Table I.

Domain/Organ System		Tools/procedures
I	Cardiopulmonary	Echocardiography with Doppler Six minute walk test Right heart catheterisation Laboratory markers (BNP, pro-BNP) Measures of dyspnea Electrocardiogram Blood pressure Treatment
II	Pulmonary	Spirometry and diffusing capacity Chest radiograph High-resolution computed tomography (HRCT) of lungs Treatment
III	Gastrointestinal	Weight and Body mass index (BMI) Laboratory markers (serum albumin, etc.) Test for gastroparesis Test for esophageal dysmotility Test for malabsorption Treatment
IV	Renal	Blood pressure Laboratory markers (serum creatinine, creatinine clearance, urine protein, etc.) Treatment
V	Musculoskeletal	Assessment of muscle weakness on physical exam Measure of joint involvement (e.g. number of tender joints) Laboratory markers (serum creatine phosphokinase) Treatment
VI	Cutaneous	Physical exam to determine skin involvement Treatment
VII	Health-Related Quality of Life	Measure of function
VIII	Serologies	Test for anti-topoisomerase I, anti centromere and anti-RNA polymerase III antibodies
IX	Prevention and Drug Monitoring