Treatment Options Are Limited for Patients with Progressive Systemic Sclerosis

Unfortunately, no definitive treatment or disease-modifying therapies exist for progressive systemic sclerosis (PSS). The most important pharmacologic advance in treating patients with this disease is use of angiotensin-converting–enzyme (ACE) inhibitors, which profoundly affect renal survival: Before ACE inhibitors were introduced, PSS patients with renal crisis often underwent bilateral nephrectomy. Skin and lung manifestations of PSS are managed with cyclophosphamide, methotrexate, mycophenolate mofetil, or other agents. Some patients achieve modest improvement with these regimens, but none are strikingly beneficial.

In a retrospective case-control study, investigators examined the effectiveness of rituximab in 63 PSS patients who had bee…