AUTOIMMUNE DISEASES R. HUGO MARTÍNEZ LOZANO, M.D. Sep-13, 2006
LEARNING OBJECTIVES K & U: - GENERAL FEATURES - IMMUNOLOGIC TOLERANCE - MECHANISMS OF AUTOIMMUNITY - THE MOST IMPORTANT CHARCT. OF: - LES - RA - SS - SYSTEMIC SCLEROSIS Ref: 223-239 & lecture’s information
GENERAL FEATURES
FRECUENCY: DISEASE VS NORMALITY
CRITERIA FOR DX OF AUTOIMMUNE DISEASES
AUTOIMMUNE REACTION NOT SECONDARY TO TISSUE DAMAGE NO OTHER CAUSE
IMMUNOLOGIC TOLERANCE
CENTRAL: DEATH OF SELF-R LYMPH PERIPHERAL: - ANERGY: NO COSTIMULATORS B7 INHIBITORY R CTLA-4 - SUPPRESSION BY REGULATORY T - CLONAL DEL. BY ACTIVATION OF INDUCED CELL DEATH (FASL-FAS)
MECHANISMS OF AUTOIMMUNE DISEASES
ROLE OF INFECTIONS
ROLE OF INFECTIONS
C. EPITOPE EXPOSITION & SPREAD.
SLE: GENERAL FEATURES
INCIDENCE: 1:700 SEX: 9:1 ETHNIC GROUP
CLINICAL CRITERIA: (TABLE 6-8. p. 228)
MALAR RASH DISCOIDE RASH PHOTOSENSITIVITY ORAL ULCERS ARTHRITIS SEROSITIS RENAL INVOLVEMENT ANA ETC.
ANA
GENERIC ANTI-DOBLE-STRANDED DNA ANTI-SMITH (Sm) ANTIHISTONE (DRUG-INDUCED LE) SS-A & B (RNP) ANTICENTROMERE (CREST)
RISK FACTORS
GENETIC ENVIRONMENTAL IMMUNOLOGIC
PATHOGENESIS: VASCULITIS (PHASES)
PENPHYGUS VULGARIS
SKIN: ANTI-B.M. INJURY
SKIN RASH
VASCULAR INJURY: GN
i o n s o f S y s t e m i LES: c ULTRASTRUCTURE. L u p u s E r
LIBMAN-SACHS ENDOCARDITIS
SUBCUTANEOUS LE
DR3 SS-A
DRUG-INDUCED LE
HYDALAZINE ISONIAZID OTHER
R.A.
SEVERE INTESTINAL VASCULITIS
SJÖGREN SYNDROME (SS)
S.S.
DEFINITION: XEROSTOMIA K-C SICCA
S.S.
FORMS: PRIMARY (SICCA SYNDROME) SECONDARY
RISK FACTORS: HLA: B8, DR3, DRW52, ETC. EBV, HCV, HTLV-1 MORPHOLOGY…
SYSTEMIC SCLEROSIS (SCLERODEMIA) DEFINITION: ABNORMAL FIBROUS TISSUE IN SKIN & MULTIPLE ORGANS TYPES:- DIFFUSSE SD - LIMITED SD - CREST SYNDROME DX: - DNA TOPOISOMERASE-I (ANTI-SCL 70) - ANTICENTROMERE
SD: MORPHOLOGY
SEVERE REYNAUD + NECROSIS
LESION?
CALCIONOSIS
SUBCUTANEOUS CALCINOSIS
ESOPHAGEAL INVOLVEMENT
DX?
QUESTIONS?