Published on February 16, 2014
Donald Thomas, MD, FACP, FACR Arthritis and Pain Associates of PG County Assistant Professor of Medicine Uniformed Services University of the Health Sciences, Bethesda
photo credit: africanleadershipacademy.org
- LUPUS Women of childbearing age More severe dz in younger patients 1/200 African American women of child bearing age
- - LUPUS Women of childbearing age More severe dz in younger patients 1/200 African American women of child bearing age “Invisible disease” Average of 4-6 years before diagnosis
- - LUPUS Women of childbearing age More severe dz in younger patients 1/200 African American women of child bearing age “Invisible disease” Average of 4-6 years before diagnosis - 5-10% die within 10 years of dx
Whitney - 24 yo
- Whitney Born 12/14/88 Died 2/20/13 from SLE photo credit: facebook.com/Lupus –Wall- Remembering- those- who- have- lost- their- Battle
- 95% of patients live 10 years or longer - Most patients live a long normal life with proper treatment - Best prognosis: - Early diagnosis Proper medical care (doctors, medications, tests, educated) photo credit: sometimesitslupus.com
- New “classification criteria” for systemic lupus - What labs to order for lupus workup - Correction of lupus triggers - Low vit D, UV light, smoking, sulfa antibiotics - Ensure vaccines are obtained - Resources to recommend to college students with lupus
- 4 out of 14 criteria = SLE - Classification criteria = for research purposes only - Not recommended for diagnostic purposes - 2004: embarked upon revision
- Missing in 1982 criteria Low complements Antiphospholipid antibodies - - 1982 weighted towards cutaneous dz (4 of 14 criteria) Excluded biopsy proven lupus nephritis as sole manifestation Neuro lupus only included psychosis and seizures - - - ACR lists 18 potential neurologic disorders in neuropsychiatric lupus Could only use one type of low blood count - LE cell prep no longer used
- Diagnosed SLE patients vs those meeting classification Many patients with early SLE didn’t meet criteria By the time they do they are: - Older Had established disease longer More end-organ damage
- SLE occurs if - Biopsy proven lupus nephritis + ANA or dsDNA - OR - - 4 out of 17 criteria At least 1 from “Clinical Criteria” and from “Immunologic Criteria”
- Renal Alopecia, nonscarring Serositis Hemolytic anemia - Oral and nasal ulcers Neurologic - Synovitis Chronic cutaneous lupus (discoid) Acute and subacute cutaneous lupus Leucopenia/lymphopenia Platelets, low
- Random urine protein/creatinine ratio ≥ 0.500 - 25 hour urine protein ≥500 mg protein/24 hours - Red blood cell casts on urine microscopy photo credit: studyblue.com
- Diffuse thinning - Hair fragility, broken hair - “Lupus hair” - Rule out alopecia areata, drugs, iron deficiency, androgenic alopecia - Grows back CellCept
- Pleuritis - “Typical pleurisy” > 1 day Pleural effusions Pleural rub - Pericarditis - “Typical pericardial pain” > 1 day (worse with lying, better sitting forward) Pericardial effusion Pericardial rub + ECG Photo credit: clinicalcases.org
- Direct Coombs antibody positive - High reticulocyte count - Low haptoglobin - Increased indirect bilirubin Photo credit: commons.wikipedia.org
- Oral ulcers - Palate, buccal, tongue Often painless - Nasal ulcers - Rule out: - - Vasculitis Behçet’s disease Infections (HSV) Inflammatory bowel disease Reactive arthritis Photo credit: de.wikipedia.org
- Seizures - Psychosis - Mononeuritis multiplex - in absence of a 1° vasculitis - Myelitis - Peripheral or Cranial neuropathy - R/o diabetes, infection (Lyme), 1° vasculitis - Acute confusional state - R/o toxic, metabolic, uremia, infection, drugs Photo credit: en.wikipedia.org
- ≥ 2 joints - Swelling or effusion OR Tender joints + AM stiffness ≥ 30 minutes Photo credit: cdaarthritis.com
- Discoid lupus - Hypertrophic (verrucous) lupus - Lupus panniculitis (profundus) - Discoid lupus/lichen planus overlap - Lupus erythematosus tumidus - Chilblains lupus - Mucosal lupus Photo credit: entindia.info
- Malar rash (don’t count discoid) - Toxic necrolysis variant of SLE - Maculopapular lupus rash - Photosensitive lupus rash - Bullous lupus - SCLE: - Non-indurated psoriasiform Annular polycyclic Photo credit: globalskinatlas.com
- WBC < 4000/mm3 (once) - R/o Felty’s syndrome, drugs, portal hypertension - Lymphs < 1000/mm3 (once) - R/o steroids, drugs, infections (virus)
- Platelets< 100,000 (once) - R/o TTP, drugs, portal hypertension
- ANA - Anti-ds DNA - Antiphospholipid antibodies - - Lupus anticoagulant False positive RPR Anticardiolipin antibody Beta-2 glycoprotein antibody - Low complements (C3, C4, CH50) - Direct Coombs’ test (in absence of hemolytic anemia)
- Out of 702 patient scenarios………. - Misclassified patients: 7% vs 10% - Sensitivity: 94% vs 86% - Specificity: 92% vs 93% (not statistically different)
“… if you use the classification criteria to diagnose SLE... I promise not to tell anyone.” Michelle Petri, MD: Medical Director Lupus Clinic Johns Hopkins
- Renal (proteinuria) Alopecia Serositis (pleuritic chest pain) Hemolytic anemia (all low blood counts) - Oral and nasal ulcers Neurologic problems - Synovitis (joint pains) Chronic cutaneous lupus (discoid) Acute cutaneous lupus (malar rash, rash with sun exposure) Leukopenia/lymphopenia and Platelets, low - Blood clots Raynaud’s phenomenon
- Basic/Initial - ANA by IFA (indirect fluorescence assay) - CBC Urinalysis with reflex microscopy Random urine protein/creatine ratio ESR, CRP, SPEP 25-OH vitamin D - - - If pleuritic chest pain - CXR ECG Echocardiogram
- If positive ANA by IFA ds-DNA ENA (Smith, RNP) Sjögren's panel (SSA/SSB) Ribosomal-P antibody C3, C4, CH50 complements Direct Coombs’ test Antiphospholipid antibodies - - RPR with reflex FTA Anticardiolipin antibodies (IgM, IgG, IgA) Lupus anticoagulant Beta-2 glycoprotein I antibodies (IgM, IgG, IgA) - Inflammatory arthritis: - CPK, RF, CCP, Lyme, HLA-B27, ASO, IgM Parvovirus
- Low vitamin D levels - UV light - Smoking - Sulfa antibiotics
- White blood cell membranes have Vit D receptors - Higher prevalence of low Vit D in SLE patients - More severe SLE at presentation associated with lower Vit D - Lower Vit D levels occur during SLE flares - Low vitamin D correlated with flares
- Petri M et al, Vitamin D and SLE, Arthr & Rheum;65(7):1865-71 1006 patients, 128 weeks 25[OH]D < 40 ng/mL TX = 50,000 IU ergocalciferol (vit D2) + daily calcium with 200 IU vit D3 - - Results: - ≥ 20 ng/mL increase 25[OH]D associated with: - .22 decrease in SELENA/SLEDAI (P = .032) 21% decrease in having a SELENA/SLEDAI ≥ 5 Random urine/protein decreased by 2% (P = .0001) 15% decrease in odds of having urine/prot > .5
- Treat patients with 25[OH]D < 40 ng/mL - Aim for a level of around 40 ng/mL or higher
Ultraviolet light Skin NUCLEUS cell
Ultraviolet light Skin NUCLEUS cell damage cell NUCLEUS
Ultraviolet light Skin NUCLEUS cell Antinuclear antibodies Cause increased lupus activity
X 15 minutes Dose of UV light = Strength X Time
X 15 minutes Dose of UV light = Strength X Time X all day long
- Wear sunscreen daily even if don’t go outside - Reapply if go outside - Use sunscreen vs UVA and UVB + waterproof + high SPF - Wide brimmed hat - UV protectant clothes - Add Rit Sunguard to wash - Avoid outside 10 AM – 3 PM
- Tobacco contains hydrazine - Hydrazine known to increase lupus activity - Smoking decreases effectiveness of Plaquenil - Smoking is associated with increased lupus prevalence - Smoking associated with more severe lupus
- Increased risk for lupus flares - Ask patients to include Bactrim and Septra in allergies
- Make sure all patients get yearly flu shot
- Dreyer L et al, High Incidence of Potentially Virus-Induced Malignancies in SLE, Arth & Rheum, 2011;63(10):3032-37 Increased HPV-associated cancers - Anal cancer Vulvovaginal Cervical Non-melanoma skin cancer - Nath R et al, High risk of Human Papillomavirus Type-16 infections and of development of squamous intraepithelial lesions in systemic lupus erythematosus patients, A&R, 2007;57(4):619-25 - High levels of HPV-16 infection and abnormal colposcopy in newly diagnosed SLE women
photo credit: beasleyallen.com
- Lupus Foundation of America DC/MD/VA chapter Patient Navigator service www.lupus.org/dmv 888-787-5380 - - “Lupus Secrets” handout (last page) - Social Media: Facebook: Lupus Encyclopedia - - - www.facebook.com/LupusEncyclopedia Daily tips and facts about lupus I answer questions posted by patients Numerous Facebook patient support groups
- SLICC new SLE classification criteria - - 4 out of 17 at least 1 from “clinical” and 1 from “immunologic” - Basic initial workup: ANA, CBC, UA - Do additional labs if ANA+ Refer to rheumatologist ASAP - Begin tx: Vitamin D, Sunscreen, no cigarettes - Vaccines: - Annual flu shot Gardasil series - Resources are available
photo credit: customink.com
References 1: Agmon-Levin N et al. International recommendations for the assessment of autoantibodies to cellular antigens referred to as anti-nuclear antibodies. Ann Rheum Dis. 2014;73:17-23 Amital H et al. Serum concentration of 25-OH vitamin D in patients with SLE are inversely related to disease activity. Ann Rheum Dis.2010,69:1155-57. Birmingham DJ et al. Evidence that abnormally large seasonal declines in vitamin D status may trigger SLE flare in non-African Americans. Lupus. 2012;21(8):855-64 Bonakdar ZS et al. Vitamin D deficiency and its association with disease activity in new cases of systemic lupus erythematosus. Lupus.2011;20:1155-60
References 2: Boeckler P et al. Association of cigarette smoking but not alcohol consumption with cutaneous lupus erythematosus. Arch of Derm. 2009;145(9):1012-16 Cooper G et al. Occupational and environmental exposures and risk of systemic lupus erythematosus: silica, sunlight, solvents. Rheum (Oxford). 2010;49(11):2172-80 Dreyer L et al. High incidence of potentially virusinduced malignancies in systemic lupus erythematosus. Arth & Rheum. 2011;63(10):3032-37
References 3: Ghaussy NO et al. Cigarette smoking and disease activity in systemic lupus erythematosus. J of Rheum. 2003;30:1215-21 Isenberg DA et al. The Systemic Lupus International Collaborating Clinics (SLICC) group – It was 20 years ago today. Lupus. 2011;20:1426-32 Mok CC et al. Vitamin D deficiency as marker for disease activity and damage in systemic lupus erythematosus. Lupus. 2012;21:36-42
References 4: Nath Ret al. High risk of human papilloma virus type 16 infections and of development of cervical squamous intraepithelial lesions in systemic lupus erythematosus patients. Arth & Rheum. 2007;57(4):619-25 Petri M et al. Vitamin D in SLE. Arth & Rheum. 2013;65(7):1865-71 Petri M et al. Derivation and validation of the systemic Lupus International Collaborating Clinics classification criteria for SLE. Arthr & Rheum. 2012:2677-86
References 5: Petri M & Magder L. Classification criteria for SLE. Lupus. 2004;13:829-37 Pons-Estel GJ et al. The ACR and the SLICC criteria for SLE in two multiethnic cohorts. Lupus. 2014;23:3-9 Rahman P et al. Smoking interferes with efficacy of antimalarial therapy in cutaneous lupus. J of Rheum. 1998;25:1716-19 Ruiz-Irastorza G et al. Changes in vitamin D levels in patients with SLE. Arthr Care & Research. 2010;62(8):1160-65
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