UCN2 and pulmonary arterial hypertension: Positivity of anti-SRP antibody is specific in the IMNM subtype of IIM, featuring severe limbic skeletal muscle involvement and frequent respiratory muscle involvement secondary to inflammatory infiltration, reticulum stress-induced autophagy pathway as well as necroptosis.36, 37, 38 Extramuscular involvement in SRP-IMNM mostly focused on interstitial lung disease, myocardial injury, etc.37Previously few cases of SRP-IMNM patient reported complication of PAH.39