Medical Health Encyclopedia

Prostacyclins (also called Prostaglandins). Prostacyclins open blood vessels and also have anti-blood clotting properties. Specific agents, such as iloprost, appear to reduce levels of connective tissue growth factor, a molecule important in the abnormal proliferation of cells that cause collagen buildup. A 2005 study reported that prostavasin, a prostacyclin analogue, improved circulation to the hands and feet. A number of prostacylins are being used for scleroderma, although none have been approved specifically for the condition. Iloprost has been studied the longest. Other promising prostacylins or similar agents include alprostadil (prostaglandin E1), epoprostenol (Flolan,), and treprostinil (Remodulin).

Endothelin Receptor Antagonists. Bosentan (Tracleer) is an oral agent called an endothelin receptor antagonist. It controls endothelin, a powerful molecule that causes blood vessels to narrow. It improves blood flow and is becoming important for treating patients with scleroderma, especially  for preventing finger ulcers and improving hand function. Although experts initially thought bosentan might help scleroderma patients with pulmonary hypertension, a 2005 study concluded that the drug did not work for such patients. In fact, the researchers found that bosentan may have even worsened their condition.

ACE Inhibitors and Similar Agents for Hypertension and Renal Crises

The most effective approach at this time for preventing renal crises is to institute aggressive anti-hypertension therapy before blood tests indicate serum creatinine levels over 3 mg/dl. (Creatinine is a nitrogen compound that is measured as an indication of kidney function.)

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Angiotensin Converting Enzyme (ACE) Inhibitors. Many medications are available for controlling blood pressure, but angiotensin converting enzyme (ACE) inhibitors appear to be the most effective for scleroderma patients because of their protective actions in the kidney. ACE inhibitors include captopril (Capoten), enalapril (Vasotec), quinapril (Accupril), benazepril, and lisinopril (Prinivil, Zestril). Side effects are uncommon but may include an irritating cough, excessive drops in blood pressure, and allergic reactions. (The drug picotamide can help reduce the frequency of coughs.) One rare but severe side effect, granulocytopenia, has been observed, which is an extreme reduction in white blood cells; this can be minimized with lower dosages. There has been some concern that they may impair lung function, but studies to date have been reassuring.