Cellular and humoral immunologic
derangement
Clinical Manifestation Treatment
 Vascular abnormalities
 Skin involvement
 Musculoskeletal system
 Intestinal Involvement
 Lungs
 Heart
 Kidney
 Exocrine Glands
Disease Modifying Treatment:
Activation of the immune system
is an outstanding disease feature
Diagnostic Investigations
 No therapy has been shown • Serumauto-antibodies,
significantly to alter the upper GI endoscopy
natural history SSc till date. • FBC, CT scan of chest
 Multiple interventions are • Urea &’ serum creatinine
available in allevating the • ESR, serum muscle
symptoms- slowly progression enzymes
of organ damage.
 Treatment approaches to be
individually tailored.
 Optimal management-
prompt, accurate diagnosis,
classification, risk stratify,early
recognition of organ based
complications, regular
monitoring of progression,
disease activity, response to
treatment, patient education
 Holistic approach
 Combination of drugs are
used
 Physician- pt. relationship to
be maintained
• Immunosuppressive agents
• Glucocorticoids
• Cyclophosphamide
• Methotrexate
• Mycophenolate mofetil
• Immunomodulation
• Immune ablation
• Stem cell transplantations
 SCLERODERMA

-a chronic connective tissue disease generally

classified as one of the autoimmune rheumatic
diseases.

Localized Scleroderma- found Systemic Sclerosis- is a multi-

systemic, autoimmune disease
onlya few places on the skin or
affecting small arteries, micro-
muscles and rarely spread
vessels and fibroblasts resulting in
elsewhere. (MILD)
vascular obliteration, collagen
accumulation and scarring (fibrosis)
of skin and internal organs.

Morphea waxy Linear Scleroderma

patches on the skin of starts as a streak or line
varying sizes, shapes & of hardened, waxy skin DIFFUSE CUTANEOUS LIMITED CUTANEOUS
color. Usually appears on crease on the head or cutaneous scleroderma - skin induration limited to
the age’s 20 & 50 but neck referred to as en begins with symmetrical hands, feet, face. CREST
often seen in young coup de sabre. Usually widespread thickening  Calsinosis: Calcium
children. develops in childhood of the skin on the deposits in the tissues.
extremeties, the face  Raynaud’s Phenomenon:
and the trunk. Intermittent vasospasm
Plaque
In the early stages of the of finger tips.
Guttate
disease: bilateral  Esophageal hardening:
Generalized
symmetrical swelling of Sclerosis of the
Bulous
the fingers, face & feet esophagus
Deep
-Skin has a tense, wrinkle-  Sclerodactyly:
free appearance, more Scleroderma of the digits
thickened, hidebound &  Telangiectasias: Capillary
shiny dilations that form
vascular lesions on the
face, lips, and fingers.

PATHOPHYSIOLOGY

Endothelial cell injury

Fibroblast activation