P113 A challenging case of mixed connective tissue disease with pulmonary hypertension
Abstract
Mixed connective tissue disease (MCTD), first described by Sharp in 1972, is a systemic autoimmune disorder characterised by features of at least two overlapping connective tissue diseases. One of its serious complications is pulmonary arterial hypertension (PAH), which affects approximately 6.7% to 17.8% of patients. Although the development of vasodilator therapies has improved outcomes in connective tissue disease-associated PAH (CTD-PAH), survival remains poorer compared to idiopathic PAH. Recently, sotatercept has been approved as a novel treatment. As the first drug targeting the molecular mechanisms of vascular remodelling, it offers a promising advance for managing this challenging condition.
A 28-year-old woman of Chinese origin was initially admitted in spring 2021 with fever, confusion, cervical lymphadenopathy and a maculopapular rash, along with a three-year history of Raynaud’s phenomenon. She also reported intermittent breathlessness and palpitations without progressive dyspnoea. Examination revealed cervical lymphadenopathy and reduced bilateral breath sounds due to pleural effusions. Initial treatment was for presumed tuberculosis, but she improved markedly within 48 hours on antibiotics and corticosteroids. Connective tissue disease screening revealed a positive ANA (6.5, speckled pattern) and moderately positive anti-U1 RNP antibodies, confirming a diagnosis of mixed connective tissue disease. Pulmonary function tests showed normal FVC but reduced TLCO…
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