LUPUS IN MEN. SLE: IMMUNOLOGIC FACTORS HALLMARK: POLYCLONAL IMMUNE HYPERACTIVITY WITH INCREASED PRODUCTION OF ANTIBODIES AGAINST “SELF” CONSTITUENTS.

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Presentation transcript:

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 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 RENAL DISORDER: PERSISTANT PROTEINURIA OR CELLULAR CASTS NEUROLOGIC DISORDER: SEIZURES OR PSYCHOSIS HEMATOLOGIC DISORDER: HEMOLYTIC ANEMIA, LEUKOPENIA, LYMPHOPENIA, OR THROMBOCYTOPENIA

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

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

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?

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

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?

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

TREATMENT

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

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

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

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”

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

LUPUS IN MEN QUESTIONS?