Oh, dear, I have SJS, what will happen now?

The prognosis of Steven Johnson Syndrome (SJS) varies depending on the amount and severity of the lesions. Most lesions typically heal within a week or two, unless a secondary infection occurs, however, many people recover without additional consequence. The prognosis of someone with SJS is correlated with mortality. Mortality is linked with the amount of skin sloughing. If a person’s body surface area (BSA) sloughing is less than 10%, their prognosis is 95-99%, with a mortality rate of 1-5%. On the other hand, when a person’s BSA is 30% the mortality rate rises to 25-35% to as high as 50%. During the course of the sloughing the most pertinent risk factor are the bacteremia and sepsis plays a major role in mortality.
To assess your prognosis, your doctor will use a severity-of-illness score for toxic epidermal necrolysis (SCORTEN). The SCORTEN calculates the risk for death in both diseases based on these variables:

  • Age >40 years
  • Malignancy (i.e. tumors)
  • Heart rate >120 (tachycardia)
  • Initial percentage of epidermal detachment >10%
  • Blood urea nitrogen (BUN) level >10 mmol/L (kidney function)
  • Serum glucose level >14 mmol/L
  • Bicarbonate level < 20 mmol/L (kidney function)

Each of these variable is assigned a value of 1 point, and mortality/prognosis rates are as follows (the percentages are correlated with the chance death due to the disease):

  • 0-1 points, ≥3.2%;
  • 2 points, ≥12.1%;
  • 3 points, ≥35.3%;
  • 4 points, ≥58.3%; and
  • 5 or more points, ≥90%.

In addition to the scoring, other risk factors that will influence your prognosis include persistent neutropenia (defined as neutropenia lasting more than 5 days), hypoalbuminemia (usually <2 g/dL), and persistent azotemia. Neutropenia puts you at risk for infections, low albumin can lead to heart failure, and azotemia signifies kidney failure. Studies have shown that some people can lose very large areas of their skins, while others can form new skin in a matter of days. Hence, it is very difficult to assess prognosis in the early days of the disease. Physiologically, re-epithelialization (regrowth of skin) is usually completed within 3 weeks, but areas with a lot of pressure and mucus may remain eroded and crusted for prolonged periods of time.

After the mortality rate drops due to time or healing or medicine, the survivors of SJS may experience numerous long-term sequelae. Of these possible sequelae, the most disabling may be the eye, known as cicatrization. This is when there are conjunctival erosions that can lead to:

  • Inverted eyelashes,
  • Photophobia,
  • A burning sensation in the eyes,
  • Watery eyes,
  • A siccalike syndrome (autoimmune disease, Sjőgren’s Syndrome, dry mouth, dry eyes), and
  • Corneal and conjunctival neovascularization.

Prognosis of your SJS can also impact your case, possibly leading to a better settlement for your family and loved ones. At Wormington & Bollinger we have dedicated an excellent team of lawyers to fight for your individual needs if you have been affected by either a misdiagnosis or improper care leading to Steven-Johnson Syndrome due to any cause. Compensation for our clients’ pain and suffering is the number motivation for our lawyers and we are here for you if you feel you are a victim of SJS due to indecorous medical practices. Contact us today for a free consultation.

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