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

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?