Disorders Of Immunity Ii Autoimmune Diseases

DISORDERS OF IMMUNITY II: AUTOIMMUNE DISEASES R. HUGO MARTÍNEZ LOZANO, M.D. Aug-12-2009.

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 (Pathologic vs physiologic autoimm.) 

CRITERIA FOR DX OF AUTOIMMUNE DISEASES  



AUTOIMMUNE REACTION NOT SECONDARY TO TISSUE DAMAGE NO OTHER CAUSE

IMMUNOLOGIC TOLERANCE 



CENTRAL: DEATH OF SELF-R LYMPH (APOPTOSIS) 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 DIS. ROLES: - GENETICS - INFECTION/INFLAMMATION MUTATIONS: -AIRE -CTLA-4 -ETC.

ROLE OF INFECTIONS

ROLE OF INFECTIONS

C. EPITOPE EXPOSITION & SPREAD.

SLE: GENERAL FEATURES    

INCIDENCE: 1:700 SEX: 9:1 ETHNIC GROUP AGE:

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)

SKIN: ANTI-B.M. INJURY

Malar rash

Idem + atrophy and depigmentation

MALR RASH & TELANGIECTASIAS

Alopecia

VASCULAR INJURY

Diffuse Proliferative glomerulonephritis: Prognosis…

i o n s o f S y s t e m i LES: c ULTRASTRUCTURE. L u p u s E r

Anterior uveitis

LIBMAN-SACHS ENDOCARDITIS

SLE: DIAGNOSIS 

THE 11 CRITERIA CLASS…



ANTI DOUBLE-STRANDED DNA Abs.

(P. 228)

SUBCUTANEOUS LE  

DR3 SS-A

DRUG-INDUCED LE      

HYDRALAZINE PROCAINAMIDE ISONIAZID CLINDAMYCIN OTHER … ANTI-HISTONES Abs.

R.A.

Pannus

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… ANTI- SS-A & SS-B Abs.

SYSTEMIC SCLEROSIS (SCLERODERMIA) DEFINITION: ABNORMAL FIBROUS TISSUE IN SKIN & MULTIPLE ORGANS  TYPES:- DIFFUSSE SD - LIMITED SD - CREST SYNDROME DX: - DNA TOPOISOMERASE-I (ANTI-SCL 70) (Diffuse) - ANTICENTROMERE (90%) (Crest) 

SD: MORPHOLOGY

CREST SX:…REYNAUD’S PHENOMENON

SEVERE REYNAUD + NECROSIS

LESIONS?

CALCINOSIS

SUBCUTANEOUS CALCINOSIS

ESOPHAGEAL INVOLVEMENT

DX?

QUESTIONS?