Does cyclophosphamide work to treat central nervous system lupus (neuropsychiatric lupus)?
One study of moderate quality provides the best evidence we have to answer this question. The study tested 32 people who had central nervous system lupus. The study compared people who took cyclosphosphamide by IV (intravenous or through a vein) to people who took steroids (methylprednisolone by IV). All people took steroid pills (prednisone) at the beginning of the study and the amount was decreased over the study. The study lasted 2 years.
What is central nervous system lupus and how could cyclophosphamide help?
Systemic lupus erythematosus (SLE) or simply 'lupus' is a group of diseases in which the body's immune system attacks the body. In CNS lupus (central nervous system lupus) the body may have attacked and damaged the cells in the brain and spine. This damage may cause a person to have convulsions/seizures, chronic headaches, confusion and psychosis. Drugs such as corticosteroids (prednisone or methylprednisolone) are usually used for lupus to decrease inflammation and control the immune system. Immunosuppressive agents or cytotoxics such as cyclophosphamide (CTX or Cytoxan) may also be used. These drugs can be given by IV (intravenous or through your veins) or as pills. Unfortunately, it has not been clear whether these drugs improve the symptoms of CNS lupus and what the side effects are.
What did the studies show?
More people who took cyclophosphamide improved than people who took methylprednisolone.
- 95 out of 100 people had at least a 20% improvement in symptoms with cyclophosphamide
- 46 out of 100 people had at least a 20% improvement in symptoms with methylprednisolone
Disease activity, seizures, CNS damage also decreased more with cyclophosphamide.
After 6 months of treatment, people who took cyclophosphamide took less prednisone pills than people who took methylprednisolone. And at the end of two years, it seemed that more people who took cyclophosphamide stayed on their treatment than the people who took methylprednisolone.
Were there any side effects?
Side effects, such as infections, high blood sugar and high blood pressure, pancreatitis and death occurred about the same amount in people who took cyclophosphamide or methylprednisolone.
What is the bottom line?
There is "silver" level evidence (www.cochranemsk.org) that cyclophosphamide may improve symptoms of central nervous system (neuropsychiatric) lupus more than methylprednisolone. It also does not appear to increase side effects. The study in this review was small and more studies are needed.