Researchers from Japan showed that immunosuppressive therapy can be successful for patients with pulmonary arterial hypertension (PAH) due to Takayasu arteritis, according to a new study published in Internal Medicine.

They concluded that, when the disease is diagnosed in its early stage, the treatment can be successful without the need for endovascular or surgical procedures.

Takuya Suda and colleagues from the Department of Rheumatology at Kanazawa University Graduate School of Medicine in Japan reported cases of 2 patients with PAH due to Takayasu arteritis who were treated with immunosuppressive therapy. They also conducted a literature review of 42 cases of pulmonary hypertension due to Takayasu arteritis with pulmonary artery involvement.

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They found that patients treated with immunosuppressive therapy within 12 months after the onset of the disease had a higher erythrocyte sedimentation rate and better outcomes without invasive therapy, compared to those treated later.

Pulmonary hypertension due to Takayasu arteritis “may be successfully treated with immunosuppressive therapy without invasive therapy when diagnosed early with high disease activity,” they wrote.

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The reported cases were of 2 women aged 27 and 49 years who were both treated with corticosteroids for 2 weeks. The first patient was also treated with methotrexate and tacrolimus after tapering off of corticosteroids followed by the addition of tocilizumab. The second was treated with cyclosporine and mizoribine after corticosteroids. In the first case, remission was achieved for 5 years while in the second it was achieved for 15 years.

The researchers said the impact of early immunosuppressive therapy on the outcome of patients with PAH due to Takayasu arteritis is not clear due to the rarity of the condition. They added that it remains to be determined whether or not invasive therapy can be avoided by using immunosuppressive therapy.

Takayasu arteritis is a chronic inflammatory disease affecting the aorta and its major branches. In rare cases, it can lead to PAH.

Internal Medicine is a publication of the Japanese Society of Internal Medicine.


Suda T, Zoshima T, Ito K, Mizushima I, Kawano M. Successful early immunosuppressive therapy for pulmonary arterial hypertension due to Takayasu arteritis: two case reports and a review of similar case reports in the English literature. Intern Med. Published online November 13, 2021. doi:10.2169/internalmedicine.8095-21