Systemic Sclerosis: Causes, Symptoms and Treatment

Systemic sclerosis (SS) is an autoimmune disorder. This suggests it’s a condition where the body immune system assaults the body. Healthy tissue is ruined because the body immune system incorrectly thinks it’s a foreign compound or infection. There are numerous sort of autoimmune conditions that can affect various body systems.

Systemic Sclerosis Definition

SS is identified by changes in the texture and appearance of the skin. This is because of increased collagen production. Collagen is a component of connective tissue.

However the disorder isn’t restricted to skin changes. It can impact your:

  • capillary
  • muscles
  • heart
  • digestive system
  • lungs
  • kidneys.

Functions of systemic sclerosis can appear in other autoimmune conditions. When this happens, it’s called a blended connective disorder.

The disease is typically seen in people 30 to 50 years old, however it can be diagnosed at any age. Women are most likely than men to be diagnosed with this condition. The symptoms and severity of the condition vary from someone to another based on the systems and organs involved.

Systemic sclerosis finger

Systemic sclerosis finger

Systemic sclerosis is likewise called scleroderma, progressive systemic sclerosis, or CREST syndrome. “CREST” represents:

  • calcinosis
  • Raynaud’s phenomenon
  • esophageal dysmotility
  • sclerodactyly
  • telangiectasia.

CREST syndrome is a minimal kind of the disorder.

Symptoms and Signs of Systemic Sclerosis

Scleroderma can be hard to diagnose, particularly early in the course of the disease. The symptoms and physical findings early on, including symptoms of Raynaud’s phenomenon, swelling of the hands, and basic pain, can overlap with the early symptoms of lupus, rheumatoid arthritis, dermatomyositis, and other conditions. A rheumatologist will typically make this diagnosis, but it may take more than one check out for the rheumatologist to be particular.

Capillary

The first symptom of scleroderma is frequently the development of the Raynaud’s phenomenon, which is when the fingers and/or toes change color and become numb when exposed to the cold. Typically the fingers turn white, then bluish or purple, and after that extremely red.

This can end up being sufficiently severe adequate to cause digital ulcer (sores on the fingers) and/or gangrene of the fingers. In addition, patients regularly have dilated capillary at the nail folds, which medical professionals can see with zoom and might help with the diagnosis.

Skin

Almost all patients with Systemic Sclerosis (SSc) have thickening of their skin. This is seen particularly in the hands but can cross the entire body. In minimal cutaneous SSc, skin participation is limited to the face, hands, forearms, lower legs, and feet. In scattered cutaneous SSc, the skin thickening can furthermore include the arms, thighs, and the trunk.

It is possible to have Systemic Sclerosis without skin thickening (sine scleroderma), which is when a patient has the internal organ manifestations without the skin findings, however this is very rare. Skin thickening and tightness can cause contractures of the joints (when an individual can not extend or bend a joint totally) and an inability to open the mouth totally. Other skin findings include the following:

  • Hyper – or hypopigmentation of the skin, when the skin looks darker or lighter than usual.
  • Telangiectasia, which are dilated capillary that show up as red spots on the skin.
  • Calcinosis or hard calcium in the skin, which can be painful.

Lung

Patients with SSc might suffer from shortness of breath, reduced workout capability, or cough. The leading cause of death in patients with SSc is lung disease, which can either be interstitial lung disease with fibrosis (scarring) of the lungs or lung hypertension, which is elevated pressures in the pulmonary artery.

Patients with SSc requirement screening for these conditions on a regular basis. This is performed with pulmonary function screening, echocardiography, and chest radiography or CT scans of the chest. If these screening tests are irregular, recommendation to a pulmonologist or cardiologist for extra screening is often required.

Heart

The heart can also be affected in SSc, and heart-related symptoms may be similar to lung-related symptoms, consisting of shortness of breath or reduced exercise capability. Extra symptoms include chest pain or swelling of the legs. Some patients have cardiomyopathy or weakness of the heart muscle, and others might experience unusual cardiac rhythms.

Kidneys

The kidneys can be associated with scleroderma. An unusual but incredibly essential condition in SSc is Scleroderma Renal Crisis. This happens when a patient with SSc all of a sudden develops really hypertension and possibly kidney failure. Patients might experience severe headaches, stroke, or seizure if this happens, or they may be asymptomatic. Patients with scattered SSc, particularly early in the course of their health problem, need to examine their blood pressure at home regularly to capture any elevation early when treatment with ACE-Inhibitors would have the best impact.

Intestinal Tract

Any part of the intestinal (GI) tract can be involved in SSc, and the symptoms vary depending upon the involvement. Over 90% of patients have GI symptoms of some sort. The most typical issue is reflux, which can seem like heartburn or cause chronic cough. Reflux can also contribute to chronic aspiration, which can then add to lung disease. Some patients may experience nausea, vomiting, diarrhea, constipation, or have a hard time swallowing.

Other patients may experience bloating for numerous factors or problems taking in nutrients. Some experience weight loss. Others can experience bleeding from the stomach due to the fact that of a condition called Gastric Antral Vascular Ectasia (GAVE, also called “watermelon stomach” due to the fact that of the way it appears on endoscopy).

Anemia

Low red cell counts (anemia) can contribute to many symptoms including tiredness, shortness of breath, or dizziness. This can take place since of low iron or vitamin levels, a chronic inflammatory state, or for other reasons.

Muscle

Patients with SSc might experience muscle weakness since of inflammation of the muscles, while others experience pain in their muscles.

Joints

Many patients with SSc experience pain in their joints, which occurs for numerous factors. Often the joints might be swollen because of an autoimmune procedure. Other times, patients have an overlapping condition with both SSc and rheumatoid arthritis. Individuals with scleroderma may also experience joint pain for the same factors people without this condition feel discomfort. For example, overuse injuries, osteoarthritis, and degenerative disc disease may lead to joint pain.

Neurological

Untreated Carpal Tunnel Syndrome

Carpal Tunnel Syndrome

Some patients experience numbness, tingling, and pain from various neuropathies (disorders of the nerve system), including carpal tunnel syndrome and other syndromes too.

Sexual Dysfunction

This can take place in both men and women with SSc for multiple factors.

General Symptoms

In addition to the symptoms above, many patients experience tiredness, problem sleeping, state of mind disorders, including depression and anxiety, weight loss, malaise, and pain.

Since of the multiple methods by which SSc can manifest itself, it is necessary for patients to let their physicians know all their symptoms so they can be dealt with.

Causes of Systemic Sclerosis

Systemic sclerosis is thought about to be an autoimmune disorder.
As an outcome, the body’s own body immune system causes damage to its own cells, rather of combating external organisms like viruses and bacteria. In this condition, the body immune system begins attacking the connective tissue cells within the body.
Sadly, there is little info about what aspects trigger the body immune system to behave in this method. Some of the elements that are thought about to set off the autoimmune response consist of,

  • Inherited propensity.
  • Cytomegalovirus infection.
  • Exposure to chemicals like vinyl chloride, silica or benzene derivative.

Price quotes have actually recommended that the condition impacts about two in 100,000 individuals, with women having a fourfold greater tendency to establish the condition compared with their male counterparts.

Risk Factors for Systemic Sclerosis

Danger aspects that can increase your possibilities of establishing the condition include:

  • being Native American
  • being African-American
  • being female
  • using particular chemotherapy drugs such as Bleomycin
  • being exposed to silica dust and organic solvents.

There’s no known method to avoid SS aside from to decrease risk aspects you can control.

Diagnosis of Systemic Sclerosis

Throughout a physical exam, your doctor can determine skin changes that are symptomatic of SS.

High blood pressure might be triggered by kidney modifications from sclerosis. Your doctor might buy blood tests like antibody screening, rheumatoid factor, and sedimentation rate.

Other diagnostic tests can include:

  • a chest X-ray
  • a urinalysis
  • a CT scan of the lungs
  • skin biopsies.

Treatment for Systemic Sclerosis

Treatment cannot cure the condition, but it can help reduce symptoms and slow disease development. Treatment is generally based on an individual’s symptoms and the have to avoid problems.

Treatment for generalized symptoms might involve:

  • corticosteroids
  • immunosuppressants, such as methotrexate or Cytoxan
  • nonsteroidal anti-inflammatory drugs.

Depending upon your symptoms, treatment can likewise include:

  • blood pressure medication
  • medication to aid breathing
  • physical treatment
  • light therapy, such as ultraviolet A1 phototherapy.

You can make lifestyle modifications to remain healthy with scleroderma, such as preventing smoking cigarettes, continuing to be physically active, and avoiding foods that trigger heartburn.

Home Remedies for Systemic Sclerosis

One should bear in mind that systemic sclerosis cannot be treated and thus the treatment program concentrates on reliving the symptoms, stopping the progression of the disease, spotting and alleviating problems and limiting disability. Here are some natural treatment options for the management of the condition,

  • Regional application of hydrating creams or Aloe Vera gel on the skin along with routine extending exercises can help avoid and restrict cutaneous symptoms.
  • Restricting diet, drinking great deals of fluids, regularizing meal schedules, etc will assist in efficient management of heart burns.
  • One might attempt a host of natural solutions and home based treatments for management of hypertension, hypothyroidism, etc and treat infections immediately.

While home remedies and natural treatment is really useful, it is typically suggested to consult your doctor if the symptoms continue to deteriorate.

What Is the Outlook for People with Systemic Sclerosis?

Treatments for SS have significantly improved in the past Thirty Years. Although there’s still no treatment for SS, there are various treatments that can assist you handle your symptoms. Speak with your doctor if any of your symptoms is getting in the way of your life. They can deal with you to adjust your treatment plan.

You should also ask your doctor to help you discover local support system for SS. Speaking to other individuals who have comparable experiences as you can make it simpler to handle a persistent condition.

Good luck! Have a nice weekend.

 

Updated: 09.01.2017 — 06:04

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