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LUPUS IN MEN

LUPUS IN MEN. SLE: IMMUNOLOGIC FACTORS. HALLMARK: POLYCLONAL IMMUNE HYPERACTIVITY WITH INCREASED PRODUCTION OF ANTIBODIES AGAINST “SELF” CONSTITUENTS. INFLUENCES: GENETIC HORMONAL ENVIRONMENTAL. SLE CLASSIFICATION CRITERIA.

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LUPUS IN MEN

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  1. LUPUS IN MEN

  2. SLE: IMMUNOLOGIC FACTORS • HALLMARK: POLYCLONAL IMMUNE HYPERACTIVITY WITH INCREASED PRODUCTION OF ANTIBODIES AGAINST “SELF” CONSTITUENTS. • INFLUENCES: GENETIC HORMONAL ENVIRONMENTAL

  3. 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

  4. SLE CLASSIFICATION CRITERIA • ORAL/NASOPHARYNGEAL ULCERS: USUALLY PAINLESS • ARTHRITIS: NONEROSIVE, INFLAMMATORY, IN TWO OR MORE PERIPHERAL JOINTS • SEROSITIS: PLEURITIS OR PERICARDITIS

  5. SLE CLASSIFICATION CRITERIA • RENAL DISORDER: PERSISTANT PROTEINURIA OR CELLULAR CASTS • NEUROLOGIC DISORDER: SEIZURES OR PSYCHOSIS • HEMATOLOGIC DISORDER: HEMOLYTIC ANEMIA, LEUKOPENIA, LYMPHOPENIA, OR THROMBOCYTOPENIA

  6. SLE CLASSIFICATION CRITERIA • IMMUNOLOGIC DISORDER: ANTI-DsDNA ANTIBODIES OR ANTI-Sm ANTIBODIES OR ANTI-PHOSPHOLIPID ANTIBODIES • ANTINUCLEAR ANTIBODIES

  7. DIAGNOSIS • CLINICAL!!!!!!!!!!! (CONFIRMED BY LABS) • FEATURES: I. EPISODIC DISEASE II. MULTISYSTEM DISEASE III. ANA PRESENT IV. AGE OF ONSET: ≥ 5 YEARS OLD

  8. LUPUS IN MEN • ETIOLOGY: SEX HORMONE DIFFERENCES ESTROGENS V.S. ANDROGENS • INCIDENCE: FOR EVERY MALE WITH LUPUS, THERE ARE NINE FEMALES • MAKING THE DIAGNOSIS…………DELAYED! WHY?

  9. LUPUS IN MEN • SYMPTOMS: DO THEY DIFFER? ARE THEY MORE SEVERE? SKIN: DISCOID PLEURISY HEMOLYTIC ANEMIA VASCULAR: RAYNAUDS, VASCULITIS OLDER ONSET?

  10. LUPUS IN MEN • TESTING: SIMILAR IN MALES, FEMALES • WHAT IS THE ROLE OF AN ANA? • RISKS FOR OTHER FAMILY MEMBERS • IS THE DISEASE MONITORED DIFFERENTLY? • ARE DIFFERENT DRUGS USED FOR MEN?

  11. LUPUS IN MEN • IS SEXUAL FUNCTION EFFECTED IN LUPUS MEN? • ARE TESTOSTERONE LEVELS ALTERED? • DO THE DRUGS ALTER THESE? • WHAT IS THE PSYCHOLOGICAL IMPACT?

  12. TREATMENT

  13. FATIGUE • COMMON!!!!!!!!!!!!! • WIDE DIFFERENTIAL DIAGNOSIS: DISEASE ITSELF DEPRESSION, FIBROMYALGIA SLEEP DIFFICULTIES MEDICATIONS: STEROIDS, ALCOHOL, ANTI-HYPERTENSIVES WEAKNESS DECONDITIONING

  14. RASHES • CLASSIFY THE RASH! • PHOTOSENSITIVITY: COVER UP! CLOTHES BLOCKERS LIFESTYLE STEROID CREAM INTRALESIONAL INJECTIONS PLAQUENIL DAPSONE, IMMUNOSUPPRESSIVES

  15. TREATMENT OF MILD SLE • ARTHRITIS: NSAIDS, PLAQUENIL • CONSTITUTIONAL: LIFESTYLE MODIFICATION, PLAQUENIL • ALOPECIA: PLAQUENIL, INTRALESIONAL STEROIDS • RAYNAUDS: CONTROL OF ENVIRONMENT, VASODILATORS,

  16. TREATMENT OF MODERATE-SEVERE SLE • CORTICOSTEROIDS • IMMUNOSUPPRESSIVES CYCLOPHOSPHAMIDE AZATHIOPRINE MYCOPHENOLATE MOFITIL

  17. TREATMENT • HYDROXYCHLOROQUINE SAFE SLOW ONSET OF ACTION GOOD FOR “MILD”DISEASE: SKIN, JOINTS, ALOPECIA, CONSTITUTIONAL SX. TOXICITIES: OCULAR, ALLERGIC RASHES, NAUSEA

  18. STEROIDS • VERY EFFECTIVE: ANTI-INFLAMMATORY IMMUNOSUPPRESSIVE • FAST ONSET OF ACTION • LASTING EFFECT • MULTIPLE PREPARATIONS

  19. 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

  20. BELIMUMAB • 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”

  21. IMPROVED PROGNOSIS • EARLIER DIAGNOSIS • BETTER KNOWLEDGE OF THE DISEASE • IMROVED LABORATORY • IMPROVED/MORE RATIONAL APPROACH TO RX: STEROIDS IMMUNOSUPPRESSIVES ANTI-HYPERTENSIVES

  22. LUPUS IN MEN QUESTIONS?

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