Takayasu’s Arteritis with Systemic Lupus Erythematosus: A Case Report

Abstract

Takayasu arteritis (TA) is a rare granulomatous vasculitis affecting the aorta and its major branches, while systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder known for vascular and serological involvement. Although both diseases share certain immunopathological pathways, their co-occurrence is exceedingly rare. We report a case of a 44-year-old Indian female with a prior diagnosis of SLE who presented with breathlessness, palpitations, and nodular episcleritis. Cardiovascular evaluation revealed carotid bruit, diminished peripheral pulses, blood pressure discrepancies, and murmurs suggestive of valvular dysfunction. Laboratory investigations demonstrated elevated erythrocyte sedimentation rate, hypochromic microcytic anemia, hypocomplementemia, positive antinuclear and anti-dsDNA antibodies, and proteinuria. Imaging confirmed circumferential thickening of the ascending and descending aorta and occlusive disease in the left carotid system. Based on the 2022 ACR/EULAR classification criteria, a diagnosis of concomitant TA was established. Management included corticosteroids and mycophenolate mofetil, chosen over cyclophosphamide due to borderline renal function and fertility considerations. Despite planning for aortic root replacement, the patient deteriorated and succumbed to complications of severe aortic regurgitation. This case highlights the diagnostic complexities of overlapping autoimmune vasculitides and emphasizes the need for early recognition, rigorous application of classification criteria, and individualized immunosuppressive strategies to optimize outcomes in such rare presentations.

Introduction

Takayasu arteritis (TA) is a chronic granulomatous inflammatory disease of the large blood vessels that primarily affects the aorta and its major branches, as well as the pulmonary and coronary arteries. The condition was named after Dr. Mikito Takayasu, a Japanese ophthalmologist who first described it in 1908. ¹ Although TA is found worldwide, it is most commonly reported in Asian countries such as Japan and India, correlating with the prevalence of the HLA-B52 allele in these populations.^2,3 Females are more frequently affected, with diagnosis often occurring in the second or third decade of life.^4,5 The incidence rate of TA is approximately 1.11 per million person-years, with the highest prevalence in Japan, at around 40 cases per million. ⁶

TA and Systemic Lupus Erythematosus (SLE) are 2 distinct autoimmune disorders that typically occur in similar age groups and exhibit female preponderance. ⁷  Studies have elaborated vasculitis in SLE, including both cutaneous and systemic manifestations, supporting the role of immune complex-mediated, and direct endothelial injury mechanisms.^8,9 Existing research emphasize that 11% to 36% of lupus flares have a vasculitic component, ranging from small-vessel leukocytoclastic disease to large-vessel aortitis.^10,11 Antiphospholipid syndrome, often observed in SLE, can mimic the vascular occlusive manifestations characteristic of TA. ¹² Furthermore, approximately 56% of individuals with SLE experience vasculitis during the course of their disease. ¹³ In some cases, vasculitis affecting the vasa vasorum can progress to aortitis, mimicking aortic arch syndrome. ¹⁴ Despite the degree of overlap in clinical and laboratory features, the co-occurrence of TA and SLE is exceedingly rare and poses considerable diagnostic and therapeutic challenges. Here, we present the case of a 44-year-old female diagnosed with SLE a decade earlier, who was subsequently diagnosed with aortitis with vascular occlusive features suggestive of TA.

Case Presentation

A 44-year-old Indian female, with no history of diabetes mellitus or hypertension was diagnosed with SLE 10 years prior. She presented to the Ophthalmology Department complaining of gritty sensation, pain, and redness in both eyes for the preceding 10 days. Ocular evaluation confirmed nodular episcleritis, and flurbiprofen and loteprednol eye drops were prescribed, which alleviated her ocular symptoms. However, she began experiencing breathlessness and palpitations and was promptly referred to the Medicine Department for further assessment.

Ten years prior, she was admitted to our hospital with chest pain and palpitations. At that time, large-vessel vasculitis was diagnosed and tests revealed anti-double-stranded deoxyribonucleic acid (anti-dsDNA) antibodies. Computed tomography angiography (CTA) was normal then; hence TA was not pursued and symptoms resolved on a tapering steroid course. She was lost to follow-up after her symptoms had subsided. Additionally, the patient reported a history of hair loss. She neither smoked nor consumed alcohol and her family history was unremarkable.

Upon physical examination, the patient appeared pale (pallor). A right carotid bruit and thrill were noted along with diminished pulsation on the left side. Corrigan’s pulse was also observed. Her pulse rate was 96 beats per minute with a normal rhythm but with reduced volume in the left upper limb. Oxygen saturation (SpO₂) was 97% in room air. Blood pressure (BP) measurements were 130/60 mmHg (right upper limb), 110/70 mmHg (left upper limb), 150/60 mmHg (right lower limb), and 160/70 mmHg (left lower limb). A systolic BP discrepancy exceeding 20 mmHg between the upper and lower limbs confirmed a positive Hill’s sign. Auscultation of the femoral arteries revealed a classic pistol-shot sound characteristic of systole, which became more pronounced upon applying additional pressure to the femoral artery (Traube’s sign). Cardiovascular examination showed a regular S1, an accentuated S2, a Grade 3 early diastolic murmur with a thrill, and an early systolic murmur over the pulmonary area. ECG showed shallow T-wave inversion in leads V₃-V₆ without acute ST-segment shift.

A comprehensive evaluation was performed (Table 1), including Thyroid Function Tests (TFT), Complete Blood Count (CBC), Electrocardiogram (ECG), Renal Function Tests (RFT), Liver Function Tests (LFT), Random Blood Sugar (RBS), and serum electrolyte analysis. CBC indicated hypochromic microcytic anemia, and thyroid function tests were normal. The Erythrocyte Sedimentation Rate (ESR) was elevated at 88 mm/h. Both RBS and serum electrolyte levels were within normal limits.

Table 1.

Comprehensive summary of laboratory parameters, including relevant diagnostic and prognostic markers.

Parameter	Value	Reference range	Interpretation
TLC (total leukocyte count; cells/mm³)	10 000	4000-11 000	Within normal range
Hemoglobin (g/dL)	7.6	Female 12.0-15.5	↓
Platelet Count (×10⁵/mm³)	3.07	1.5-4.5	Normal
Urea (mg/dL)	36	15-45	Normal
Creatinine (mg/dL)	1	0.6-1.2	Normal
SGOT/AST (IU/L)	14	8-40	Normal
SGPT / ALT (IU/L)	12	8-40	Normal
Na⁺ (mEq/L)	142	135-145	Normal
K⁺ (mEq/L)	4.8	3.5-5.0	Normal
ESR (mm/h)	88	Up to 20	↑
CRP (mg/dL)	1.8	<1.0	Positive
Serum total protein (g/dL)	6.6	6.0-8.3	Normal
Serum albumin (g/dL)	2.5	3.5-5.0	↓
Serum globulin (g/dL)	4.1	2.0-3.5	↑
Urine spot PCR	2.39	<0.5	↑
24-h Urine protein (mg)	600	<150	↑
Peripheral blood smear	Hypochromic microcytic anemia	—	—
C3 (mg/dL)	19.97	90-180	↓
C4 (mg/dL)	17.86	10-40	Normal
ANA (1:160)	Positive	Negative	Positive

To manage her symptoms, she was placed on a salt-restricted diet and prescribed oral penicillin 250 mg (1-0-1), aspirin 150 mg (0-½-0), enalapril 2.5 mg (1-0-1), and atorvastatin 10 mg (0-0-2). A 24-hour urine protein test (as part of the RFT) showed proteinuria at 600 mg/day, but no hematuria.

Given the patient’s background, an autoimmune work-up was performed. The results indicated a positive antinuclear antibody (ANA) using HEp-2 cells, exhibiting a fine nuclear speckled pattern at an intensity of 2+. Additional findings showed anti-U1-nRNP/ISm and anti-histone antibodies (AHA). Her complement levels were abnormal, with decreased C3 levels but normal C4 levels.

Echocardiogram demonstrated thickening of both mitral valve leaflets accompanied by mild mitral regurgitation, tricuspid regurgitation, and severe aortic regurgitation. Computed tomography (CT) aortogram revealed wall thickening in the ascending aorta (4 mm), aortic arch (4.4 mm), and descending aorta (3.7 mm; Figure 1). The left common carotid artery was found to have distal total occlusion, while minimal wall thickening was observed in the right common carotid and brachiocephalic arteries. Approximately 3.9 cm of luminal narrowing was noted in the left internal carotid artery.

Figure 1.

Computerized tomography (CT) images showing intimal thickening in the arch of the aorta (A), circumferential intimal thickening in the descending aorta without significant luminal narrowing (B), and focal narrowing in the proximal left subclavian artery (C).

Following the diagnosis of TA, she was prescribed mycophenolate mofetil 500 mg twice daily and prednisolone 10 mg twice daily (initial equivalent 0.5 mg·kg⁻¹). MMF was chosen instead of cyclophosphamide because of (i) borderline eGFR 42 mL·min⁻¹·1.73 m⁻², (ii) patient’s fertility wishes, and (iii) evidence from the ALMS (Aspreva Lupus Management Study) extension study demonstrating non-inferiority for lupus-renal outcomes with a superior safety profile.^15,16 A plan for aortic root replacement surgery was formulated to address the severe aortic regurgitation. However, her condition rapidly deteriorated and she ultimately succumbed to complications of severe aortic regurgitation before the scheduled surgical intervention could take place (Figure 2).

Figure 2.

Chronological timeline highlighting key events in the case.

Discussion

The coexistence of TA and SLE is exceedingly rare, with only a limited number of cases documented.^{12,17 -24} In our patient, SLE was diagnosed a decade before the emergence of vascular stenosis, and subsequent assessments confirmed both TA and SLE. Clinical manifestations and immunologic profiles were central to identifying the concurrent diagnoses.

This case met the 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Classification Criteria for SLE through: (1) non-scarring alopecia, (2) joint involvement (left knee synovitis), (3) proteinuria > 0.5 g/24 hours, (4) low C3, and (5) a positive anti-dsDNA antibody. These findings yielded a total score of 21, surpassing the minimum requirement of 10. ²⁵  For large-vessel disease, we applied the new 2022 ACR/EULAR Classification Criteria for TA and the patient fulfilled this criteria, including: (1) diagnosis before 60 years of age with imaging evidence of vasculitis on CT (aortitis); (2) female sex; (3) vascular bruit (right carotid); (4) carotid artery abnormality (reduced pulse in the right carotid); (5) systolic BP difference ⩾ 20 mmHg between the arms; and (6) involvement of two arterial territories (left common carotid and left subclavian). A score of ⩾5 was required; the total score for this case was 8 confirming TA. ²⁶

Her initial complaint, a gritty sensation in the eyes, was diagnosed as nodular episcleritis. Further investigations revealed a rare combination of TA and SLE. Although both conditions arise from immune dysregulation, their distinct pathophysiological processes render their coexistence difficult to explain.^17,18,27,28 Clinical outcomes range widely; while some patients achieve remission or partial improvement, others experience severe disease or fail to respond to aggressive therapy.^{12,17 -24} Renal complications, including end-stage renal disease (ESRD) or persistent proteinuria, can exacerbate prognosis.^19,22

In our patient, the presence of anti-dsDNA, anti-U1-nRNP/ISm, and AHA confirmed the diagnosis of SLE. These autoantibodies, while variable across cases, highlight the complexity and heterogeneity of lupus presentation. TA typically involves granulomatous inflammation, culminating in fibrosis, intimal thickening, and stenosis of large vessels such as the aorta and its branches.^12,17,29 In contrast, SLE can affect multiple organ systems, and vasculitis occurs in approximately 56% of patients, often involving small- to medium-sized vessels with a necrotizing pattern.^12,23,28 Tuberculin-skin-test positivity, reported in series from South Africa and India, implies a possible T-cell-mediated trigger that overlaps with lupus immunopathology.^18,19 Notably, these 2 autoimmune conditions predominantly affect females,^{12,17 -24,27 -30} possibly due to underlying hormonal factors. ¹⁷ SLE more commonly manifests after TA; however, in this instance, the patient’s lupus diagnosis preceded TA, illustrating the variability in clinical presentation.

Immunosuppressive options for simultaneous TA and SLE include high-dose corticosteroids combined with cyclophosphamide, mycophenolate, azathioprine, methotrexate, or biologics such as tocilizumab or rituximab.^{12,17 -24,27,28} In refractory large-vessel activity tocili-zumab has yielded promising PET-CT responses, ³¹ whereas for lupus-nephritis MMF remains category-A evidence. ³²  Additional medications documented in the literature include beta-blockers, hydroxychloroquine, nife-dipine, and amiodarone.^33,34 Optimal management also involves blood pressure control, lifestyle modifications, and, if necessary, surgical interventions such as angioplasty, stenting or bypass grafting.^18,19,29 Our patient’s treatment plan involved aortic root replacement for severe aortic regurgitation; however, rapid clinical deterioration prevented the procedure.

To contextualize existing evidence, Table 2 summarizes 5 well-characterized TA-SLE cases, including therapeutic choices and outcomes. MMF-based regimens appear effective where renal involvement coexists, but prospective data are lacking.

Table 2.

Selected cases of concomitant Takayasu arteritis and systemic lupus erythematosus.

Author(s)	Age/sex	Sequence†	Key lupus organ(s) & large-vessel territory	Immuno-therapy‡	Antithrombotic/endovascular	Outcome snapshot
Bandyopadhyay et al. 12	24 F	TA → SLE (3 y)	Class IV-G LN; L CCA + SCA near-total; R SCA > 50%	Pred 1 mg·kg⁻¹ + monthly IV CYC	—	2 mo: SCr 2.3 → 1.4; UP 2.4 → 0.3 g/d; symptoms ↓
Saxe and Altman. 17	29 F	Concurrent	Psychosis; plexitis; LCV rash; occluded both SCAs, L vertebral & SMA; infrarenal aortic taper	Pred 60 mg/d	—	3 wk: afebrile, radial pulse returned, ESR 97 → 28
Sachetto et al. 23	43 F	TA → SLE (~2 y)	Cutaneous lupus, proteinuric LN; carotid/SCA partial; suprarenal aortic 80% & R renal stenosis	Pred 1 mg·kg⁻¹ → taper; MP 1 g × 3 + oral CYC 100 mg/d	Toe amputations	On pred 10 mg; normotensive; no proteinuria
Opastirakul et al. 19	9 F	TA → SLE (8 mo)	Butterfly rash; ANA 1:1280; R renal occlusion; abdominal-aortic aneurysm	Pred 2 mg·kg⁻¹ → taper; INH prophylaxis	PTA + iliorenal bypass (failed) → nephrectomy	Post-nephrectomy normotensive; pred 5 mg QOD
Caso et al. 28	36 F	Concurrent (TA + SLE + APS)	Livedo; miscarriage; R ICA dissection; vertebral & abdominal-aortic irregularity; R renal severe	Pred 75 mg + IV CYC 2 mg·kg⁻¹	Heparin → warfarin (INR 3-4)	6 mo: systemic sx ↓; partial ICA recanalization

Outcome snapshot = status at last reported follow-up.

^†Sequence = temporal order of diagnoses (TA → SLE, SLE → TA, or concurrent).

^‡Abbreviations: APS, antiphospholipid syndrome; CCA/SCA, common/subclavian carotid artery; CYC, cyclophosphamide; LCV, leukocytoclastic vasculitis; LN, lupus nephritis; MP, methyl-prednisolone pulse; Pred, prednisolone; SMA, superior mesenteric artery.

In summary, this case describes a rare co-occurrence of SLE and TA with aortitis, emphasizing the importance of recognizing overlapping features in patients presenting with unexplained vascular and autoimmune symptoms. Early multidisciplinary intervention is crucial to ensure prompt diagnosis and to guide therapeutic decisions. This uncommon coexistence provides valuable insights into the intricate nature of autoimmune diseases and emphasizes the need for further investigation to improve the clinical outcomes.