Coronary disease indicative of autoimmune vasculopathy in a patient treated with two coronary artery bypass grafts and multiple percutaneous transluminal coronary angioplasties: a clinical case

Learning points.

• The management of coronary disease in the context of autoimmune vasculopathy is intricate;

• Prompt recognition of the underlying condition and initiation of appropriate immunosuppressive therapy played a pivotal role in achieving clinical improvement;

• Anti-inflammatory therapy has been shown to be particularly effective in stabilizing coronary artery disease, but has not stopped the evolution of atherosclerosis in the lower limbs;

• The complexity of recurrent In-Stent Restenosis and the need for multiple interventions highlight the importance of a multidisciplinary approach in managing such cases;

• Continuous monitoring and adherence to immunosuppressive therapy are crucial for long-term outcomes in patients with autoimmune vasculopathy.

Introduction

Takayasu arteritis (TAK) is a rare, chronic immune-mediated vasculitis that affects large vessels, predominantly the aorta and its major branches. The clinical presentation of TAK can be misleading as it has non-specific symptoms, often resulting in delayed diagnosis. In particular, coronary artery involvement is rare in TAK. However, it contributes to significant morbidity and mortality.¹ The current case is unique due to extensive coronary involvement and repeated episodes of in-stent restenosis, which required multiple revascularizations before achieving the TAK diagnosis. A previous study has shown that TAK primarily affects young women, with coronary lesions usually located in the proximal segments or ostia, complicating timely identification and management.²

Summary figure

Case presentation

A 58-year-old female patient who was a former smoker (two or three cigarettes per day) sought a second opinion after experiencing recurrent episodes of unstable angina since 2008. The first coronary angiography, performed at the age of 46 years, revealed a subcritical occlusion of the left coronary ostium and a critical occlusion in the mid-segment of the left main coronary artery (LMCA), distal to the first diagonal branch, which also exhibited critical stenosis at its origin. The patient underwent percutaneous transluminal coronary angioplasty (PTCA) with stent placement for the LMCA stenosis, plain old balloon angioplasty of the first diagonal branch, and final treatment with kissing balloon inflation. In 2009, just a few months after treatment, the patient had recurrent episodes of unstable angina. A repeat coronary angiography revealed in-stent restenosis. Left internal mammary artery-to-LMCA bypass was performed. In 2011, she had another episode of angina recurrence. Thus, she underwent a second coronary artery bypass graft, in which the right internal mammary artery was anastomosed to the left anterior descending artery.

Subsequent coronary angiographies showed that the two bypass grafts were occluded.

From 2011 to 2015, the patient underwent 30 PTCA procedures due to recurring restenosis.

In July 2015, she underwent molecular analysis of the rs1333040 polymorphism (9p21) as part of cardiovascular risk profiling. The analysis of the locus revealed the 9p21g.22083404 T/T genotypic configuration.

In November 2015, new episodes of angina occurred, which required another PTCA with drug-eluting stents on the proximal LMCA (stent-in-stent) and plain old balloon angioplasty of the common trunk of the LMCA (Figure 1, panels A and B). Two weeks after these percutaneous revascularizations, the patient was evaluated for the first time.

Figure 1.

Panels A and B showing images of percutaneous transluminal coronary angioplasty performed in 2015 with drug-eluting stents on the proximal left main coronary artery (stent-in-stent) and plain old balloon angioplasty, respectively.

Considering the absence of traditional risk factors and the patient’s history of recurrent coronary occlusions, coronary vasculitis was suspected. Arterial Doppler ultrasonography showed narrowing at the origins of the common iliac arteries, with 60% stenosis of the left iliac artery and a non-specific morphological appearance. Clinical evaluation revealed normal bilateral arterial blood pressures, no abnormal bruits, and a normotympanic and treatable abdomen with normal peristalsis. The superficial lymphatic system was intact. Lower extremity oedema, active arthritis, or enthesitis was not observed. Electrocardiogram showed sinus rhythm (63 beats per minute), signs of left atrial enlargement, incomplete right bundle branch block, and negative T wave from V1 to V3 derivations.

Echocardiography revealed a mildly reduced left ventricular ejection fraction (50%), elevated inflammatory marker levels (erythrocyte sedimentation rate: 22 mm/h and C-reactive protein level: 15 mg/dL), and high rheumatoid factor levels. The patient’s thyroid function was normal.

Steroid therapy (prednisone at a dose of 1 mg/kg/day) was recommended. In September 2016, the patient experienced episodes of angina. Coronary angiography showed that the latest procedures had good outcomes. In 2016, the full-body positron emission tomography scan was performed while the patient was receiving corticosteroid therapy. This diagnostic examination did not obtain any pathological findings, except for a modest thyroid goiter (Figure 2; Figure 3, panels A and B).

Figure 2.

Full-body positron emission tomography scan conducted in 2016.

Figure 3.

Full-body positron emission tomography scan carried out in 2016.

A multidisciplinary team of specialists suspected Takayasu arteritis based on the clinical findings. The clinical criteria used to differentiate Takayasu arteritis from aggressive atherosclerotic diseases were as follows: age at diagnosis younger than 60 years, female sex, number of affected arterial territories, abdominal aorta involvement, a high erythrocyte sedimentation rate, and the absence of cardiovascular risk factors, except for light tobacco use (two or three cigarettes per day).

Immunosuppressive therapy with azathioprine (50 mg twice a day) was initiated, and the steroid dose was tapered. The patient’s condition improved, with resolution of angina and normalization of inflammatory marker levels (erythrocyte sedimentation rate and C-reactive protein level).

The patient’s treatment included the following: aspirin 100 mg, ticagrelor 60 mg twice a day, beta-blocker, nitrates, ranolazine, ACE inhibitor, ivabradine 5 mg twice a day, statin, and azathioprine 75 mg.

The diagnosis of autoimmune vasculopathy was based on the disease’s clinical course and favourable response to immunosuppressive therapy. Although there were no signs of vasculitis on imaging examinations, the systemic symptoms and coronary history were consistent with TAK. Vascular imaging, recognized as a diagnostic criterion instead of biopsy, revealed no signs of active vasculitis, and this finding was likely attributed to ongoing corticosteroid therapy. The patient continually received treatment with dual antiplatelet therapy and azathioprine (50 mg twice a day).

During the coronavirus disease 2019 pandemic, the patient missed scheduled imaging studies. Since 2021, she has experienced claudication symptoms. Computed tomography angiography showed moderate (50%) stenosis of the common iliac arteries at their origins (Figures 4; Figure 5, panels A and B). However, revascularization was not indicated.

Figure 4.

Abdominal computed tomography angiography performed in 2021.

Figure 5.

Abdominal computed tomography angiography conducted in 2021.

At her last follow-up in September 2025, no relapse of TAK was observed. Transthoracic echocardiography showed a stable left ventricular ejection fraction. Meanwhile, computed tomography angiography revealed that stenosis of the common iliac arteritis did not change. A full-body positron emission tomography scan has been recommended for the next follow-up to guide the management of the anti-inflammatory therapy.

Discussion

Takayasu arteritis affects medium to large vessels. However, data about its coronary manifestations are limited. This case illustrates the diagnostic challenges in identifying TAK, particularly if the primary manifestation is coronary artery involvement. Some studies have revealed that coronary ectasia/aneurysms or stenoses involve ostial/proximal portions of coronary arteries.^2,3 TAK is frequently misdiagnosed due to non-specific symptoms, and coronary artery lesions can worsen patient prognosis. Previous studies, such as that by,⁴ reported an average diagnostic delay of 66 months in patients with TAK, due to the absence of specific biomarkers.

This case underscores the importance of considering autoimmune vasculopathy in patients with persistent coronary complications and unexplained systemic symptoms to facilitate earlier intervention and improve prognosis.

The literature also emphasizes the role of immune-mediated inflammation in TAK pathogenesis, including cytokine overproduction and hormonal factors. Imaging techniques, particularly magnetic resonance imaging and ¹⁸F-fluorodeoxyglucose-positron emission tomography/CT scan, are recommended for detecting vessel inflammation. Meanwhile, angiography can confirm structural damage. In 2023, a study reported an unusual case of a mismatch in systemic and local inflammatory activity in TAK with coronary involvement.⁵ This mismatch causes refractory TAK with coronary artery involvement.

Based on the 2021 recommendations of the American College of Rheumatology/Vasculitis Foundation for the Management of Giant Cell Arteritis and Takayasu Arteritis, the decision regarding vascular surgery and the timing and type of intervention should be a joint decision between the vascular surgeon and the rheumatologist.⁶

According to the current guidelines from the American College of Rheumatology, vascular interventions should ideally be performed during remission phases to achieve optimal outcomes.⁷

Despite therapeutic advancements, the ideal treatment duration for TAK remains uncertain. To identify disease activity biomarkers, prognostic indicators, and clinical sequelae of vascular abnormalities, randomized clinical trials comparing current immunosuppressive therapies and longitudinal studies with laboratory and imaging follow-ups should be performed.

Patient perspective

The patient expressed relief after experiencing symptom improvement and having fewer hospitalizations since the initiation of immunosuppressive therapy. She reported feelings of frustration due to prolonged diagnostic process, but felt optimistic with the multidisciplinary approach and ongoing management.

This case emphasizes the importance of considering autoimmune vasculopathy in patients with complex coronary presentations, particularly in the presence of recurrent restenosis and systemic symptoms. A comprehensive approach combining clinical assessment, imaging, and immunosuppressive therapy can improve patient outcomes. Nevertheless, further research should be performed to establish standardized treatment protocols. In particular, anti-inflammatory therapy has been effective in stabilizing coronary artery disease. However, it did not prevent the progression of atherosclerosis in the lower limbs. Continuous monitoring and adherence to immunosuppressive therapy remain crucial for the long-term management of autoimmune vasculopathy.

Lead author biography

Agata Puzzovivo is a clinical cardiologist with a PhD in medical and experimental sciences applied to clinical practice from the University of Bari. She specializes in heart failure, cardiomyopathy, and cardio-oncology.

Consent: Written informed consent for the publication of this case report and any accompanying images was obtained from the patient. The authors confirm that this work is in accordance with the ethical standards and recommendations outlined by the Committee on Publication Ethics.

Funding

Ministero della Salute ‘Ricerca Corrente 2025’.

Data availability

There are no new data associated with this article.