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
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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?