lupus in men. sle: immunologic factors hallmark: polyclonal immune hyperactivity with increased...
TRANSCRIPT
LUPUS IN MEN
SLE: IMMUNOLOGIC FACTORS
• HALLMARK: POLYCLONAL IMMUNE HYPERACTIVITY WITH INCREASED PRODUCTION OF ANTIBODIES AGAINST “SELF” CONSTITUENTS.
• INFLUENCES:GENETICHORMONALENVIRONMENTAL
SLE CLASSIFICATION CRITERIA
• MALAR RASH: FIXED ERYTHEMA, FLAT OR RAISED,SPARING THE NASOLABIAL FOLDS
• DISCOID RASH: RAISED PATCHES, ADHERENTKERATOTIC SCALING, FOLLICULAR PLUGGING, OLDER LESIONS MAY CAUSE SCARRING
• PHOTOSENSITIVITY: RASH FROM SUNLIGHT
SLE CLASSIFICATION CRITERIA
• ORAL/NASOPHARYNGEAL ULCERS: USUALLYPAINLESS
• ARTHRITIS: NONEROSIVE, INFLAMMATORY, IN TWO OR MORE PERIPHERAL JOINTS
• SEROSITIS: PLEURITIS OR PERICARDITIS
SLE CLASSIFICATION CRITERIA
• RENAL DISORDER: PERSISTANT PROTEINURIAOR 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 ANTIBODIESOR ANTI-PHOSPHOLIPID ANTIBODIES
• ANTINUCLEAR ANTIBODIES
DIAGNOSIS
• CLINICAL!!!!!!!!!!!(CONFIRMED BY LABS)
• FEATURES: I. EPISODIC DISEASE II. MULTISYSTEM DISEASEIII. ANA PRESENTIV. AGE OF ONSET: ≥ 5 YEARS OLD
LUPUS IN MEN
• ETIOLOGY: SEX HORMONE DIFFERENCESESTROGENS 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: DISCOIDPLEURISYHEMOLYTIC ANEMIAVASCULAR: RAYNAUDS, VASCULITISOLDER 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 ITSELFDEPRESSION, FIBROMYALGIASLEEP DIFFICULTIESMEDICATIONS: STEROIDS, ALCOHOL, ANTI-HYPERTENSIVESWEAKNESSDECONDITIONING
RASHES
• CLASSIFY THE RASH!• PHOTOSENSITIVITY: COVER UP!
CLOTHESBLOCKERSLIFESTYLESTEROID CREAMINTRALESIONAL INJECTIONSPLAQUENILDAPSONE, 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
CYCLOPHOSPHAMIDEAZATHIOPRINEMYCOPHENOLATE MOFITIL
TREATMENT
• HYDROXYCHLOROQUINESAFESLOW ONSET OF ACTIONGOOD FOR “MILD”DISEASE: SKIN,
JOINTS, ALOPECIA, CONSTITUTIONAL SX.
TOXICITIES: OCULAR, ALLERGIC RASHES, NAUSEA
STEROIDS
• VERY EFFECTIVE:ANTI-INFLAMMATORYIMMUNOSUPPRESSIVE
• FAST ONSET OF ACTION• LASTING EFFECT• MULTIPLE PREPARATIONS
IMMUNOSUPPRESSIVES
• CYCLOPHOSHAMIDE: CNS, RENAL DISEASETOXICITIES: 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:
STEROIDSIMMUNOSUPPRESSIVESANTI-HYPERTENSIVES
LUPUS IN MEN
QUESTIONS?