LUPUS CAN DO EVERYTHING, BUT NOT EVERYTHING IS LUPUS LUPUS 101 SLE SUBSETS AUTOIMMUNE DISEASE 11/4/2013 HOWARD HAUPTMAN, MD IDIOPATHIC DISCOID LUPUS

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1 LUPUS 101 LUPUS CAN DO EVERYTHING, BUT NOT EVERYTHING IS LUPUS HOWARD HAUPTMAN, MD IDIOPATHIC DISCOID LUPUS SLE SUBSETS SUBACUTE CUTANEOUS LUPUS DRUG INDUCED LUPUS NEONATAL LUPUS LATE ONSET LUPUS ANTI-PHOSPHOLIPID SYNDROME AUTOIMMUNE DISEASE IMMUNOLOGY 101: SELF VS NON-SELF AUTOIMMUNE DISEASE: PROBLEMS WITH IMMUNOLOGY 101 END RESULT: OVERACTIVE IMMUNE SYSTEM INFLAMING THE BODY ORGAN SPECIFIC OR SYSTEMIC FACTORS: GENETIC, HORMONAL, ENVIRONMENTAL 1

2 SLE CLASSIFICATION CRITERIA MALAR RASH: FIXED ERYTHEMA, FLAT OR RAISED,SPARING THE NASOLABIAL FOLDS DISCOID RASH: RAISED PATCHES, ADHERENT KERATOTIC SCALING, FOLLICULAR PLUGGING, OLDER LESIONS MAY CAUSE SCARRING PHOTOSENSITIVITY: RASH FROM SUNLIGHT SLE CLASSIFICATION CRITERIA ORAL/NASOPHARYNGEAL ULCERS: USUALLY PAINLESS ARTHRITIS: NONEROSIVE, INFLAMMATORY, IN TWO OR MORE PERIPHERAL JOINTS SEROSITIS: PLEURITIS OR PERICARDITIS SLE CLASSIFICATION CRITERIA SLE CLASSIFICATION CRITERIA RENAL DISORDER: PERSISTANT PROTEINURIA OR CELLULAR CASTS NEUROLOGIC DISORDER: SEIZURES OR PSYCHOSIS HEMATOLOGIC DISORDER: HEMOLYTIC ANEMIA, LEUKOPENIA, LYMPHOPENIA, OR THROMBOCYTOPENIA IMMUNOLOGIC DISORDER: ANTI-DsDNA ANTIBODIES OR ANTI-Sm ANTIBODIES OR ANTI-PHOSPHOLIPID ANTIBODIES ANTINUCLEAR ANTIBODIES 2

3 DIAGNOSIS 4 YEAR DELAY CLINICAL FEATURES ON PRESENTATION ARTHRITIS OR ARTHRALGIAS: 75% SKIN INVOLVEMENT: 53% RENAL: 38% CONSTITUTIONAL: 53% NEUROLOGIC: 24% RAYNAUDS: 33% ORGAN INVOLVEMENT IN SLE ARTHRITIS OR ARTHRALGIAS: 85% SKIN: 78% SEROSITIS: 40% RENAL: 74% CONSTITUTIONAL: 77% NEUROLOGIC: 54% RAYNAUDS: 60% 3

4 4

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6 ORGAN SYSTEMS PREGNANCY CARDIAC: PERICARDITIS PULMONARY: PLEURISY, PNEUMONIA NEUROLOGIC: ANY SYMPTOMS RENAL: FIVE FORMS OF INFLAMMATION HIGH RISK POPULATION! INCREASED RISK OF DISEASE FLARE IF ACTIVE DISEASE AT CONCEPTION RISK OF ADVERSE FETAL OUTCOME DOUBLES SPECIFIC PROBLEMS: ANTIPHOSPHOLIPID SYNDROME NEONATAL LUPUS SYNDROME 6

7 LABORATORY ANA 90% DSDNA 40-60% SSA (RO) 30-45% SSB (LA) 10-15% Sm 20% RNP 20-30% C3,C4 SEDIMENTATION RATE ANTIPHOSPHOLIPID SYNDROME VENOUS THROMBOSIS ARTERIAL THROMBOSIS RECURRENT FETAL LOSS EARLY MI/CVA LIVEDO RETICULARIS TREATMENT 7

8 FATIGUE COMMON!!!!!!!!!!!!! WIDE DIFFERENTIAL DIAGNOSIS: DISEASE ITSELF DEPRESSION, FIBROMYALGIA SLEEP DIFFICULTIES MEDICATIONS: STEROIDS, ALCOHOL, ANTI-HYPERTENSIVES WEAKNESS DECONDITIONING RASHES CLASSIFY THE RASH! PHOTOSENSITIVITY: COVER UP! CLOTHES BLOCKERS LIFESTYLE STEROID CREAM INTRALESIONAL INJECTIONS PLAQUENIL DAPSONE, IMMUNOSUPPRESSIVES TREATMENT OF MILD SLE ARTHRITIS: NSAIDS, PLAQUENIL CONSTITUTIONAL: LIFESTYLE MODIFICATION, PLAQUENIL ALOPECIA: PLAQUENIL, INTRALESIONAL STEROIDS RAYNAUDS: CONTROL OF ENVIRONMENT, VASODILATORS, TREATMENT OF MODERATE-SEVERE SLE CORTICOSTEROIDS IMMUNOSUPPRESSIVES CYCLOPHOSPHAMIDE AZATHIOPRINE MYCOPHENOLATE MOFITIL 8

9 TREATMENT HYDROXYCHLOROQUINE SAFE SLOW ONSET OF ACTION GOOD FOR MILD DISEASE: SKIN, JOINTS, ALOPECIA, CONSTITUTIONAL SX. TOXICITIES: OCULAR, ALLERGIC RASHES, NAUSEA STEROIDS VERY EFFECTIVE: ANTI-INFLAMMATORY IMMUNOSUPPRESSIVE FAST ONSET OF ACTION LASTING EFFECT MULTIPLE PREPARATIONS STEROIDS: TOXICITIES WEIGHT GAIN OSTEOPOROSIS AVASCULAR NECROSIS DIABETES CATARACTS, GLAUCOMA PANCREATITIS INFECTIONS POOR WOUND HEALING SALT, WATER RETENTION PSYCHIATRIC SYMPTOMS IMMUNOSUPPRESSIVES CYCLOPHOSHAMIDE: CNS, RENAL DISEASE TOXICITIES: MALIGNANCIES, INFECTIONS, HEMATOLOGIC, CONSTITUTIONAL, GI, STERILITY MYCOPHENOLATE MOFITIL: CNS, RENAL, SEVERE SKIN TOXICITIES: GI, HEMATOLOGIC, INFECTIONS AZATHIOPRINE: CNS, RENAL, SEVERE SKIN TOXICITIES: GI, HEMATOLOGIC, INFECTIONS 9

10 BELIMUMAB SURVIVAL FIRST DRUG APPROVED IN 50 YEARS TO TREAT SLE MECHANISM OF ACTION: MONOCLONAL ANTIBODY INHIBITS BIOLOGICAL ACTIVITY OF B LYMPHOCYTE STIMULATOR (BLyS) 2 STUDIES (BLISS 52,76): DECREASED DISEASE ACTIVITY, NOT ORGAN-SPECIFIC IMPROVED PROGNOSIS EARLIER DIAGNOSIS BETTER KNOWLEDGE OF THE DISEASE IMROVED LABORATORY IMPROVED/MORE RATIONAL APPROACH TO RX: STEROIDS IMMUNOSUPPRESSIVES ANTI-HYPERTENSIVES 10

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