Systemic Lupus Erythematosus (Lupus) Presentation - November 15, 2024 - PDF

Systemic Lupus Erythematosus (LUPUS) November 15, 2024 Steve S Lee, DO, FACR Partner Rheumatologist, Kaiser Permanente, Fontana Associate Clinical Professor Western University of Health Sciences California University of Scie...

Systemic Lupus Erythematosus (LUPUS) November 15, 2024 Steve S Lee, DO, FACR Partner Rheumatologist, Kaiser Permanente, Fontana Associate Clinical Professor Western University of Health Sciences California University of Science and Medicine Assistant Clinical Professor Loma Linda University School of Medicine OUTLINE Definition and history Epidemiology Pathophysiology Cases: Update: Classification and diagnosis Clinical Features Update: Treatment “erythema centrifugum” and later “lupus érythèmateux” (1838, 1850): Laurent-Théodore Biett Pierre Louis Cazenave “seborrhoea congestiva” “inflammatio folliculorum”  DEFINITION “Inflammatory heterogeneous autoimmune disorder affecting multiple organ systems characterized by the production of auto-antibodies directed against cell nuclei”  EPIDEMIOLOGY Age, gender, race and genetics Sex, race, and ethnicity Native American 271 Peak incidence 14-45 years African American 211 Female predominance Hispanic 138 severity is = Asian 91 Ethnic predisposition White 64 White Men 4 Genetics: 0 50 100 150 200 250 300 protein tyrosine phosphatase, non-receptor type 22 (PTPN22) Prevalence (per 100,000) HLA DRB1, ITGAM or ITGAX 85% are women Female-to- male ratio: 9 to 1 Mean age at diagnosis: 31 years Hom G, Graham RR, Modrek B, Taylor KE, Ortmann W, Garnier S, Lee AT, Chung SA, Ferreira RC, Pant PK, Ballinger DG. Association of systemic lupus erythematosus with C8orf13–BLK and ITGAM–ITGAX. New England Journal of Medicine. 2008 Feb 28;358(9):900-9. Costs of Lupus Lupus: 2% of all dialysis patients in the US 4000 at any time 1000 new/year US ESRD Registry 1995-2008: 12,533 with lupus nephritis 82% female, 49% Black Statistically significant findings: African-American Medicaid underinsured Mortality rates 50% at 7 years in 1950; now 18% at 15 years Of those aged 15–24y, SLE as cause of death: Ranked 10th (5th among Black and Hispanic females)  Etiology Environmental UV light Viruses Hormones (Estrogen) TOBACCO Question 1 What lab is most specific for lupus? anti-histone CRP thrombocytopenia anti-dsDNA ANA  CLINICAL FEATURES: General Clinical Fatigue Fevers Weight loss Anorexia Alopecia Lymphadenopathy Raynaud’s  Dermatologic domain Malar Rash Fixed erythema; malar eminences Spares the nasolabial folds Discoid Lupus Erythematosus (DLE) Erythematous patches with central clearing keratotic scaling follicular plugging  ORAL ULCERS Oral/nasopharyngeal ulceration Usually painless CLINICAL FEATURES: Musculoskeletal transient, small joints, symmetrical “Jaccoud’s” arthritis Most common presenting feature of SLE (90%)  Case 2 23 Cambodian female with several weeks of worsening white, painful fingers that can turn blue and red. She tried natural options including CBD, plant based diet and mindfulness as well as echinacea for “immune health” She now presents to ED with dyspnea and hemoptysis. She is intubated. CT chest reveals…. Question What is the best initial treatment for this severe presentation of lupus? Stop echinacea Mycophenolate Methylprednisolone Cyclophosphamide Plasmapheresis CLINICAL FEATURES: SEROSITIS Pleural Pulmonary hemorrhage (EMERGENCY) Peritoneal Mesenteric vasculitis Cardiac Pericardial effusion (Steroid and mycophenolate worked for my patient!)  SLE - VASCULOPATHY Dilated nailfold capillary loops Raynaud’s phenomenon Digital ulcers  CLINICAL FEATURES: Cardiac Nonserosal: Cardiac Arrythmias Accelerated Atherosclerosis Immunologic: Libman Sacks endocarditis Valvular heart disease Noninfective thrombotic endocarditis involving mitral valve in SLE extending onto chordae tendineae. CLINICAL FEATURES: HEMATOLOGIC DISORDER A) Hemolytic anemia – “AHA” OR B) Leukopenia - less than 4,000/mm3 OR C) Lymphopenia - less than 1,500/mm3 OR D) Thrombocytopenia - less than 100,000/mm3 PANCYTOPENIA PANCYTOPENIA PANCYTOPENIA CLINICAL FEATURES: Neurologic Psychosis Seizures behavior/personality changes migraines depression cognitive impairment stroke chorea catatonia pseudotumor cerebri longitudinal myelitis – neuromyelitis optica (Devic’s) peripheral neuropathy aseptic meningitis ** steroid psychosis or primary psychiatric disease CLINICAL FEATURES: Renal (Lupus Nephritis) Hallmark: proteinuria (>0.5 gms daily) and casts “Foamy” urine Nephrotic syndrome – Hypoalbuminemia – Hyperlipidemia – Thrombophilia  ISN/RPS Lupus Nephritis Classification System and Prevalence (In Those Biopsied) Class I Class II Class III Class IV Class V Minimal Mesangial Focal Diffuse Membranous LN1,2 mesangial LN1,2 proliferative LN1,2 proliferative LN1,2 proliferative LN1,2 with global or segmental, continuous, granular, ▪Normal glomeruli1 ▪Mesangial ▪6 months High rates of flares: Preeclampsia Fetal Loss Preterm Delivery Low Birth Weight Infant DVT/PE Neonatal lupus and complete heart block SSA  In Summary…. Quintessential autoimmune condition Autoantibodies and risk factors and demographics Clinical domains Skin and MSK Renal, pulmonary, hematologic CBC, urine protein/creatinine, complement and anti-dsDNA Hydroxychloroquine, steroids, immunomodulators, biologics THANK YOU!