Systemic Lupus Erythematosus Part II Level Basic Version

Systemic Lupus Erythematosus Part II Level: Basic Version No: Version Date: February 2012 Author(s): Eric W. Orlowsky, MD Affiliation(s): Division of Rheumatology, Duke University Medical Center L. I. T. Reviews

Disclaimer/Liability Pending input from NUS legal department. The VAP team will insert this information later. This work is licensed under a Creative Commons Attribution-Non. Commercial-No. Derivs 3. 0 Unported License. To view a copy of this license, visit [http: //creativecommons. org/licenses/by-nc-nd/3. 0/] L. I. T. Reviews

Financial Disclosures (past 3 years) “No Disclosures” L. I. T. Reviews

Learning Objectives • To recognize the various clinical manifestations of SLE • To talk briefly about treatments • To review diagnostic criteria for SLE • To talk about special cases – Lupus in Pregnancy – Drug-Induced Lupus L. I. T. Reviews

Outline (Parts I and II) • I. Epidemiology • II. Pathophysiology • III. Clinical Presentations • IV. Classification Criteria • V. Special Situations L. I. T. Reviews

Classificaiton Criteria for SLE (4 of 11) • Anti-Nuclear Ab (ANA) • Immune Ab – Anti-ds. DNA – Anti-Smith – Antiphosolipids • Hematologic Disorder – Hemolytic Anemia – Leukopenia – Lymphopenia L. I. T. Reviews Reference 3 • • Malar Rash Discoid Rash Photosensitivity Oral/Nasal Ulcers Arthritis Nephritis Serositis Neurologic Disorder

Malar Rash (Canis Lupus= Wolf) • “Butterfly Rash” • Only 30 to 60% • Waxes and wanes L. I. T. Reviews Reference 1, 2, 3 and 5

Discoid Rash • Disfiguring scar • Better prognosis • Only 15 to 30% L. I. T. Reviews References 2, 3 and 5

Mucocutaneous Ulcers • Oral or nasopharyngeal • Painless • Physician observed L. I. T. Reviews References 2 and 3

Arthritis • Nonerosive • Reducible – Increased joint laxity • “Jaccoud’s Arthropathy” L. I. T. Reviews References 2 and 3

Nephritis • Criteria: > 0. 5 mg proteinuria (2+ on dipstick) Cellular casts on UA • RBC, granular casts • Half to 2/3 patients • Renal biopsy guides Rx L. I. T. Reviews References 2, 3 and 5

Serositis • Only 25 to 30% • Lungs – Pleural effusions • Heart – Pericardial effusions L. I. T. Reviews Reference 3 and 5

Neuropsychiatric • Criteria – Seizures – Psychosis • Cognitive Impairment • Demyelinating disorders L. I. T. Reviews Reference 1 and 3

Special Cases: Neonatal Lupus • Mother-Anti-Ro + • Antibody crosses placenta • at risk for congenital heart block L. I. T. Reviews Reference 1, 3, 4 and 5

Special Cases: Drug-Induced Lupus • Rash and arthritis • Medications: – – Hydralazine/Methyldopa Minocycline Procainamide/Quinidine TNF inhibitors • ANA positive – Anti-histone Pattern • Improves with removal of offending agent L. I. T. Reviews Reference 2, 3, and 4

Summary • Diagnosis needs 4 of 11 criteria • Babies of mothers with Anti-Ro antibodies are at risk for neonatal lupus. • Drug-induced Lupus: anti-histone ANA & usually self-limited with stopping medication L. I. T. Reviews

Key References 1) American College of Rheumatology. www. rheumatology. org [website accessed 2/18/12]. 2) Hochberg, MC et al. Rheumatology. 5 th ed. Elsevier: 2011 (Philadelphia). 3) Klippel, JH et al. Primer on the Rheumatic Diseases. 13 th ed. Arthritis Foundation. 4) Le, TH et al. First Aid for the USMLE Step 3. 5) Wikimedia Commons. http: //commons. wikimedia. org/ [website accessed 2/23/12]. L. I. T. Reviews

Acknowledgements • Dr. Lisa Criscione-Schreiber • Faculty & Staff of Duke Rheumatology • All my former patients and teachers L. I. T. Reviews