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Rashes, Joints and ANAs: 2015 Lupus Update Benjamin J Smith, PA-C McIntosh Clinic, P.C. Thomasville, Georgia Kori A. Dewing, DNP, ARNP Virginia Mason Medical Center Seattle, Washington Slide 2 - Presentation was developed in collaboration with The Lupus Initiative http://thelupusinitiative.org/ -No conflicts of interest to disclose Slide 3 3 Member, AAPA Commission on Continuing Professional Development and Education Slide 4 Slide 5 Upon completion of this session, participants should be able to: -Summarize the diagnostic criteria for lupus, including the American College of Rheumatology classification criteria. -Discuss current health disparities that exist for persons with lupus. -Select the proper laboratory tests when evaluating a person with lupus symptoms. -Describe the currently available therapeutic options for the treatment of lupus. Slide 6 1. Which of the following is not considered part of the current criteria for classification of systemic lupus erythematosus: a. discoid rash. b. rosacea. c. serositis. d. oral ulcers. Slide 7 2. Which of the following lupus treatments requires a periodic eye exam? a. prednisone. b. azathioprine. c. hydroxychloroquine. d. belimumab. Slide 8 3. Which of the following ANA results is most likely not to be a false positive result? a. 1:1280, homogenous in a 20 y.o female with joint pain and 2+ proteinuria. b. 1:160, speckled in a 85 y.o male with no other symptoms c. 1:80, speckled in a 32 y.o. female with arthralgia/myalgia, sleep difficulty and fatigue. d. 1:40, homogenous in a 35 y.o female with low back pain. Slide 9 -www.rheumatology.org -www.uptodate.com -The Rheumatology Image Bank (http://images.rheumatology.org/)http://images.rheumatology.org/ -The Lupus Initiative (http://thelupusinitiative.org/)http://thelupusinitiative.org/ -West, Sterling, M.D. Rheumatology Secrets, 2nd Edition. Philadelphia: Hanley & Belfus, Inc.; 2002. - Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus [letter]. Arthritis Rheum 1997;40:1725. -Schur, Peter H. The Rheumatologist. Know Your Labs: Part 1 and 2. February and April 2009. Slide 10 Slide 11 http://bcove.me/5w0pa1xw http://bcove.me/5w0pa1xw (0:00 to 4:09) Slide 12 -An inflammatory, multisystem, autoimmune disease of unknown etiology with protean clinical and laboratory manifestations and a variable course and prognosis -Lupus can be a mild disease, a severe and life-threatening illness, or anything in between Slide 13 Prevalence: 2140/100,000 worldwide but as high as 207/100,000 Incidence: 110/100,000 worldwide Population at highest risk: Women in their reproductive years Female:male ratio is approximately 9:1 postpuberty and premenopausal Variation in race/ethnicity: More common in African American (36x), Hispanic and Native American (23x), and Asian (2x) populations Cost: There are direct costs associated with treatment (eg, $100 billion in healthcare cost associated with autoimmune diseases) and indirect cost related to lost productivity and wages Slide 14 Death rates have decreased by 60% in the United States since the 1970s, especially for infections and renal disease Risks of death increased in females, Blacks, and younger-onset patients Most common causes of death in SLE patients in the United States Heart disease and stroke (1.7 x general population) Hematologic malignancies and lung cancer (2.1 x general population) Infections (5 x general population; also a common cause of hospitalization) Renal disease (7.9 x general population) Bernatsky S, Boivin JF, Joseph L, et al. Arthritis Rheum. 2006;54(8):2550-2557. Slide 15 Upon completion of this session, participants should be able to: -Summarize the diagnostic criteria for lupus, including the American College of Rheumatology classification criteria. -Discuss current health disparities that exist for persons with lupus. -Select the proper laboratory tests when evaluating a person with lupus symptoms. -Describe the currently available therapeutic options for the treatment of lupus. Slide 16 -Systemic Lupus Erythematosus -Cutaneous Lupus Erythematosus -Drug-induced Lupus Erythematosus -Neonatal Lupus Slide 17 Definite Procainamide, hydralazine, minocycline, diltiazem, penicillamine, isoniazid, quinidine, anti-tumor necrosis factor alpha therapy, interferon-alfa, methyldopa, chlorpromazine, and practolol. Probable Anticonvulsants (phenytoin, mephenytoin, trimethadione, ethosuximide, carbamazepine), antithyroid drugs, antimicrobial agents (sulfonamides, rifampin, nitrofurantoin), beta blockers, lithium, paraaminosalicylate, captopril, interferon gamma, hydrochlorothiazide, glyburide, carbamazepine, sulfasalazine, terbinafine, amiodarone, ticlopidine, hydrazine, and docetaxel. Possible Gold salts, penicillin, tetracycline, reserpine, valproate, statins (eg, lovastatin, simvastatin, and atorvastatin) griseofulvin, gemfibrozil, valproate, lamotrigine, ophthalmic timolol, and 5-aminosalicylate. Schur PH, et al. Drug-induced lupus. UpToDate. Accessed 05 May 2014. Slide 18 Malar rashFixed erythema, flat or raised, sparing the nasolabial folds Discoid rashRaised patches, adherent keratotic scaling, follicular plugging; older lesions may cause scarring PhotosensitivitySkin rash from sunlight Oral or nasopharyngeal Usually painless ulcers ArthritisNonerosive, inflammatory in two or more peripheral joints SerositisPleuritis or pericarditis - Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus [letter]. Arthritis Rheum 1997;40:1725. Slide 19 Renal disorderPersistent proteinuria or cellular casts Neurologic disorderSeizures or psychosis HematologicHemolytic anemia, leukopenia ( -Increase in proteinuria is most common Measured by spot protein:creatinine ratio >0.5 or 24-hour collection >500 mg/24 hours The absolute increase in proteinuria that defines a nephritis flare is arbitrary -Microscopic abnormalities on urinalysis White cells or red blood cells >5 cells/hpf in the absence of infection or other causes Cellular casts (white cell or red cell) White cells and red blood cells are seen more frequently than casts Hahn BH, McMahon MA, Wilkinson A, et al. Arthritis Care Res (Hoboken). 2012;64(6):797-808. Slide 28 -19 case definitions of neuropsychiatric manifestations -Most commonly: Cognitive dysfunction Headache Psychiatric disorders (anxiety, psychosis,* depression) Seizures* Stroke (may be associated with antiphospholipid antibodies) Peripheral neuropathies *Part of the classification criteria for SLE. Bertsias GK, Boumpas DT. Nat Rev Rheumatol. 2010;6:358-367. Slide 29 Depression Fatigue Memory thief Lupus Intangibles Slide 30 Signs and Symptoms Symptoms Occurrence (ever) Arthralgias95% Neurologic90% Fever >100 F (38 C)90% Prolonged or extreme fatigue81% Arthritis80% Skin rashes74% Anemia71% Kidney involvement50% Pleurisy and/or pericarditis45% Butterfly-shaped rash across the cheeks and nose42% Sun or light sensitivity (photosensitivity)30% Hair loss27% Abnormal blood clotting problems20% Raynauds phenomenon17% Seizures15% Mouth or nose ulcers12% Slide 31 Upon completion of this session, participants should be able to: -Summarize the diagnostic criteria for lupus, including the American College of Rheumatology classification criteria. -Discuss current health disparities that exist for persons with lupus. -Select the proper laboratory tests when evaluating a person with lupus symptoms. -Describe the currently available therapeutic options for the treatment of lupus. Slide 32 Health disparities are the differences in the incidence, prevalence, mortality, and burden of disease and other adverse health conditions that exist among specific population groups in the United States Healthcare disparities refer to differences in access to or availability of facilities and services National Institutes of Health Slide 33 -Black women are 3 times more likely to develop lupus than White women Affects up to 1 in 250 Black women in the United States -Hispanic, Asian, and Native American populations are also more likely to develop lupus -Women are 9 times more likely to develop lupus than men Helmick CG, Felson DT, Lawrence RC, et al. Arthritis Rheum. 2008;58(1):15-25. Chakravarty EF, Bush TM, Manzi S, Clarke AE, Ward MM. Arthritis Rheum. 2007;56(6):2092-2094. Fessel WJ. Rheum Dis Clin North Am. 1988;14(1):15-23. Slide 34 CDC. MMWR Morb Mortal Wkly Rep. 2002;51:371-374. Slide 35 The reality is that to get to the root cause of disparities, it is not going to be just one factor. For example, poor health literacy perpetuates health disparities, as does a lack of access to care, a lack of access to a regular provider, and a lack of access to a medical home. No single factor can be considered to be the root cause of disparities. Anne Beal, Institute of Medicine Slide 36 Upon completion of this session, participants should be able to: -Summarize the diagnostic criteria for lupus, including the American College of Rheumatology classification criteria. -Discuss current health disparities that exist for persons with lupus. -Select the proper laboratory tests when evaluating a person with lupus symptoms. -Describe the currently available therapeutic options for the treatment of lupus. Slide 37 1948---LE cell test (phagocyte with ingested nucleus) Hargraves MM. Discovery of the LE cell and its morphology. Mayo Clin Proc. 1969;44:579-99. Todays techniques *Immunofluorescent microscopy *Immunodiffusion *Hemagglutination *Complement fixation *Solid-phase immunoassay (ELISA or immunoblotting) *Radioimmunoassays Slide 38 1948---LE cell test (phagocyte with ingested nucleus) Hargraves MM. Discovery of the LE cell and its morphology. Mayo Clin Proc. 1969;44:579-99. Todays techniques *Immunofluorescent microscopy *Immunodiffusion *Hemagglutination *Complement fixation *Solid-phase immunoassay (ELISA or immunoblotting) *Radioimmunoassays Slide 39 ANA Patterns Peripheral or rim Diffuse Speckled Nucleolar Slide 40 ANA Pattern Centromere Slide 41 -95 - 99% sensitivity for SLE, discoid (15%), drug induced (100%) -