To make some examples, while ACA positivity is mainly associated with consistent vascular injury, a limited cutaneous involvement, pulmonary arterial hypertension without lung fibrosis, subcutaneous calcinosis and long-standing RP [48,49], ATA+ patients display more frequently diffuse cutaneous involvement, interstitial lung disease (ILD), gastrointestinal involvement, heart fibrosis, digital ulcers and hand disabilities due to metacarpophalangeal (MCF) and proximal interphalangeal (IFP) joints flexion contractures [50,51]. Here, ATM is linked to pulmonary arterial hypertension.