The earliest manifestation of systemic sclerosis (also known as scleroderma) can be seen in the skin according to a study from the American Journal of Clinical Dermatology. In patients with systemic sclerosis, the skin can become thick and velvety with a waxy appearance due to excess collagen deposits.
This can lead to changes in skin texture and accumulation of skin folds in certain areas. Additionally, early signs of systemic sclerosis can include swelling and stiffness of the hands and feet, as well as Raynaud’s phenomenon, where the fingers and toes experience a temporary lack of blood supply due to small blood vessels constricting.
Over time, parts of the skin may also become itchy, tight, and shiny due to excessive collagen accumulation. Other symptoms of systemic sclerosis include fatigue, chest pain, difficulty swallowing, loss of appetite, and dry mouth.
If untreated, systemic sclerosis can progress and lead to more serious medical complications, such as pulmonary hypertension, organ fibrosis, and cardiopulmonary failure.
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How quickly does systemic sclerosis progress?
Systemic sclerosis (also called scleroderma) is a difficult condition to predict— often, the progression of the disease differs widely from person to person. Some cases of scleroderma progress rapidly, while others stay fairly stable or even resolve over time.
Factors such as age, gender, and severity of the condition can affect the rate of progression. Women tend to progress more quickly than men, as do individuals under the age of 50. Severe cases can progress quickly, while milder forms of the disease may be more manageable and less progressive.
In general, systemic sclerosis can progress in three stages— active, stabilized, or progressive. During the active stage, new symptoms can appear and existing symptoms may worsen. During the stabilized phase, symptoms tend to remain the same and don’t worsen.
The progressive stage is characterized by a worsening of existing symptoms as well as an increase in new symptoms.
Overall, it is difficult to predict how quickly systemic sclerosis may progress as it varies so significantly from person to person. However, in general, individuals younger than 50 and those with more severe forms of systemic sclerosis may experience a more rapid progression of the disease.
Where does scleroderma usually start?
Scleroderma usually starts in the skin, where it may lead to changes in skin color, texture, thickness, and even hair loss; joint swelling, stiffness and pain; and/or calcinosis, which is the abnormal deposits of calcium salts in the skin and clump organs.
In some cases, however, the disease can manifest in the lungs and kidneys. Generally speaking, scleroderma results from the body’s immune system attacking the body’s own tissues, which leads to inflammation and thickening of certain body parts.
Depending upon the type of scleroderma and its severity, the disease may affect specific areas of the body and organs, as well as particular joints and muscles. Scleroderma can sometimes be limited to the skin, making it a localized form of the disease, or it can affect many different systems of the body, making it a systemic form of the disease.
In some cases, scleroderma can result in serious, life-threatening complications.
Which of the following are clinical manifestations of patient with systemic sclerosis?
Systemic sclerosis is a chronic, progressive, multi-system connective tissue disorder which can affect many areas of the body, leading to a variety of symptoms and clinical manifestations.
Common clinical manifestations of systemic sclerosis include digital ulcers on the fingertips, arthritis, fatigue, Raynaud’s phenomenon (cold, discoloration of the fingers, toes, and lips), difficulty swallowing and dysphagia, reduced elasticity of the skin with fibrotic plaques, and telangiectasia (dilation of blood vessels on the skin, face, and hands).
Other common clinical manifestations include increased thickness of the skin with shin spots, pulmonary disease (scarring of the lung), heart problems, interstitial lung disease, esophageal dysmotility, sclerodactyly (thickening and tightening of skin on the fingers and toes), muscle weakness, and irritable bowel syndrome.
As the disease progresses, many patients can experience pulmonary hypertension, osteoporosis, infertility, and an elevated risk of certain cancers. Clinical manifestations can vary significantly between individuals and may or may not be present.
Additionally, while the clinical manifestations may be initially mild, they can worsen over time as the disease progresses.
How does systemic scleroderma start?
Systemic scleroderma, or systemic sclerosis, is an autoimmune disorder that affects the skin, blood vessels, and internal organs of the body. It is characterized by the buildup of excess collagen in the skin and other organs, which can cause significant damage and discomfort.
While the exact cause of systemic scleroderma is not known, it is believed that a combination of genetic, environmental, and immunological factors contribute to the development of the disorder.
The onset of systemic scleroderma typically occurs in middle age, although it can affect children and young adults. Symptoms tend to start as small patches of hard, thickened skin that may be discolored.
Over time, these patches can become widespread and cause the skin of the fingers, hands, feet, and face to become stiffer and thickened. Other signs and symptoms can develop throughout the body, such as shortness of breath, difficulty swallowing, and heartburn.
In more severe cases, systemic scleroderma can lead to more serious health concerns, including progressive organ damage and failure. A variety of treatments are available to help manage the symptoms of systemic scleroderma and slow the progression of the disorder.
How do you know if scleroderma is systemic?
Scleroderma is a chronic connective tissue disorder that is characterized by the hardening and thickening of the skin. It is classified as either localized or systemic. Systemic scleroderma affects other organs in the body besides the skin and requires more aggressive treatment to manage the condition.
The diagnosis of systemic scleroderma is based on recognition of systemic features of the disease. In order to diagnose systemic scleroderma, the physician may review the patient’s medical history, symptoms, and the results of blood tests and scans.
Abnormal lab values can reveal the presence of antinuclear antibodies, which is a marker of systemic scleroderma. Imaging tests, such as chest x-rays or MRIs, may reveal signs of thickening in the lungs or heart, weakening of the pulmonary arteries, or stretching of the aorta, which are all signs of systemic scleroderma.
Additionally, a doctor may conduct a physical examination and look for telangiectasias (tiny red spots beneath the skin), areas of calcinosis (calcium deposits beneath the skin), and Raynaud’s phenomenon (feeling of cold or numbness of the fingers in response to cold).
A definitive diagnosis of systemic scleroderma requires several individual tests that help to identify the underlying cause of symptoms. However, if any of these tests come back positive, it could indicate systemic scleroderma and further evaluation may be necessary in order to confirm the diagnosis.
How quickly does scleroderma develop?
The speed of progression of scleroderma varies widely and depends on the type of scleroderma a person is experiencing. In general, it can be a gradual process with mild symptoms that progress slowly over several years, or it can progress quite rapidly over months in some cases.
For example, localized scleroderma, which is confined to the skin, can take months to years to develop, while generalized scleroderma, which can involve the skin, lungs, heart, and other organs, can progress more quickly over a few months or a year.
Symptoms can also remain stable for long periods of time and then suddenly worsen. With the systemic form of scleroderma, organ involvement can cause life-threatening complications and symptoms if not monitored and managed appropriately.
It is important for people with scleroderma to be monitored regularly and to receive proper medical care from a multidisciplinary team to properly track the disease and its progression.
How do you rule out scleroderma?
Ruling out scleroderma requires several steps. First, a physician will typically take a detailed medical history and perform a physical exam. During the physical exam, the doctor will carefully examine the skin for signs of scleroderma, such as thickened skin or discoloration.
Blood tests may also be done to check for blood markers associated with scleroderma. Imaging tests such as X-rays, CT scans, and MRIs can be used to help diagnose the condition and look at affected organs in more detail.
A biopsy of affected tissue can also be used to obtain more information. Finally, if the physician cannot make a definitive diagnosis, they may refer the patient to a specialist in rheumatology for further testing.
Can you stop the progression of scleroderma?
Unfortunately, there is no way to stop or cure scleroderma at this time. Treatment for scleroderma focuses on managing symptoms and preventing further damage. But the effectiveness of these medications can vary.
The most commonly prescribed medications are disease-modifying antirheumatic drugs (DMARDs), nonsteroidal anti-inflammatory drugs (NSAIDs), immunosuppressants, immunomodulators, supplements, and antimalarials.
Physical and occupational therapy may also be helpful in managing pain, increasing joint range of motion and flexibility, improving muscle strength and restoring movement of affected areas. It is important to note that the effectiveness of these treatments may vary person to person.
Does scleroderma come on suddenly?
No, scleroderma usually does not come on suddenly. It typically starts with slow and mild symptoms that gradually get worse over time. Symptoms can range from fatigue and joint pain, to Raynaud’s phenomenon and clubbing of the fingers.
As the disease advances, the skin might become thick and tight, leading to loss of mobility. This can also lead to digestive problems as the thickening of the esophagus makes it hard to swallow. Eventually, the skin can form calcium deposits and the connecting tissues can become inflamed, stiff, and full of scar tissue.
These symptoms can take years to develop and can vary greatly in severity. For those living with scleroderma, it is important to monitor the progression of symptoms to ensure that the disease is being managed properly.
Which is the major criterion required for diagnostic of systemic scleroderma?
The major criterion required for the diagnostic of systemic scleroderma, also known as systemic sclerosis is the presence of skin thickening in two or more regions of the body for more than 6 months, in addition to at least one of the following: microvascular changes, histological evidence of scleroderma on a skin biopsy, or the presence of antinuclear antibodies (ANA).
The most important clinical indicators of systemic sclerosis are Raynaud’s phenomenon and digital ulcerations. Raynaud’s phenomenon is a condition caused by an excessive narrowing of the blood vessels in the fingers and toes in response to cold temperatures or stress.
Digital ulcers are sores or blisters on the fingers or toes due to decreased blood flow in those areas. Other symptoms may include joint pain, fatigue, difficulty in swallowing and gastrointestinal issues.
Based on the clinical manifestations, it is important to order additional tests including complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein test (CRP), chest X-ray (CXR), pulmonary function testing, pulse oximetry, echocardiography, and GI endoscopy.
Additionally, the clinician should order tests for antinuclear antibodies (ANA), anti-Scl-70, anti-RNA polymerase, and anti-centromere antibodies. Serum and urinary levels of protein, calcium and phosphorus (to check for renal involvement) should also be obtained.
Lastly, a skin biopsy may be ordered to confirm a diagnosis of systemic scleroderma.
In summation, the criteria required for a diagnosis of systemic scleroderma include skin thickening in two or more regions lasting more than 6 months, as well as at least one of the following: microvascular changes, histological evidence on skin biopsy, or the presence of antinuclear antibodies (ANA).
To confirm a diagnosis, testing, imaging, GI endoscopy, and a skin biopsy may be required.
When a client is diagnosed with systemic sclerosis scleroderma what symptoms is the first to occur?
Systemic sclerosis scleroderma is a chronic, autoimmune disorder that affects the skin, joints, and internal organs. Symptoms of systemic sclerosis scleroderma can vary depending on the severity and reach of the disease.
Common symptoms include tight, thickening skin; joint pain and stiffness; altered finger and toe nails; swelling in the hands and feet; digestive problems such as reflux, bloating, and constipation; shortness of breath; and fatigue.
The first symptom to usually occur in systemic sclerosis scleroderma is generally tight, thickening skin on the hands and face. This is due to extra collagen in the skin, leading to tightness, increased skin tone, and a paper-like texture.
Other symptoms may be experienced depending on the type of systemic sclerosis scleroderma, the tissues that are affected, and the rate of progression of the disease. It is important to consult with a doctor to get an accurate diagnosis and determine the best treatment plan.
