Amyloid Light Chain Amyloidosis Involving the Heart and Temporal Arteries, Mimicking Giant Cell Arteritis

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: AL amyloidosis (n=1). Intervention: Bortezomib, cyclophosphamide, and dexamethasone.

Ameer Rashed; Ezara Fernandes; Ritsuko Saito; Michael Stewart; Ernest Wong; Kaushik Guha

doi:10.1016/j.jaccas.2026.108680

September 1, 2026JACC Case ReportsOpen Access

Amyloid Light Chain Amyloidosis Involving the Heart and Temporal Arteries, Mimicking Giant Cell Arteritis

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Key result

Bortezomib-based therapy achieves complete remission in AL amyloidosis mimicking giant cell arteritis.

n=1

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Graphical abstract published by JACC Case Reports — Graphical abstract · Published by JACC Case Reports · CC BY-NC-ND

Why the study?

Amyloid light chain (AL) amyloidosis is an uncommon multisystem condition with rare simultaneous involvement of the heart and temporal arteries, mimicking giant cell arteritis.

Key Points

To describe a case of amyloid light chain (AL) amyloidosis simultaneously affecting the heart and temporal arteries that initially mimicked giant cell arteritis.
Assessed a 74-year-old man presenting with jaw claudication, temporal artery tenderness, and an erythrocyte sedimentation rate of 128 mm/h.
Performed temporal artery ultrasonography, admission biomarker testing, transthoracic echocardiography, and cardiac magnetic resonance imaging.
Confirmed the diagnosis using a temporal artery biopsy and initiated chemotherapy with bortezomib, cyclophosphamide, and dexamethasone.
Initial echocardiography demonstrated increased left ventricular wall thickness with an ejection fraction of 64%, while cardiac magnetic resonance revealed elevated T1 mapping and extensive subendocardial late gadolinium enhancement.
Temporal artery biopsy confirmed amyloid light chain (AL) amyloidosis presenting concurrently with infiltrative cardiomyopathy.
Treatment normalized the serum kappa/lambda ratio within 2 weeks and lowered NT-proBNP from 3,871 pg/mL to 438 ng/L at 18 months, leading to complete disease remission.

Study Design

Type

Case Report (n=1)

Structured PICO

P

Population

A 74-year-old man with AL amyloidosis affecting the heart and temporal arteries mimicking giant cell arteritis, followed for 18 months.

I

Intervention

Bortezomib, cyclophosphamide, and dexamethasone (8 cycles)

AL amyloidosis can present with rare, simultaneous involvement of the heart and temporal arteries mimicking giant cell arteritis, underscoring the critical need for cardiac phenotyping in atypical multiorgan presentations.

Limitations

Temporal artery biopsy was initially deferred according to local guidelines, leading to a delay in diagnosis.
Biopsy of other possibly affected large vessels seen on computed tomography imaging was not performed.
PET was not performed during the acute hospital admission.
Laser capture mass spectrometry was not performed.
Daratumumab was not used as it was not licensed for use in AL amyloidosis in the United Kingdom at the time.

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Implication

Supports biopsy confirmation in GCA mimics with cardiac involvement; extends amyloid phenotypes but leaves open generalizability.

Abstract

BACKGROUND: Amyloid light chain (AL) amyloidosis is an uncommon multisystem condition. We report a globally unique case of a patient with AL amyloidosis affecting the heart and temporal arteries. CASE SUMMARY: A 74-year-old man presented with jaw claudication and temporal artery tenderness and was treated for giant cell arteritis. He was later admitted to the cardiology service with elevated cardiac biomarkers with echocardiographic features of an infiltrative cardiomyopathy. Cardiac magnetic resonance suggested amyloidosis, and diagnosis of AL amyloidosis was confirmed via temporal artery biopsy. Treatment with bortezomib, cyclophosphamide, and dexamethasone yielded an appropriate clinical response. DISCUSSION: Although AL amyloidosis is known to be associated with cardiac involvement with isolated cases of large vessel involvement, this is the first global case to our knowledge of AL amyloid affecting the temporal arteries and cardiac function simultaneously. TAKE-HOME MESSAGES: AL amyloidosis is a multisystem disorder. Cardiac phenotyping should be mandatory.

Discussion

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Cite This Study

Rashed et al. (2026) conducted a case report in AL amyloidosis (n=1). Bortezomib, cyclophosphamide, and dexamethasone was evaluated. Treatment with bortezomib, cyclophosphamide, and dexamethasone yielded an appropriate clinical response and complete remission in a patient with AL amyloidosis mimicking giant cell arteritis.

synapsesocial.com/papers/6ad0fd263afa093ea9ad0253 https://doi.org/10.1016/j.jaccas.2026.108680

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Amyloid Light Chain Amyloidosis Involving the Heart and Temporal Arteries, Mimicking Giant Cell Arteritis

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

Amyloid Light Chain Amyloidosis Involving the Heart and Temporal Arteries, Mimicking Giant Cell Arteritis

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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