Pulmonary hypertension in Takayasu arteritis

Area of Science:

• Cardiology
• Rheumatology
• Pulmonology

Background:

• Takayasu arteritis (TA) is a rare, chronic inflammatory disease affecting large arteries, primarily the aorta and its branches.
• Pulmonary hypertension (PH) is a potential complication of TA, but its frequency and causes in this population require further investigation.

Purpose of the Study:

• To determine the prevalence of pulmonary hypertension (PH) in patients diagnosed with Takayasu arteritis (TA).
• To identify and categorize the underlying causes of PH in TA patients.

Main Methods:

• Sixty-four TA patients underwent transthoracic echocardiography (TTE) to estimate pulmonary arterial pressure.
• Pulmonary hypertension (PH) was defined by echocardiographic systolic PAP ≥40 mmHg or right heart catheterization mean PAP ≥25 mmHg.
• Clinical, imaging, and laboratory data were retrospectively collected from patient hospital records.

Main Results:

• Pulmonary hypertension (PH) was diagnosed in seven (10.9%) of the 64 TA patients.
• Identified causes included left-sided heart disease (Group 2 PH), pulmonary arterial involvement (PAI, Group 4 PH), and atrial septal defect (Group 1 PH).
• Patients with PH showed a higher incidence of treatment with cyclophosphamide and/or biologics (P=0.015).

Conclusions:

• Pulmonary hypertension (PH) is a significant comorbidity in Takayasu arteritis (TA) patients.
• Comprehensive evaluation for all potential PH etiologies is essential in TA management.
• Severe or treatment-resistant TA may predispose patients to PH, and PAH-specific agents may benefit Group 4 PH cases.