Systemic Sclerosis (SS) is a chronic but rare autoimmune disease in which the immune system attacks the body. Also called scleroderma or progressive systemic sclerosis, the condition is characterized by the hardening and tightening of the skin and connective tissues. The normal and healthy tissues get destroyed (because the immune system mistakenly thinks it’s a foreign substance or infection) and replace it with dense, thick fibrous tissue. Generally, the immune system helps defend the body against diseases and infections. However, in patients with SS, the immune system triggers other cells to produce too much collagen (a protein). This extra collagen gets deposited in the skin and organs, which causes hardening and thickening (similar to the scarring process). Even though SS most commonly affects the skin, it can also affect other parts of the body including the gastrointestinal tract, lungs, kidneys, heart, blood vessels, muscles and joints. There are different forms of scleroderma and the condition in its most severe forms can be life-threatening. If left untreated, it can lead to mild or even severe complications that can affect the teeth, digestive system, fingertips, lungs, kidneys, heart and sexual function. For correct clinical documentation of this autoimmune disorder, physicians can choose to outsource to medical billing companies.

As per reports from Clevelandclinic.org (2019 statistics), approximately 250 persons per million American adults are affected by scleroderma. It usually develops between the ages of 35 and 55 and is expected to be four times more common in women than in men. It is estimated that about 1 in 3 people with this condition develop severe symptoms of the disease.

Causes and Types of Systemic Sclerosis (SS)

As mentioned above, SS results from an overproduction and accumulation of collagen in body tissues. However, physicians are unsure about the exact causes or specific factors that cause the body to produce too much collagen. In most cases, the condition is caused by a combination of factors, including immune system problems, genetics and environmental triggers. Scleroderma comprises two major forms – localized and systemic.

  • Localized Scleroderma – Regarded as the most common form, the condition tends to only affect a person’s skin, usually in just a few places.
  • Systemic Scleroderma – This can be broken down into two main types – diffuse and limited. Diffuse form of scleroderma affects many parts of the body (like many internal organs) and hinder digestive and respiratory functions, and cause kidney failure. In case of limited scleroderma (also known as CREST syndrome), the skin thickening is restricted to the fingers, hands and forearms, and also sometimes the feet and legs.

Signs and Symptoms of SS

Scleroderma’s signs and symptoms vary depending on the type of the condition and which parts of your body get affected. In the early stages, SS may only affect the skin. The skin may begin to thicken and shiny areas develop around the mouth, nose, fingers and other bony areas. As the condition progresses, patients may begin to experience limited movement of the affected areas. Early symptoms include changes in the fingers and hands, such as stiffness, tightness, and puffiness because of sensitivity to cold or emotional stress. There may be swelling in the hands and feet, especially in the morning. Other associated symptoms include –

  • Small, dilated blood vessels under the skin’s surface
  • Ulcerations on the fingertips and toes
  • Tight, mask-like facial skin
  • Shortness of breath
  • Red spots on the skin (telangectasias)
  • Persistent dry cough
  • Pain and stiffness in the joints
  • Joint contractures (rigidity)
  • Hair loss
  • Excessive calcium deposition in the skin (calcinosis)
  • Esophageal reflux
  • Difficulty swallowing
  • Diarrhea and constipation
  • Calcium deposits or white lumps under the skin
  • Abdominal bloating after meals

Diagnosis and Treatment of Scleroderma

As scleroderma tends to affect different areas of the body, it is rather difficult to diagnose the condition. However, the diagnosis may begin with a physical exam wherein the physician may study and identify skin changes that are symptomatic of SS. High blood pressure may be caused by kidney changes from sclerosis. Therefore, physicians may request blood tests like antibody testing, rheumatoid factor, and sedimentation rate – to check for elevated levels of certain antibodies produced by the immune system. In addition, other imaging or organ-function tests like – chest X-rays, urinalysis, CT scan of the lungs and skin biopsies (removing a small sample of the affected skin for lab examination) may be performed to determine whether the digestive system, heart or lungs are affected.

Even though treatment modalities cannot cure the condition fully, it can help reduce symptoms and slow disease progression. Typically, treatment modalities are based on a person’s symptoms and aim to prevent complications. There is no medication that can cure or stop the overproduction of collagen that is the classic feature of scleroderma. However, a variety of medications can help control scleroderma symptoms and prevent complications. Common medications include – corticosteroids, non-steroidal anti-inflammatory drugs, immune-suppressants (such as methotrexate or Cytoxan), blood pressure medications and nitroglycerin ointments to treat localized areas of tightening of the skin. In addition, therapies like physical therapy or occupational therapy can help better manage pain and improve mobility and strength. In certain cases, surgical options will be used as a last resort and these include amputation and lung transplants.

ICD-10 Codes for Systemic sclerosis (SS)

The diagnosis and treatment procedures for this condition have to be carefully documented using the correct medical codes. Medical coding services provided by reliable medical billing companies can help physicians use the correct codes for their billing purposes. ICD-10-CM codes for SS include –

  • M34 – Systemic sclerosis [scleroderma]
  • M34.0 – Progressive systemic sclerosis
  • M34.1 – CR (E) ST syndrome
  • M34.2 – Systemic sclerosis induced by drug and chemical
  • M34.8 – Other forms of systemic sclerosis
    • M34.81 – Systemic sclerosis with lung involvement
    • M34.82 – Systemic sclerosis with myopathy
    • M34.83 – Systemic sclerosis with polyneuropathy
    • M34.89 – Other systemic sclerosis
  • M34.9 – Systemic sclerosis, unspecified

Even though there is no specific cure for SS (as mentioned above), there are different treatments that can help people better manage the symptoms. If any of these symptoms are getting in the way of one’s daily life, it is important to contact the treating physicians immediately as they help adjust the treatment plan accordingly. Incorporating serious lifestyle changes like – avoiding smoking cigarettes, remaining physically active, and avoiding foods that trigger heartburn can help people stay healthy even with scleroderma.