SCLERODERMADEFINATION :-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 ORGANS-GIT,LUNGS,HEART,& KIDNY

CLASSIFICATION OF SCLERODERMASYSTEMIC SCLEROSIS

1 .LIMITED CUTANEOUS DISEASE 2.DIFFUSE CUTANEOUS DISEASE 3.SINE SCLERODERMA 4.OVERLAPE SYNDROME

LOCALIZED SCLERODERMA 1.MORPHEA 2.LINER SCLERODERMA

3.En COUP de sabre CHEMICALLY INDUCED SCLERODERMA TOXIC-OIL SYNROMEVINYL CHLORIDE BLEOMYCINEPENTAZOCINE

EPOXI & AROMATIC HYDROCARBONES

PATHOGENESISHOSTGENETIC SUSCEPTIBILITYINFECTIONENVIRONMENTAL FACTORSMICROCHIMERISM

VASCULARENDOTHELIAL CELL INJURYVASCULAR OCCLUSIONTISSUE HYPOXIA

IMMUNET CELL ACTIVATIONMACROPHAGE ACTIVATIONAUTOANTIBODIESCYTOKINES

FIBROBLAST ACTIVATION

FIBROSIS

CLINICAL PRESENTATION OF S SRAYNAUD’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 SCLEROSISDIFFUSEDIFFUSE LIMITEDLIMITED

SKIN INVOLVEMENTSKIN INVOLVEMENT

RAYNAUD’S RAYNAUD’S PHENOMENONPHENOMENON

ORGAN ORGAN INVOLVEMENTINVOLVEMENT

NAIL FOLD NAIL FOLD CAPILLARIESCAPILLARIES

ANTINUCLEAR ANTINUCLEAR ANTIBODIESANTIBODIES

DISTAL & DISTAL & PROXIMAL PROXIMAL EXTREMITIES,FACEXTREMITIES,FACE,E,

TRUNKTRUNK

ONSET WITH IN 1 ONSET WITH IN 1 YEAR OR AT THE YEAR OR AT THE TIME OF SKIN TIME OF SKIN CHANGESCHANGES

PULMONARY,PULMONARY,

RENAL,GITRENAL,GIT

DILATATION & DILATATION & DROPOUTDROPOUT

ANTITOPOISOMERASE ANTITOPOISOMERASE 1 ~ 40 %1 ~ 40 %

DISTEL TO DISTEL TO ELBOW,FACEELBOW,FACE

MAY PRECEDS SKIN MAY PRECEDS SKIN LESIONSLESIONS

ORGAN ORGAN INVOLVEMENT INVOLVEMENT 10TO 15 YEARS 10TO 15 YEARS AFTER OF DISEASE AFTER OF DISEASE

DILATATION DILATATION WITHOUT DROPOUTWITHOUT DROPOUT

ANTICENTROMEREANTICENTROMERE

60 to 80 %60 to 80 %

MANAGEMENTD-PENICILLAMINE 750 TO 1000 mg/DAYIMMUNOSUPPRESIVE CYCLOPHOSPHMIDE CORTICOSTEROIDSCYCLOSPORINE

ANTITHROMBOCYTE AGENTSVASODILATORS

CALCIUM CHANNEL BLOCKERS-NIFEEDIPINEPENTOXIPHYLLINESKIN CAREPHYOTHERAPY