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Treatment of CTD-PAH includes non-specific therapies including diuretics, oxygen, anticoagulation and digoxin, and specific drug therapies such as calcium channel blockers, PA, endothelin receptor antagonists (ERA) and phosphodiesterase type-5 inhibitors (PDE5-i) in selected patients. When compared to Group 4 PAH, the use of anticoagulant in idiopathic is associated with a higher bleeding event rate (19 vs 5.4 vs 2.4 event per 100
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2 Immune checkpoint inhibitors and pathophysiology of irAE
Activation of T-cells requires costimulation signals from major histocompatibility complex (MHC) to T-cell receptor (TCR) and from CD80/86 to CD28 between T-cells and antigen presenting cells (APCs). CTLA-4 is normally expressed on T-cell as a competitor of CD28 for CD80/86. It blocks the costimulation signal leading to inhibitory response to
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