Median (IQR) unless otherwise indicated.

*p indicates significance of Kruskal-Wallis test (for continuous variables) or Fisher's exact test (for categorical variables).

†Of the 26 patients who had previously received immunosuppressant therapy, in 2 patients this was for cancer.

‡86 patients had a sPAP/RVSP value assumed to be normal and thus not measured. If those cases are omitted, only 38 values of sPAP/RVSP are missing (11.7%). Median values are ‘falsely’ high because calculation omits unmeasured (normal) values.

§Renal involvement is defined as renal crisis and/or moderate-to-severe renal impairment.

¶Despite the significant p-value for the Kruskal-Wallis test, post hoc tests reject any between-group differences in the SF36 mental scores.

** Cochin hand function scores were not performed in all centres because of translational issues.

CRP, C reactive protein; DLCO, carbon monoxide diffusing capacity; eGFR, estimated glomerular filtration rate; ESR, erythrocyte sedimentation rate; FACIT, Functional Assessment of Chronic Illness Therapy; FVC, forced vital capacity; GI, gastrointestinal; HAQ-DI, Health Assessment Questionnaire Disability Index; mRSS, modified Rodnan skin score (17 sites); RVSP, right ventricular systolic pressure; SF36, Short-Form 36; sPAP, systolic pulmonary artery pressure.