TY - JOUR T1 - Pulmonary Arterial Hypertension in Systemic Sclerosis: Can We Predict Responders for Successful Therapy? JF - The Journal of Rheumatology JO - J Rheumatol SP - 665 LP - 666 DO - 10.3899/jrheum.081322 VL - 36 IS - 4 AU - ARTHUR A. SASAHARA Y1 - 2009/04/01 UR - http://www.jrheum.org/content/36/4/665.abstract N2 - Pulmonary artery hypertension (PAH) in patients with connective tissue disease (CTD), especially systemic sclerosis (SSc), is a devastating complication that is currently the most common cause of death in these patients1. In 2003, the World Health Organization sponsored a meeting of PAH specialists to update the criteria and nomenclature for defining its different forms2. The PAH associated with SSc would now fall into the category of Group 1.3.1, which is the type associated with collagen vascular diseases, hereafter referred to as APAH. Previously, this entity was called secondary pulmonary artery hypertension. This category also includes PAH associated with congenital heart disease, portal hypertension, HIV infection, and drugs and toxins, among others (thyroid disorders, hereditary hemorrhagic telangiectasis, etc.).Patients with SSc have an incidence of APAH ranging from 10% to 35%, but those with the CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) have a much higher incidence (50%–65%)3. Patients with mixed CTD also have a high incidence of APAH (23%–53%), while those with systemic lupus erythematosus have a much lower incidence (0.5%–14%). APAH is uncommon in … Address reprint requests to Dr. Sasahara, E-mail: aasasahara{at}comcast.net ER -