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1. Early Symptoms
The most common early symptoms of SSc are
Raynaud’s phenomenon and insidious edematous skin changes of extremities and
face. About 70% of the cases first present with Raynaud’s phenomenon, which
appears either 1-2 years prior to or simultaneously with other manifestations. Polyarthral
lesions are also prominent early manifestations. Patients may complain of
irregular fever, appetite and weight loss before onset of symptoms.
2. Skin Changes
According to the skin changes, SSc can
be divided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc
(dcSSc), but the former is more common. These two types are different in the
skin involvement area. Lesions of lcSSc begin in the areas of hands, feet and
face, which are limited, progress slowly and have a good prognosis, while those
of dcSSc are more diffused. Skin lesions may develop into three phases, named swelling,
hardening and atrophy.
(1)
Swelling Phase
The skin is taut, thickened, with wrinkles
disappearing, pale or light yellow, lower skin temperature and nonpitting
edema. The skin lesions of lcSSc usually start from hands, feet and face, and
spread into upper limbs, shoulders and neck. The lesions of dcSSc, however, can
also be seen in body trunk.
(2)
Hardening phase
The skin gets hardened with a wax-like
shine and cannot be pinched up by fingers. As the lesions occur in different
areas, a variety of symptoms may be presented, such as restricted finger
flexion, fixed facial expression, difficulties in opening mouth and closing
eyes and chest tightness. In the involved skin, pigmentation, hypopigmentation
and oligotrichosis can be seen. Besides, itchiness and paraesthesia may also
happen at the same time.
(3)
Atrophy phase
The skin undergoes parchment-like change of
thinning and atrophy. In some cases, even the subcutaneous tissue and muscles
are weakened and hardened, which tightly stick to the skeleton. Pitting scars
are formed on extremities and usually cause intractable skin joints and ulcers.
Hair loss and hypohidrosis can be seen. Few cases may present with
telangiectasia.
The skin lesions above are the most
prominent in all types of SSc. But it should be noted that some SSc remain
asymptomatic in the skin.
3. Muscle Involvement
Muscle involvement is not rare. Abnormal
electromyography is viewed in about 50% of the cases early in the disease.
Clinical manifestations include myasthenia, diffuse myalgia and
polymyositis-like symptoms in some cases. Severe involvement of muscle may
advance into amyotrophy.
4. Skeleton and joint change
Inflammatory joint pain is not rare in
initial stage, which occurs in 12% of the cases in their early manifestations
and 46% in the overall course of the disease. Patients may have slight limit in
range of movement, while severe cases shows ankylosis or even contracture and
deformity of the joints. Hand involvement is most common, which can be measured
as total stiffness, shortening in fingers and deformation. Chronic dactyl
ischemia, however, may result in osteolysis in terminal phalanges, which can be
viewed in X-ray. Other imaging findings include joint space narrowing, bone
sclerosis adjacent to articular surface and osteoporosis.
Visceral organ involvement is the critical
feature to differ dcSSc from lcSSc:
(1) Gastrointestinal System
Tongue movement may be limited by
contracture of the glossodesmus and apical root resorption causes loosening of
the teeth. Esophageal involvement is quite common (45%-90%) and dysfunction of
the lower esophageal sphincter may result in heartburn and sour regurgitation.
If it lasts long, complications like erosive esophagitis, bleeding and lower
esophageal stenosis may arise. Bradydiastalsis of the lower 2/3 of the
esophagus causes dysphagia and odynophagia. Among the cases with Abnormal X-ray
results, about 40% do not have any symptoms. Part of the patients may present
with abdominal pain, abdominal distension and alternating diarrhea and constipation.
(2) Cardiovascular System
Cardiac involvement of various degree
occurs in about 61% of the patients, which can be myocarditis, pericarditis and
endocarditis. Clinical manifestations include dyspnea, chest distress, angina, arrhythmia,
left or whole heart failure in severe cases (or right heart failure as a consequence
of pulmonary heart disease caused by lung lesion), and even sudden cardiac
death. Electrocardiographic abnormality is discovered in approximately 50% of
the patients.
(3) Respiratory System
Abnormal chest imaging results are
discovered in 40% of the patients, most of which are diffuse interstitial
fibrosis of the lungs. Pulmonary dysfunction is also noticed, with a 70%
decrease in vital capacity. Clinical manifestations include cough and
progressive dyspnea.
(4) Urology System
75% of the patients are discovered to have kidney
involvement, which can be sclerosing glomerulonephritis accompanied by proteinuria,
hypertension, nitremia, and acute kidney injury in severe cases. Prognosis of
individuals with kidney damage is often poor.
(5) Nervous and Mental System
A small number of patients suffer from
polyneuritis (cranial nerves included), convulsions, seizure, personality
change, cerebral arteriosclerosis or cerebral hemorrhage. Accesory examinations
show protein elevation in cerebrospinal fluid and abnormality in
electroencephalogram.
6. Others
(1) Calcium deposits occur in the skin above
the finger and other joints and soft tissues in the extension sides of the
limbs.
(2) CREST Syndrome
CRST syndrome is named according to calcinosis cutis, Raynaud’s phenomenon, sclerodactyly and telangiectasia. Cases of CRST
syndrome that present with esophagus
hypomotility are called CREST syndrome, which is a subtype of SSc, whose
prognosis is good.