MRCP2-4695

MRCP2-4695

A 30-year-old woman with diffuse cutaneous systemic sclerosis presents to the emergency department with worsening shortness of breath over the past three months. She has severe Raynaud’s phenomenon, skin tightness, sclerodactyly, and skin thickening up to her shoulders. Despite having no history of orthopnea, her exercise tolerance has decreased from 500 yards to less than 50 yards. On examination, she has a raised JVP and mild bi-pedal edema. Her blood pressure is 110/70 mmHg, pulse is 109/min, SaO2 is 91% on air, and respiratory rate is 20/min. Muscle strength is normal, and examination of her heart, lungs, and abdomen is unremarkable. Her investigations show a hemoglobin level of 121 g/L (115-165), WBC count of 7.8 ×109/L (4-11), neutrophils at 70% (40-75), platelet count of 270 ×109/L (150-400), ESR of 36 mm/hr (0-20), normal U&E and creatinine, negative D-dimers, a normal CXR, and an ECG with right axis deviation. What is the most likely diagnosis?