By Lawrence. G.
Kibanda
OBJECTIVES
Meaning and origin of the word Scleroderma
Types of scleroderma and its classification
Etiology of scleroderma
Epidemiology of scleroderma
Pathophysiology of scleroderma
Clinical manifestations
Diagnosis
Treatment
Management
Meaning and origin
The word scleroderma comes from the Greek word
“Sclero” means hard and “derma” means skin.
Scleroderma is the autoimmune disease that causes the
body to produce too much collagen leading to hardening
and thickening of the skin , and some cases damage to
internal organs.
Types of scleroderma and its
classification
There are two main types of scleroderma, which are
1) Localized scleroderma
2) Systemic scleroderma
Localized scleroderma
Scleroderma that causing inflammation and hardening of the
skin and underlying tissues in specific body areas. Affects only
skin and structures beneath but not internal organs.
Can be classified into
i. Morphea , discolored patches or plagues on the skin.
ii. Linear, lines or streaks of thickened skin often appear on Arm
,legs or fore head.
Localized scleroderma
Morphear Liner
Types
Systemic scleroderma
Affect the skin and internal organs such as lungs ,kidneys, and
digestive tract.
Can be classified into
i. Limited cutaneous systemic sclerosis , marked by skin
thickening primarily on the face, hands , feet and lower arms,
sometimes it is called CREST syndrome due to its
manifestation symptoms.
ii. Diffuse cutaneous systemic sclerosis , wide spread skin
involvement including trunk ,associated with severe internal
organ involvement and higher mortality.
Systemic scleroderma
Limited cutaneous systemic Diffuse cutaneous systemic
sclerosis sclerosis
Etiology
The exact etiology of scleroderma is not well understood
and known but it is believed that genetic and
environmental factors contribute to its development.
A. Genetic factor, Scleroderma shows family genes
inherited characters, some one might be born with a
permissive genetic back ground that certain genes
make the immune system to overreact.
B. Environmental factor, Out side factors act as the
facilitator to the scleroderma ,this is due to exposure
to certain chemicals like silica dust or organic solvents
and viruses contamination.
Epidemiology
Scleroderma is considered a rare disease but its impact
varies significantly depending on demographics.
Scleroderma shows strong female predominance ,affect
women more than men with ratio 5:1
It can affect at any age but most common between 45
and 54.
African Americans have a higher incidence of develop
the diffuse form of the disease often appears at young
age between 15 and 24.
Pathophysiology
The exact cause of scleroderma is still researched and not entirely
understood ,but the occurrence can be explained in 3 biological events.
[Link] injury
First thing happens is damage to the thin lining of the small blood vessels.
The body tries to fix these vessels but the repair process is leaky ,this causes
blood vessels to narrow and eventually disappear which is why Raynaud’s
phenomenon is often first symptoms.
[Link] activation
As vessels are damaged they release danger signals that alert immune
system.
Body produces specific autoantibodies that mistakenly target it’s own proteins.
T cell and B cell rush to the site of the vascular injury releasing cytokines that
calling more cells to the area and creating chronic inflammation.
[Link] (hardening)
Cytokines from the immune stage act on fibroblasts ,normally they make
collagen to keep skin health but in scleroderma they turn into myofibroblasts.
The hyperactive cells pumps out massive amounts of collagen and other
proteins ,this builds up in the space between cells making the skin and organs
thick , stiff and scarred.
These processes result in irreversible tissue damage and organ dysfunction.
Clinical manifestations
i. Skin ; skin thickening , sclerodactyly , telangiectasia .
ii. Vascular ; Raynaud phenomena , digital ulcers.
iii. Musculoskeletal ; tendon friction rubs , arthralgia
myopathy.
iv. Gastrointestinal ; esophageal dysmotility reflux ,
malabsorption.
v. Pulmonary ; interstitial lung disease , pulmonary
arterial hypertension.
vi. Renal ; scleroderma renal crisis.
[Link] ; arrhythmias , cardiomyopathy , pericardial
disease.
Diagnosis
It base on 3 key steps
[Link] symptoms and physical examination.
Doctor check and note the symptoms on skin changes ,heart
burn ,swallowing difficulty , shortness of breath ,joint pain and fatigue.
Physical signs , check for skin thickening ,puffy fingers and digital ulcers.
[Link] tests (auto antibodies)
Screening test on auto antibodies(ANA) is positive in most scleroderma
patients, if ANA is positive more specific tests helps on classify the disease.
SCI-70 (Anti topoisomerase1), linked to diffuse scleroderma.
Anti centromere , associated with limited scleroderma.
RNA polymerase iii, linked to diffuse scleroderma and kidney risk.
[Link] and function tests
Pulmonary function tests ,to check for lung fibrous or pulmonary
hypertension.
Echocardiogram ,to assess heart function.
Treatment
There is no single cure or specific cure for scleroderma ,so
it’s treatment focus on controlling symptoms, preventing
complications and slowing organ damage.
Treatment by clinical features
A)Skin thickening and inflammation
Methotrexate
Mycophenolate mofetil
Cyclophosphamide
B)Raynaud’s phenomena
Calcium channel blockers
ACE inhibitors or ARBs
re a tm e n t
T
C)Digital ulcers
Bosentan ,for preventing new ulcers
Prostacyclin analogue
D)Gastrointestinal involvement
Proton pump inhibitors
Pro kinetic agents
E)Lung involvement
Nintedanib , are anti fibratic
Prostacyclin analogues
F)Renal crisis
ACE inhibitors
G)Joint and muscle pain
Behaviors that helps on control and
management
Stop smoking and using of chemicals
Doing regular exercise
Obtaining and involve in vaccinations
Doing regular skin care
Keeping the body warm
Thank you for listening
By king Larry