosphamide
Belimumab

28. 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

29. 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

30. Why do I need a primary care doctor?
Increased risk of early cardiovascular disease
Cholesterol
Blood pressure monitoring
Vaccinations
Coordination of care between specialists

31. 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

32. Other Specialists Pulmonology - lungs Nephrology - kidney
Hematology -blood
Dermatology - skin
Ophthalmology - eyes
Neurology – brain/nerves
Gastroenterology - gut

33. 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

34. 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)

35. Thank you!
Arthritis and Rheumatism Associates