Risk factors for Pulmonary Arterial Hypertension in Systemic Sclerosis
• lcSSc (especially longstanding disease).
• Duration of Raynaud’s (longer duration = higher risk).
• ACA.
• Isolated nucleolar-pattern ANA.
• Extensive telangiectasia.
• DLco <60% in the absence of extensive ILD or other cause of low DLco (e.g., emphysema, as described in some SSc patients with combined pulmonary fibrosis & emphysema).
• FVC%/DLco% ratio > 1.6 (meaning a disproportionately low DLco).
