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SYSTEMIC SCLEROSIS
PRESENT BY: JEANNE D‘ARCMVUGO
DP: PHYSICAL MEDICINE AND
REHABILITATION
DEFINITION
• Systemic sclerosis is a generalized disorder of connective tissue affecting the skin,
internal organs and vasculature.
CAUSES OF SYSTEMIC SCLEROSIS
• SS occurs when your body begins to overproduce collagen and it accumulates in
your tissues. Collagen is the main structural protein that makes up all of your
tissues.
• Doctors aren’t sure what causes the body to produce too much collagen. The
exact cause of SS is unknown
DIAGNOSIS OF SYSTEMIC SCLEROSIS
• During a physical exam, your doctor can identify skin changes that are symptomatic of SS.
• High blood pressure may be caused by kidney changes from sclerosis. Your doctor may
order blood tests like antibody testing, rheumatoid factor, and sedimentation rate.
• Other diagnostic tests can include:
• a chest X-ray
• a urinalysis
• a CT scan of the lungs
• skin biopsies
SCLERODERMA: EDEMATOUS CHANGES,
HANDS
SYSTEMIC SCLEROSIS
 Fibrotic contractures .
 Acrolysis of the distal phalanges .
 Pigmentation .
 Spots of depigmentation
• Skin involvement restricted to sites distal to
the elbow or knee (apart from the face) is
classified as 'limited cutaneous
disease' or CREST syndrome …
Involvement proximal to the knee and elbow
and on the trunk is classified as 'diffuse
cutaneous disease'. fingertips.
SCLERODERMA
Head and Neck Manifestations
• tight skin, thin lips, vertical perioral furrows
• dermal and subcutaneous inflammatory process
• edema preceeds epidermal atrophy, loss of appendages
SCLERODERMA
Head and Neck Manifestations
• decreased mouth opening
• Peaked nose
• Tight neck bands
SCLERODERMA
• facial changes
• marked telangiectasia
RAYNAUD’S PHENOMENON, BLANCHING OF
HANDS
• problem that causes decreased blood
flow to the fingers. In some cases, it
also causes less blood flow to the
ears, toes, nipples, knees, or nose.
This happens due to spasms of blood
vessels in those areas. The spasms
happen in response to cold, stress, or
emotional upset.
MUSCULOSKELETAL FEATURES
• Arthralgia, morning stiffness and flexor tenosynovitis
are common. Restricted hand function is due to skin
rather than joint disease and erosive arthropathy is
uncommon. Muscle weakness and wasting are usually
due to myositis.
GASTROINTESTINAL FEATURES
• Gut involvement is common. Smooth muscle atrophy and
fibrosis in the lower two-thirds of the oesophagus lead to
acid reflux with erosive oesophagitis. Since this may progress to
further fibrosis, adequate treatment of reflux (usually with
proton pump inhibitors) is important. Dysphagia and
odynophagia (painful dysphagia) may also occur.
CARDIORESPIRATORY FEATURES
• Pulmonary involvement is a major cause of morbidity and
mortality. Fibrosing alveolitis mainly affects patients with
diffuse disease, particularly those with antibodies to
topoisomerase 1. Pulmonary hypertension is a complication of
long-standing disease and is six times more prevalent in limited
than in diffuse disease.
RENAL FEATURES
• One of the main causes of death is hypertensive renal crisis
characterized by rapidly developing malignant hypertension and
renal failure. Treatment is by angiotensin-converting enzyme
(ACE) inhibition even if renal impairment is present.
Hypertensive renal crisis is much more likely to occur in
patients with diffuse rather than limited disease
CREST SYNDROME: CALCINOSIS CUTIS,
FINGERS
SCLERODERMA: CALCINOSIS AND
ACROLYSIS (RADIOGRAPH)
Symptoms of Systemic Sclerosis
SS may only affect the skin in the early stages of the disease. You may notice your skin thickening and
shiny areas developing around your mouth, nose, fingers, and other bony areas.
As the condition progresses, you may begin start to have limited movement of the affected areas. Other
symptoms include:
•hair loss
•calcium deposits, or white lumps under the skin
•small, dilated blood vessels under the skin’s surface
•joint pain
•shortness of breath
•a dry cough
•diarrhea
•constipation
•difficulty swallowing
•esophageal reflux
•abdominal bloating after meals
You may begin to experience spasms of the blood vessels in your fingers and toes. Then, your
extremities may turn white and blue when you’re in the cold or feeling extreme emotional stress. This is
called Raynaud’s phenomenon.
TREATMENT FOR SYSTEMIC SCLEROSIS
• Treatment can’t cure the condition, but it can help reduce symptoms and slow disease
progression. Treatment is typically based on a person’s symptoms and the need to prevent
complications.
• Treatment for generalized symptoms may involve:
• corticosteroids
• immunosuppressants, such as methotrexate or Cytoxan
• nonsteroidal anti-inflammatory drugs
• Depending on your symptoms, treatment can also include:
• blood pressure medication
TREATMENT FOR SYSTEMIC SCLEROSIS
• medication to aid breathing
• physical therapy
• light therapy, such as ultraviolet A1 phototherapy
• nitroglycerin ointment to treat localized areas of tightening of the skin
• You can make lifestyle changes to stay healthy with scleroderma, such
as avoiding smoking cigarettes, remaining physically active, and
avoiding foods that trigger heartburn.
THANK YOU .