Background/Purpose: Buerger disease or tromboangitis obliterans (TAO) is a distinct form of systemic vasculitis of unknown etiology though strongly linked to cigarette smoking. It affects the small and medium-sized arteries and veins in the extremities of the limbs, frequently requiring amputation. Tobacco withdrawal is the only known effective treatment. Endothelial dysfunction appears to be of relevance in this condition and a report has even found that high serum levels of endothelin correlate with the presence of necrosis.

This prelimary study assessed the effectiveness and safety of a sequential treatment with intravenous prostaglandins followed by bosentan in patients with severe TAO

Methods: A series of patients with TAO and severe ischemic ulcer or gangrene were treated with intravenous  alprostadil (PGE-1) for 21 days, followed by bosentan p.o at dose of 62.5 mg twice daily during the first month, which was thereafter up-titrated to 125 mg twice daily. Bosentan was administered on a compassionate-use basis. The study endpoints were clinical improvement of the pain and trophic lesions and the need of amputation.

Results: To date, 4 patients have been included, all of them fulfilling the diagnostic criteria proposed by De Olin (Current Opin Reumatol 2006;18:18-24). Two patients were male and 2 women, with a mean age of 45 years. All patients were current smokers and withdrawn tobacco during follow-up as well as received antiaggregants.

In all cases a complete healing was achieded within the first 4 months after starting the sequential treatment, with disappearance of pain and complete healing of the ischemic lesions in the patient’s toes. None of the patients required amputation. Bosentan could be stopped after a mean of 12 months (SD: 4.22, range 10-14), without relapses. After discontinuation of bosentan, patients were followed for a median of 21 months (4.54; 14-24) and none developed new ischemic lesions.

In all patients bosentan was well tolerated, without any observed adverse reaction.

Conclusion:  This preliminary data suggest that a sequential treatment with intravenous prostaglandins followed by bosentan may be considered a therapeutic option for patients with TAO and severe ischemic lesions. Larger studies are required to confirm these results.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/effectiveness-of-a-sequential-treatment-with-intravenous-prostaglandins-followed-by-bosentan-in-patients-with-buerger-disease-and-severe-ischemic-lesions-a-case-series/