Takayasu's Arteritis Case Study

By John Drummond

Welcome Back Rheumatology Fans,

We have a guest post in this week! The first one!

John Drummond posted about a fascinating case on “X” (it will always be twitter to me) and I suggested he writes it up as a case study… well here we are, after making the suggestion I had to host it!

Comments very welcome and Thank You to John for sharing and putting so much time and effort into this!

Title

Takayasu’s Arteritis (TA) in a 78-Year-Old Female with Polymyalgia Rheumatica: A Case of progressive systemic decline and a call for MSK clinicians to consider vascular involvement in rheumatic diseases as a rare mascarader or co-existing pathology in an outpatient musculoskeletal setting. 

Abstract

A 78-year-old female with a history of Polymyalgia Rheumatica (PMR) presented with right cubital tunnel syndrome, which developed to include right shoulder pain and multilevel arm weakness. This was followed by an abrupt onset of significant systemic symptoms such as anaemia, rapid weight loss, night sweats, leg weakness, and fatigue. Despite initial investigations focusing on peripheral nerve entrapment and cervical spine pathology, further imaging and clinical assessments by the medical team revealed large vessel vasculitis consistent with the autoimmune disorder, Takayasu’s arteritis. This case highlights the complexity of managing seemingly musculoskeletal conditions especially when neurological, mechanical and systemic symptoms are intertwined. As with Takayasu- as the symptoms are often non-specific and signs easily missed – a diagnostic delay is common

Image adapted from - https://www.vasculitisfoundation.org/education/vasculitis-types/takayasu-arteritis/

Introduction

Takayasu’s Arteritis in an elderly female with Polymyalgia Rheumatica: A Case of progressive systemic decline and a call for musculoskeletal (MSK) clinicians to consider vascular involvement in rheumatic diseases as a rare mascarader or co-existing pathology in an outpatient musculoskeletal setting.

Case Presentation

Patient Information

• Demographics: 78-year-old Caucasian female

• Previous Medical History: Polymyalgia Rheumatica (PMR) (long-term steroid use with recent tapering and discontinuation) and osteoporosis. Right cubital tunnel syndrome 2 years prior, treated conservatively. 

• Medication History: Calci-D, Lansoprazole

• Social Factors: Ex-smoker (approx. 12.5 pack years). She lives with her partner and enjoys walking, though her recent symptoms have significantly limited her activity. Her BMI is 19.64 kg/m², reflecting a recent, unintended weight loss.

Progression of Symptoms (May - August)

• May 2024: Recurrent right cubital tunnel symptoms after re-injury to the elbow, with increasing burning pain, numbness, and weakness in the right hand. The patient also began experiencing right shoulder pain and weakness, particularly in the triceps muscle, which was not explained by cervical MRI or brachial plexus findings.

• June - August: Over a 6-8 week period, the patient developed significant systemic symptoms, including:

  • Appetite loss + Rapid and significant weight loss (2.5kg in 1 month, 4kg total-> BMI 19.64 kg/m²)

  • Night sweats

  • Heavy legs, generalised weakness and difficulty walking

  • Severe fatigue (tired all the time) 

  • New and persistent dry cough, worse at night

  • Shortness of breath on exertion

  • Oral thrush / mouth sores

Screening: Denies any headache, visual disturbance, jaw or tongue claudication or scalp/ temporal tenderness. No personal history of cancer. No other wider bony pains, although later developed a variety of arthralgias. No left sided symptoms. No balance or coordination issues. 

Investigations

• Objective examination: 

  • Gait: Normal no loss of balance 

  • Neck and shoulder: Near full pain free range of the neck. Right shoulder limited active motion globally. 

  • Hoarseness of voice: Considered whether this was related to her history as an ex-smoker or potentially due to laryngeal spasm secondary to Horner’s syndrome, prompting exclusion of an apical lung tumour (Pancoast tumour) given the C7/8 neurological symptoms that were not fully explained by imaging.

• Laboratory Findings:

  • Anti-neutrophil cytoplasm antibodies (ANCA): Negative

  • Blood Tests:

    • Haemoglobin: 83 g/L (Females: 121–151 g/L)

    • Platelets: 617 × 10⁹/L (150 and 400  × 10⁹/L)

    • C-reactive protein (CRP): 203 mg/L (on admission), then 186 mg/L (3 to 10 mg/L)

• Imaging (Primary Care):

  • MRI (Cervical Spine and Brachial Plexus): Showed degenerative changes at C4-C5 and C5-C6 with severe bilateral foraminal narrowing and likely impingement of the C6 nerves bilaterally. 

• Imaging (Secondary Care):

  • CT chest, followed by MRA of the Aorta (14.8.24): Revealed diffuse enhancing circumferential mural thickening and fine intimal calcification throughout the aorta from the distal aortic arch to the aortic bifurcation. There was focal stenosis of the mid left subclavian artery and proximal right renal artery, consistent with large vessel vasculitis.

Treatment and Follow-Up

• Initial Management:

  • Oral Prednisolone:

    • 60 mg for the first week

    • 50 mg for the second week

    • 40 mg for the third week

The 2018 EULAR Algorithm for Pharmacologic Treatment of Takayasu Arteritis

• Monitoring: Follow-up with rheumatology to assess response to treatment and adjust therapy accordingly.

Discussion

This case illustrates the diagnostic challenges of Takayasu’s arteritis in an elderly patient with a complex presentation (Antón, 2005). Initially presenting with right cubital tunnel syndrome, the patient’s symptoms rapidly progressed over 6-8 weeks, raising concerns about a systemic disease. 

The lack of clear findings on cervical MRI and brachial plexus imaging complicated the clinical picture, particularly with the development of significant triceps weakness, which was not directly explained by the imaging results. The patient’s neurological findings presented a complex and progressive pattern of weakness, particularly in the right shoulder and triceps, which was not fully explained by the positive ulnar nerve tests or the cervical spine MRI findings. This did not convincingly present as brachial neuritis but this alongside a compressive lesion remained in the differential. The brisk reflexes, while within normal limits, and the positive Tinel’s and Froment’s signs pointed towards some degree of ulnar nerve involvement. However, the extent and distribution of muscle weakness, especially in the triceps and shoulder, suggested that the pathology might be more widespread than a simple ulnar nerve lesion.

The non-neurological examination findings added further complexity to the case. The reduced range of motion in the right shoulder, coupled with tenderness in the supraspinatus region, suggested a possible rotator cuff issue; however, this would not explain the associated triceps weakness or the broader distribution of symptoms. The normal cervical spine motion and lack of significant neck or elbow tenderness further complicated the diagnosis, as these findings were not consistent with a straightforward radiculopathy or cubital tunnel syndrome. The progression of symptoms, particularly the increased weakness in the shoulder and triceps, raised concerns about a multifactorial issue that could involve both nerve root compression and a broader systemic or vascular process. The cervical spine MRI, which showed severe foraminal narrowing at C5-C6, did not fully account for the triceps weakness, and a rotator cuff pathology would not explain the overall clinical picture. 

The patient’s constitutional signs and systemic symptoms, including shortness of breath, significant weight loss, night sweats, fatigue, and hoarseness of voice, required a broad differential diagnosis. Gamage et al (2012) describe a case with hoarse voice being associated with Takayasu. The hoarseness, in particular, raised concerns about potential laryngeal involvement, either from her history as an ex-smoker or as a possible manifestation of Horner’s syndrome secondary to apical lung pathology, however, this was ruled out with further imaging. Pancoast tumors is most common in middle-aged men, with history of smoking and complaining of chest and shoulder pain, neurological symptoms in upper extremity, and Horner syndrome, but have no risk factors at work or a history of previous trauma. It is worth noting normal/only mildly raised ESR and CRP should not give false reassurance as this may be normal in up to 50 percent of patients with TA.

There could be an argument to be made to consider routine observations such as pulse and blood pressure recorded as a ‘vital sign’ within primary care and musculoskeletal assesments. As Takaysu’s is known as the pulseless disease, high blood pressure may have been observed and helped to point towards a cardiovascular system, usually as a result of decreased blood flow to the kidneys. There is complexity here however as recommendations for both brachial and ankle which is less common within an MSK setting. This is especially the case with introduction of enhanced and advanced practice roles. As a trainee ACP, if access and appropriate supervision, I may also have been able to observe bruit and would demonstrate the generalist ACP benefit, but acknowledging that general practice doctors are already well placed to help differentiate these.

Takayasu’s arteritis is rare in elderly patients but should be considered in cases where systemic symptoms are accompanied by vascular findings. The overlap between polymyalgia and TA, particularly in terms of risk factors and symptomatology, is documented, and the recent tapering of steroids may have contributed to the unmasking of TA in this patient. The negative ANCA and the pattern of vascular involvement confirmed on MRA supported the diagnosis of TA rather than another vasculitis or systemic disease.

Conclusion

This case highlights the importance of considering alternative diagnoses especially when numerous significant systemic concerns are expressed. Takayasu’s arteritis in elderly patients is not a typical demographic but should be considered alongside GCA and PMR as a rare vascular inflammatory manifestation. Early diagnosis and aggressive treatment are crucial in managing TA to prevent further vascular complications and improve patient outcomes.

Reflection: 

The open communication between the general practice team and MSK outpatient team and proactivity of the patient likely allowed for rapid diagnosis in an otherwise conspicuous case. Of course, her medical history of a long-term heavy smoker with shortness of breath and new cough required medical work up to exclude respiratory pathology. Although I was aware of apical lung tumours, there was not significant evidence of Horner’s syndrome or supraclavicular fullness, and they tend not to have typical respiratory symptoms as this is extraparenchymal. 

Initially when I saw the deranged blood tests and she described systemically being unwell with her past medical history of smoking, I considered haematological or respiratory malignancy.  As someone who is not currently able to request bloods and is not used to interpretation, I will aim to complete further learning such as that posted by Giles Hazan

I initially screened for GCA with probing questions relating to headaches, jaw claudication, visual disturbance which I am aware can be associated with PMR but I was not aware of other vasculitis conditions or appropriate screening questions for this. This case however does highlight the importance of appropriate broad ‘red flag’ screening questions to indicate serious or systemic disease. And when the weighting/cluster of these is significant, it suggests that MSK investigation/management should be discontinued. 

Finicune, et al 2020) (Finucane, L. M., Downie, A., Mercer, C., Greenhalgh, S. M., Boissonnault, W. G., Pool-Goudzwaard, A. L., Beneciuk, J. M., Leech, R. L. and Selfe, J. (2020) ‘International Framework for Red Flags for Potential Serious Spinal Pathologies’. Journal of Orthopaedic & Sports Physical Therapy. Journal of Orthopaedic & Sports Physical Therapy, 50(7) pp. 350–372

Potentially nerve conduction studies could help to consider if there is an isolated peripheral nerve lesion which explains the weakness, re-examination on steroids would also aid in differentiating if this was musculoskeletal and physiological or related to inflammatory cause of pain inhibition. Shoulder repeat examination and if indicated, X-ray and ultrasound could be requested for completion to exclude a separate isolated shoulder cause.

I could have considered more routine general safety-netting, I think we can get siloed to consider very specific safety-netting for cauda equina, metastatic spinal cord compression/myelopathy, DVT, trauma/fracture however general practice provides an art of ‘if no better or worse, to return’ advice. Arguably we may not be the most appropriate service to present to, and we do not want to overburden primary care but I should consider routine follow up, safetynetting and written communication with the GP team to ensure patients do not slip through the system. (Silverson, 2014,2020, Roland et al., 2014; Almond, Mant, and Thompson, 2009:973). 

Further safety netting information Thread

Learning Points

• Consider large vessel vasculidites such as GCA (typically younger patient with headache/jaw claudication/visual symptoms with PMR) or Takyasu (typically younger patient) and both presenting with unexplained systemic symptoms

• Having good relationships with medical team to be able to articulate ‘gut instinct’ concerns which ‘don’t fit’ your typical MSK presentations but to be vulnerable to acknowledge one doesn’t know what one doesn’t know. Appropriate wide investigations with high sensitivity but low specificity may be useful as initial screening to hone in the systems which are involved. 

• Her smoking history, weight loss and new cough pointed to malignancy, it is important to consider anchoring bias and ‘zoom out’ to consider alternative explanations 

• Prompt referral and investigation are crucial for diagnosing and managing serious complications.

• It is important to consider that alternative system pathology may co-exist with more simple MSK pathology and we should employ systems thinking to screen for mascaraders 

• Although this case highlights a condition which is ‘rare but there’ the main learning point has been to consider systemic signs not only as a prompt for malignancy but other systemic causes such as inflammatory disease

Figure 1: The final 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis.

Resources and Signposting: 

https://www.vasculitisfoundation.org/education/vasculitis-types/takayasu-arteritis/ 

https://www.vasculitis.org.uk/about-vasculitis/takayasu-arteritis 

Reference List: 

Antón, E. (2005) 'Large vessel arteritis: a diagnostic challenge in the elderly', Journal of Clinical Pathology, 58, pp. 782-784. Available at:

https://www.jclinpath.com

Gamage, N. D. P., Jayasinghe, A. C., Dias, L. D., and Perera, A. K. (2012) 'Bronchiectasis and hoarseness of voice in Takayasu arteritis: a rare presentation', BMC Research Notes, 5, 447. Available at: http://www.biomedcentral.com/1756-0500/5/447.

Grayson, P. C., Ponte, C., Suppiah, R. et al. (2022) '2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis', Annals of the Rheumatic Diseases, 81, pp. 1654-1660.

Huaitao, Y., Wenzhao, L., Yuan, Z., Xuefang, Y., Na, L., Yun, T., and Peili, B. (2021) 'A Case Report: An Elderly Male Patient With Takayasu Arteritis After Coronary Artery Bypass Grafting', Frontiers in Cardiovascular Medicine, 8. DOI: 10.3389/fcvm.2021.766574. Available at: https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2021.766574.

Joseph, G., Goel, R., Thomson, V. S., Joseph, E., and Danda, D. (2023) 'Takayasu Arteritis: JACC Focus Seminar 3/4', Journal of the American College of Cardiology, 81(2), pp. 172-186. Available at: https://doi.org/10.1016/j.jacc.2022.09.051.

Poudel, P., Oli, P., Ojha, P., Yadav, R. S., and Poudel, B. R. (2022) 'Takayasu Arteritis Masquerading as Shoulder Pain: A Case Report', Journal of Nepal Medical Association, 60(248), pp. 393-395. Available at: https://doi.org/10.31729/jnma.7443.

Saadoun, D., Bura-Riviere, A., Comarmond, C. et al. (2021) 'French recommendations for the management of Takayasu’s arteritis', Orphanet Journal of Rare Diseases, 16(Suppl 3), 311. Available at: https://doi.org/10.1186/s13023-021-01922-1.

Shanshan, Z., Dongli, Y., Ge, T. (2019) 'Neurological Involvement in Primary Systemic Vasculitis', Frontiers in Neurology, 10. DOI: 10.3389/fneur.2019.00430. Available at: https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2019.00430.