Stevens-Johnson Syndrome (SJS) is an extreme allergic reaction, usually to a drug, and has been associated with the rheumatoid arthritis drug Arava. This autoimmune disease often leads to severe morbidity and death.
What’s the problem?
Stevens-Johnson Syndrome (SJS) is a form of skin disease that can cause rash, skin peeling, and sores on the mucous membranes. SJS is an immune-complex–mediated hypersensitivity disorder that may be caused by many drugs, viral infections, and malignancies. Often, the drugs causing the onset of Stevens-Johnson Syndrome, such as Arava, did not have warnings placed on their labels until recently. Patients unknowingly took these drugs and many developed the potentially fatal Stevens-Johnson Syndrome.
SJS is characterized by inflammation of the mucous membranes of the mouth, throat, eyes, genital tract, and intestinal tract. Ulcers inside the mouth are the most common, causing inflammation and irritation to the tongue, gums, and lips and can extend into the throat. Affected individuals may also have skin lesions, blisters and bleeding in the lips, eyes, mouth, nasal passage and genital areas.
Stevens-Johnson Syndrome Symptoms
As SJS evolves, the skin literally sloughs off in sheets. Patients are typically treated in a hospital’s burn unit. If the skin lesions become infected, or the patient develops lesions in the lungs, it can cause death. Other symptoms and complications of SJS include:
- permanent blindness
- dry-eye syndrome
- lung damage
- chronic obstructive pulmonary disease (COPD)
- permanent loss of nail beds
- scarring of the esophagus and other mucous membranes
- chronic fatigue syndrome
While a small percentage of Stevens-Johnson Syndrome cases have been linked to bacterial infections and skin graft problems, the majority are caused by adverse drug reactions (ADRs). In an ADR, a drug such as Arava acts as an allergen and causes the immune system to respond by producing antibodies and other disease fighting cells. These antibodies actually injure the surrounding cells and cause the physical symptoms of an allergic reaction.
Stevens-Johnson Syndrome is a life-threatening condition, yet it is often misdiagnosed and under-reported by many physicians. Emergency facilities and doctors often do not recognize the symptoms, causing a delay in treatment and increasing the harmful effects of the reaction.
Complications from SJS can include blindness, dry-eye syndrome, esophagus and lung damage, chronic obstructive pulmonary disease (COPD), asthma, arthritis, and chronic fatigue syndrome. In some SJS patients, their pores scar shut, causing them to retain heat. If left untreated, SJS can be fatal.