Takayasu Arteritis: What Can Go Wrong in The Glomeruli for Large Vessel Vasculitis? A Case Report of an Unusual Cause of Persistent Microscopic Hematuria in a Patient with Takayasu Arteritis

Takayasu arteritis is a rare systemic large vessel vasculitis affecting the aorta and its branches. Sarcoidosis, too, is an inflammatory disease. Both entities are granulomatous conditions with a question of possible linkquestionable association in their etiopathogenesis. Only a few cases of their coexistence have been reported in the literature. To our knowledge, no such cases have been reported from Nepal. We report a Nepalese woman who presented with non-productive cough, progressive shortness of breath and chest tightness of 3 years duration. She had a history of recurrent bilateral granulomatous uveitis over the previous 3 years. Examination revealed clubbing of digits, absent pulses over the left radial, ulnar and brachial arteries, and a weak pulse over the right arm including the bilateral carotid arteries. Pulmonary function test showed restrictive pattern, a high-resolution computed tomography (HRCT) scan of the chest revealed findings suggestive of pulmonary sarcoidosis. A CT aortogram suggested large vessel vasculitis. Bronchoscopy with biopsy revealed granulomatous inflammation, negative for malignancy and tuberculosis. She was hence, diagnosed with co-existing Takayasu arteritis and sarcoidosis, and treated with Prednisolone 60 mg once daily with dramatic improvement over 4 days and was discharged stable on domiciliary oxygen. She is currently on azathioprine 50 mg, prednisolone 10 mg without the need for supplemental oxygen. This case report highlights the importance of a proper physical examination as a guide to the use of modern technology in making a correct diagnosis. Furthermore, in countries where tuberculosis is endemic, it should always come as the most important differential diagnosis of granulomatous inflammation.

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Ankylosing spondylitis with concurrent Takayasu arteritis: a clinical case report

Modern Rheumatology Journal ◽

10.14412/1996-7012-2019-4-75-78 ◽

2019 ◽

Vol 13 (4) ◽

pp. 75-78

Author(s):

O. B. Nesmeyanova ◽

G. M. Khusainova ◽

E. A. Bogdanova ◽

O. V. Solovyeva ◽

K. P. Ratushnaya ◽

...

Keyword(s):

Cardiovascular Disease ◽

Case Report ◽

Ankylosing Spondylitis ◽

Acute Phase ◽

Takayasu Arteritis ◽

Clinical Case ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

The Common ◽

Disease Characteristic

The paper describes a rare clinical case of ankylosing spondylitis (AS) with concurrent with Takayasu arteritis (AT). It considers the common immunopathogenetic mechanisms that can link these two diseases. Taking into account that there may be common triggering factors for AS and AT, it is necessary to make an examination to diagnose AT when a patient with AS is found to have fever, an increase in the indicators of acute phase inflammation, and the objective signs of cardiovascular disease characteristic of large-vessel vasculitis.

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Case Report: Co-existence of sarcoidosis and Takayasu arteritis

Wellcome Open Research ◽

10.12688/wellcomeopenres.15837.2 ◽

2020 ◽

Vol 5 ◽

pp. 73

Author(s):

Jiwan Poudel ◽

Ujjwol Risal ◽

Keshav Raj Sigdel ◽

Buddhi Prasad Paudyal ◽

Sudeep Adhikari ◽

...

Keyword(s):

Case Report ◽

Takayasu Arteritis ◽

Correct Diagnosis ◽

Granulomatous Inflammation ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Dramatic Improvement ◽

High Resolution Computed Tomography ◽

Ct Angiogram ◽

Bilateral Carotid

Takayasu arteritis is a rare systemic large vessel vasculitis affecting the aorta and its branches. Sarcoidosis, too, is an inflammatory disease. Both entities are granulomatous conditions with a questionable association in their etiopathogenesis. Only a few cases of their coexistence have been reported in the literature. To our knowledge, no such cases have been reported from Nepal. We report a Nepalese woman who presented with non-productive cough, progressive shortness of breath and chest tightness of 3 years duration. She had a history of recurrent bilateral granulomatous uveitis over the previous 3 years. Examination revealed clubbing of digits, absent pulses over the left radial, ulnar and brachial arteries, and a weak pulse over the right arm including the bilateral carotid arteries. Pulmonary function test showed restrictive pattern, a high-resolution computed tomography (HRCT) scan of the chest revealed findings suggestive of pulmonary sarcoidosis. A CT angiogram suggested large vessel vasculitis. Bronchoscopy with biopsy revealed granulomatous inflammation, negative for malignancy and tuberculosis. She was hence, diagnosed with co-existing Takayasu arteritis and sarcoidosis, and treated with Prednisolone 60 mg once daily with dramatic improvement over 4 days and was discharged stable on domiciliary oxygen. She is currently on azathioprine 50 mg, prednisolone 10 mg without the need for supplemental oxygen. This case report highlights the importance of a proper physical examination as a guide to the use of modern technology in making a correct diagnosis. Furthermore, in countries where tuberculosis is endemic, it should always come as the most important differential diagnosis of granulomatous inflammation.

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AB1009 THE EFFICACY OF TOCILIZUMAB ON THE TREATMENT OF TAKAYASU ARTERITIS IN CHINESE CHILDREN

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5778 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1798.2-1798

Author(s):

C. Wang ◽

H. Song ◽

Z. Yu ◽

M. Quan

Keyword(s):

Takayasu Arteritis ◽

Case Series ◽

Large Vessel Vasculitis ◽

Chinese Children ◽

Large Vessel ◽

Efficacy And Safety ◽

Imaging Data ◽

Eular Recommendations ◽

Interleukin 6 Receptor ◽

Sparing Effect

Background:Takayasu arteritis (TA) is the most prevalent large-vessel vasculitis in children. Patients with TA have a high mobidity and mortality.It remains a therapeutic challenge because corticosteroids monotherapy can rarely cure TAK and the relapse rate is high during GC tapering.Objectives:The aim of this study is to investigate the efficacy and safety of tocilizumab (TCZ)in Chinese children with Takayasu arteritis(TAK).Methods:We retrospectively studied 6 TAK children treated with TCZ in our hospital from July 2017 to October 2018. The demographic and clinical data, laboratory examination results and vascular imaging data were collected.Results:Six pediatric patients with critical or refractory TAK treated with TCZ were analyzed, including 3 males and 3 females.The diagnosis age was ranging in age from 2 to 13 years(median age:7 years).Three patients were initially treated with TCZ and Mycophenolate Mofetil(MMF) as the first-line regimen without corticosteroid or with a quite rapid GC taper duration,two of which had lifte-threatening coronary arteries involved and heart failure.The other three paitients were swcithed to TCZ from conventional disease modifying anti-rheumatic drugs (DMARDs) or other biologics due to being refractory to them and recurrent relapses.Four patients were given TCZ at 4 weeks regular intervals for 10 to 22 months,while two patients withdrew TCZ because of disease deterioration and unbearable abdominal or chest pain after the second dose.After 6 months follow-up,four patients experienced significant clinical and biological improvement with angiographically progression in one patient. A corticosteroid-sparing effect is obvious. Drug-related side effects occur in 1 patients manifesting as a mild elevated liver fuction. Neither neutropenia nor infection was observed.Conclusion:Our study shows a clinical, biological, and radiological response in patients with refractory TAK treated with TCZ.References :[1]Hellmich B, Agueda A, Monti S,et al.2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2019;0:1–12. doi:10.1136/annrheumdis-2019-215672.[2]BravoMancheño B, Perin F, Guez Vázquez Del ReyMDMR, García Sánchez A, Alcázar Romero PP. Successful tocilizumab treatment in a child with refractory Takayasu arteritis.Pediatrics 2012;130(6):e1720-724.[3]Goel R, Danda D, Kumar S, Joseph G. Rapid control of disease activity by tocilizumab in 10 «difficult-to-treat» cases of Takayasu arteritis. Int J Rheum Dis 2013;16(6):754–61.[4]Cañas CA, Cañas F, Izquierdo JH, Echeverri A-F, Mejía M, Bonilla-Abadía F, et al. Efficacy and safety of anti-interleukin 6 receptor monoclonal antibody (tocilizumab) in Colombian patients with Takayasu arteritis. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis 2014;20(3):125–9.[5]Batu ED, Sönmez HE, Hazirolan T, Özaltin F, Bilginer Y, Özen S. Tocilizumab treatment in childhood Takayasu arteritis: case series of four patients and systematic review of the literature. Semin Arthritis Rheum 2017 Feb;46(4):529–35.Disclosure of Interests:None declared

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Reducing the Toxicity of Long-Term Glucocorticoid Treatment in Large Vessel Vasculitis

Current Rheumatology Reports ◽

10.1007/s11926-020-00961-0 ◽

2020 ◽

Vol 22 (12) ◽

Author(s):

Andriko Palmowski ◽

Frank Buttgereit

Keyword(s):

Adverse Events ◽

Takayasu Arteritis ◽

Term Treatment ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Serious Adverse Events ◽

Glucocorticoid Treatment ◽

Long Term Treatment ◽

Higher Doses

Abstract Purpose While glucocorticoids (GCs) are effective in large vessel vasculitis (LVV), they may cause serious adverse events (AEs), especially if taken for longer durations and at higher doses. Unfortunately, patients suffering from LVV often need long-term treatment with GCs; therefore, toxicity needs to be expected and countered. Recent Findings GCs remain the mainstay of therapy for both giant cell arteritis and Takayasu arteritis. In order to minimize their toxicity, the following strategies should be considered: GC tapering, administration of conventional synthetic (e.g., methotrexate) or biologic (e.g., tocilizumab) GC-sparing agents, as well as monitoring, prophylaxis, and treatment of GC-related AEs. Several drugs are currently under investigation to expand the armamentarium for the treatment of LVV. Summary GC treatment in LVV is effective but associated with toxicity. Strategies to minimize this toxicity should be applied when treating patients suffering from LVV.

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