Transcript SYSTEMIC LUPUS ERYTHEMATOSUS

SCLERODERMA
 DEFINATION : CHRONIC MULTISYSTEM DISORDER OF
UNKNOWN ETIOLOGY CHARECTERISED
CLINICALLY BY THICKENING OF SKIN CAUSED
BY ACCUMULATION OF CONNECTIVE TISSUE
AND BY STRUCTURAL & FUNCTIONAL
ABNORMALITIES OF VICERAL ORGANSGIT,LUNGS,HEART,& KIDNY
CLASSIFICATION OF SCLERODERMA
 SYSTEMIC SCLEROSIS
.LIMITED CUTANEOUS DISEASE
CUTANEOUS DISEASE
4.OVERLAPE SYNDROME
 LOCALIZED SCLERODERMA
2.LINER SCLERODERMA
1
2.DIFFUSE
3.SINE SCLERODERMA
1.MORPHEA
3.En COUP de
sabre
 CHEMICALLY INDUCED SCLERODERMA
 TOXIC-OIL SYNROME
 VINYL CHLORIDE
 BLEOMYCINE
 PENTAZOCINE
 EPOXI & AROMATIC HYDROCARBONES
PATHOGENESIS
HOST
GENETIC SUSCEPTIBILITY
INFECTION
ENVIRONMENTAL FACTORS
MICROCHIMERISM
IMMUNE
VASCULAR
ENDOTHELIAL CELL INJURY
VASCULAR OCCLUSION
TISSUE HYPOXIA
T CELL ACTIVATION
MACROPHAGE ACTIVATION
AUTOANTIBODIES
CYTOKINES
FIBROBLAST ACTIVATION
FIBROSIS
CLINICAL PRESENTATION OF S S
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RAYNAUD’S PHENOMENON 90 TO 100 %
SKIN THICKENING
98 TO 100 %
TELENGIECTASIA
40 TO 85 %
ARTHRALGIAS
40 TO 70 %
ESOPHAGEAL DYSMOTILITY ~ 80 %
PULMONARY FIBROSIS
~40 %
MYOPATHY
~50 %
RENAL CRISIS
~ 15 %
CARDIAC DISEASE
~ 10%
SUBSET OF SYSTEMIC SCLEROSIS
DIFFUSE
SKIN INVOLVEMENT
DISTAL &
PROXIMAL
EXTREMITIES,FACE,
TRUNK
RAYNAUD’S
ONSET WITH IN 1
PHENOMENON
YEAR OR AT THE
TIME OF SKIN
CHANGES
ORGAN INVOLVEMENT PULMONARY,
RENAL,GIT
NAIL FOLD
CAPILLARIES
DILATATION &
DROPOUT
ANTINUCLEAR
ANTIBODIES
ANTITOPOISOMERASE 1
~ 40 %
LIMITED
DISTEL TO
ELBOW,FACE
MAY PRECEDS SKIN
LESIONS
ORGAN INVOLVEMENT
10TO 15 YEARS
AFTER OF DISEASE
DILATATION
WITHOUT DROPOUT
ANTICENTROMERE
60 to 80 %
MANAGEMENT
 D-PENICILLAMINE 750 TO 1000 mg/DAY
 IMMUNOSUPPRESIVE
 CYCLOPHOSPHMIDE
 CORTICOSTEROIDS
 CYCLOSPORINE
 ANTITHROMBOCYTE AGENTS
 VASODILATORS
 CALCIUM CHANNEL BLOCKERS-NIFEEDIPINE
 PENTOXIPHYLLINE
 SKIN CARE
 PHYOTHERAPY