Why is the prevalence of pulmonary hypertension higher in patients with mixed connective tissue disease?

Mixed connective tissue disease (MCTD) is an autoimmune disease characterized by high serum anti-U1 ribonucleoprotein (U1 RNP) antibody titers, and with systemic lupus erythematosus (SLE), systemic sclerosis (SSc) ) and idiopathic inflammatory myopathy (IIM) with overlapping clinical features)

research method:

Fifty patients with newly diagnosed and untreated MCTD participated in this multicenter, prospective, and observational study

Research result:

All MCTD patients had Raynaud's phenomenon, positive anti-U1 ribonucleoprotein antibody, and 22.

Furthermore, when we divided MCTD patients into two groups based on the presence or absence of an NVC scleroderma pattern, we found that patients with an NVC scleroderma pattern had a higher prevalence of PAH

Analysis conclusion:

MCTD differs from SLE, SSc, and IIM in the prevalence and responsiveness of NVC scleroderma patterns to immunosuppressive therapy

Clinical Implications:

The incidence of nailfold microvascular abnormalities is lower in mixed connective tissue disease (MCTD) than in SSc

references:

Todoroki Y, Kubo S, Nakano K, Miyazaki Y, Ueno M, Satoh-Kanda Y, Kanda R, Miyagawa I, Hanami K, Nakatsuka K, Saito K, Nakayamada S, Tanaka Y.