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Juvenile Takayasu Arteritis: A case report
G Model JICC 392 No. of Pages 3
Journal of Indian College of Cardiology xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Indian College of Cardiology journal homepage: www.elsevier.com/locate/jicc
Case Report
Juvenile Takayasu Arteritis: A case report Narendra Nath Khannaa,* , Rini Pramestib , Santosh Sinhab , Emmanuel Auchi Edafeb , Peter Dodo Yakubub , Obiora Ajuluchukwub , Suparna Raob a MD, DM, FRCP (Lond., Glasg., Edin.), FACC, FSCAI, FESC, FAPSIC, FCCP, FIMSA. FEISI, FCSI, FICP, FICC, Sr. Consultant, Interventional Cardiology & Vascular Interventions, Advisor – Apollo Group of Hospitals, Indraprastha Apollo Hospital, New Delhi b Clinical Associate (Fellows- Interventional Cardiology & Vascular Interventions), Indraprastha Apollo Hospital
A R T I C L E I N F O
Article history: Received 1 September 2016 Accepted 12 January 2017 Available online xxx Keywords: Endovascular intervention Juvenile Takayasu Arteritis
A B S T R A C T
Takayasu Arteritis (TA) is rare systemic large vessel chronic inflammatory vasculitis affecting the aorta and its main branches. It was first reported by Mikito Takayasu, a Japanese ophthalmologist. Its etiology is still unknown, however, it has been considered to be of autoimmune origin. TA is more common among young women of childbearing age. It is mostly found in Asia. Takayasu arteritis is rare in children. We present a six years old girl, referred with complaints of low grade fever, headache and dyspnea of 2 months’ duration. She had a significant blood difference between upper and lower extremities on both sides with absent pedal pulses on both sides. She also had anemia and raised ESR. Computed Tomographic imaging showed stenosis of the descending aorta and left subclavian artery. She underwent percutaneous endovascular therapy and blood pressure gradient reduced significantly and pulse volume improved. Her symptoms of headache and breathlessness improved at the time of discharge after the procedure after 48 hours. © 2017 Indian College of Cardiology. All rights reserved.
1. Introduction Takayasu Arteritis (TA) is rare systemic large vessel chronic inflammatory vasculitis affecting the aorta and its main branches.1 Some cases involved pulmonary and coronary arteries.2 It was first reported in 1908 by Mikito Takayasu, a Japanese ophthalmologist. Its etiology is still unknown, however, it has been considered to be of autoimmune origin. TA is more common among young women of childbearing age. Takayasu's arteritis is recognized world-wide, although it is commoner in South-east Asia, Africa and South America.3 Despite being the third most frequent vasculitis in childhood, the occurrence of juvenile TA is scarce. The youngest patient reported with TA was 6-month old baby. The pathologic course of the disease is pan arteritis, characterized by granulomatous inflammation of the aorta and its major branches, leading to stenosis, thrombosis and aneurysm formation with resultant morbidity & mortality. Juvenile TA presents majorly with nonspecific clinical features. This leads to delay in its early
* Corresponding author at: L-117, Sarita Vihar, New Delhi 9810494072. E-mail address: [email protected] (N.N. Khanna).
1100076. Tel.: +91
diagnosis and consequently resulting in serious complications. Complications include cardiovascular, pulmonary, neurologic and secondary to long-term steroid and immunosuppressive therapy. Advanced imaging including magnetic resonance angiography play a vital role in the diagnosis of juvenile TA.4 Endovascular intervention or open surgery is required for the treatment of stenosis/occlusive or aneurysmal vessels in chronic juvenile TA. 2. Case report A six years old girl, resident of Kanpur, Uttar Pradesh was referred by a general practitioner with complaints of low grade fever, headache and dyspnea of 2 months duration. There was no history of hypertension or valvular heart disease. On admission, she looked small for her age with body weight 17 kgs, conscious and afebrile. Blood pressure on both arms were 120/80 mmHg and 70/40 mmHg on both legs. On examination, there was absent pulse on tibial and femoral arteries on both legs. Other examination findings were not remarkable. Laboratory investigations showed mild anemia with hemoglobin 11.4 mg/dl, erythrocyte sediment rate (ESR) using the western green method was elevated (25 mm/hour). Chest radiography was normal. Computed Tomography (CT) scan of the thorax and
http://dx.doi.org/10.1016/j.jicc.2017.01.001 1561-8811/© 2017 Indian College of Cardiology. All rights reserved.
Please cite this article in press as: N.N. Khanna, et al., Juvenile Takayasu Arteritis: A case report, J Indian Coll Cardiol. (2017), http://dx.doi.org/ 10.1016/j.jicc.2017.01.001
G Model JICC 392 No. of Pages 3
2
N.N. Khanna et al. / Journal of Indian College of Cardiology xxx (2016) xxx–xxx
between upper and lower limbs. Femoral and tibial pulses were palpable on both legs. Patient was started on aspirin 75 mg once daily and discharged on the second day. Methylprednisolone 20 mg per day was given orally for 3 months and then tapered. After 3 months’ follow-up, blood pressure was stable at 100/60 mmHg and patient was asymptomatic.
3. Discussion
Fig. 1. CT abdomen and aortography. A. Thoraco-abdomen CT showing narrowing of the aorta and left subclavian artery B. Aortography showing narrowing of the aorta & left subclavian artery
abdomen showed narrowing of the mid aorta and left subclavian artery. (Fig. 1A) An aortography revealed narrowing of descending thoracic abdominal aorta. (Fig. 1B) The patient fulfilled the EULAR (European League Against Rheumatism)/PRES (Paediatric Rheumatology European Society) criteria5 and clinically was diagnosed as Takayasu Arteritis with Hypertension. Patient was planned for Percutaneous Transluminal Angioplasty (PTA) for the treatment of stenosed descending thoracic and abdominal aorta. Access was gained through the right femoral artery (retrograde approach) using micro puncture set. Six French long sheath was advanced through a Teflon 0.035-inch guide wire. Predilatation was performed gradually. First predilatation was done using Mustang 6 40 mm balloon (Boston Scientific), followed by Mustang 8 40 mm. (Fig. 2A–C) Aortography post PTA was performed with good results, no dissections. Pressure gradient trans-aorta before procedure was about 50 mmHg, after the procedure pressure gradient became 10 mmHg. No stent was deployed in this patient because she was a growing child. Her symptoms resolved completely post-procedure. Her blood pressure was 100/60 mmHg, there was no significant difference
Patients with juvenile TA generally present below the age of 8 years with non-specific systemic symptoms such as fever and weight loss. This differs from adult TA patients, who usually present with hypertension, decreased peripheral artery pulsations or claudication of the extremities. Hypertension is one of major clinical features in TA. There was unknown history of hypertension in this patient, but at presentation, the blood pressure in the upper limb was 120/80 mmHg, which is stage II hypertension for a six-year-old child.6 The difference in blood pressure of more than 20 mmHg between the upper and lower extremities is a major finding in juvenile TA. Vascular involvement included narrowing of thoraco-abdominal aorta and proximal left subclavian artery, consistent with other studies of juvenile TA. Ultrasound and MR angiography are the preferred mode of diagnosis in children rather than PET scan as it does not involve radiation. Endovascular interventions and surgical procedures are performed for stenotic/occlusive or aneurysmal vessels in chronic TA. Percutaneous Transluminal Angioplasty (PTA) in children has high-success rate with good patency. The success indicator in endovascular treatment is reduction of pressure gradient more than 50%. Stenting is reserved only for the children with recurrent stenosis. In this patient, pressure gradient reduced to more than 50% after PTA and as such, there was no indication for stenting.7 PTA in children has been reported to have success rate of about 80% with good patency on short term. On follow up, restenosis was found in 20% with 60% patients having new lesions. Other pediatric studies have shown success rates of PTA ranging from 83-93%.
Fig. 2. PTA procedure of Juvenile Takayasu Arteritis. A. Thoraco-abdominal aorta before the procedure, Pressure gradient 50 mmHg B. Thoraco-abdominal aorta ballooning with non compliant balloon C. Thoraco-abdominal DSA post procedure; pressure gradient 10 mmHg
Please cite this article in press as: N.N. Khanna, et al., Juvenile Takayasu Arteritis: A case report, J Indian Coll Cardiol. (2017), http://dx.doi.org/ 10.1016/j.jicc.2017.01.001
G Model JICC 392 No. of Pages 3
N.N. Khanna et al. / Journal of Indian College of Cardiology xxx (2016) xxx–xxx
Intervention should be performed during period of remission because of higher complications and restenosis rate in patients with active disease. 4. Conclusion Juvenile Takayasu Arteritis is a rare disease seen in children less than 8 years of age. It differs from adult TA in that it has more systemic symptoms and less specific symptoms. The diagnosis is sometimes difficult and missed because of lack of specific symptoms, and presence of non-specific symptoms such as fever and weight loss. Treatment is restricted to balloon angioplasty as children are in growing age and stents are only reserved for suboptimal angioplasty results or flow limiting dissection. Conflict of interest
3
Reference 1. Jain S, Pondaiah SK. Takayasu’s arteritis: review of epidemiology and etiopathogenesis. Indian J Rheum. 2015;10:S22–29. 2. Kothari SS. Takayasu's arteritis in children a review. Images Paediatr Cardiol. 2001;3(4):4–23. 3. Lande A, Bard R, Rossi P, Passariello R, Castrucci A. Takayasu's arteritis. A worldwide entity. NY State J Med. 1976;32:379–392. 4. Cirillo RL, Cho KJ, Coombs BD. Takayasu arteritis imaging: overview, computed tomography, magnetic resonance imaging. (PubMed) http://emedicine. medscape.com/article/417244-overview#a1. 5. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–941. 6. US Department of Health and Human Service, National Institutes of Health, National Heart, Lung, and Blood Institute. A Pocket Guide to Blood Pressure Measurement in Children. NIH Publication; 2007. 07–5268 May https://www. nhlbi.nih.gov/files/docs/bp_child_pocket.pdf. 7. Khanna NN, Rao S. Takayasu arteritis. In: Khanna NN, Henry M, eds. Handbook of interventions for structural heart and peripheral vascular disease. Jaypee Brothers; 2015:154–162.
The authors have none to declare.
Please cite this article in press as: N.N. Khanna, et al., Juvenile Takayasu Arteritis: A case report, J Indian Coll Cardiol. (2017), http://dx.doi.org/ 10.1016/j.jicc.2017.01.001
Journal of Indian College of Cardiology xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Indian College of Cardiology journal homepage: www.elsevier.com/locate/jicc
Case Report
Juvenile Takayasu Arteritis: A case report Narendra Nath Khannaa,* , Rini Pramestib , Santosh Sinhab , Emmanuel Auchi Edafeb , Peter Dodo Yakubub , Obiora Ajuluchukwub , Suparna Raob a MD, DM, FRCP (Lond., Glasg., Edin.), FACC, FSCAI, FESC, FAPSIC, FCCP, FIMSA. FEISI, FCSI, FICP, FICC, Sr. Consultant, Interventional Cardiology & Vascular Interventions, Advisor – Apollo Group of Hospitals, Indraprastha Apollo Hospital, New Delhi b Clinical Associate (Fellows- Interventional Cardiology & Vascular Interventions), Indraprastha Apollo Hospital
A R T I C L E I N F O
Article history: Received 1 September 2016 Accepted 12 January 2017 Available online xxx Keywords: Endovascular intervention Juvenile Takayasu Arteritis
A B S T R A C T
Takayasu Arteritis (TA) is rare systemic large vessel chronic inflammatory vasculitis affecting the aorta and its main branches. It was first reported by Mikito Takayasu, a Japanese ophthalmologist. Its etiology is still unknown, however, it has been considered to be of autoimmune origin. TA is more common among young women of childbearing age. It is mostly found in Asia. Takayasu arteritis is rare in children. We present a six years old girl, referred with complaints of low grade fever, headache and dyspnea of 2 months’ duration. She had a significant blood difference between upper and lower extremities on both sides with absent pedal pulses on both sides. She also had anemia and raised ESR. Computed Tomographic imaging showed stenosis of the descending aorta and left subclavian artery. She underwent percutaneous endovascular therapy and blood pressure gradient reduced significantly and pulse volume improved. Her symptoms of headache and breathlessness improved at the time of discharge after the procedure after 48 hours. © 2017 Indian College of Cardiology. All rights reserved.
1. Introduction Takayasu Arteritis (TA) is rare systemic large vessel chronic inflammatory vasculitis affecting the aorta and its main branches.1 Some cases involved pulmonary and coronary arteries.2 It was first reported in 1908 by Mikito Takayasu, a Japanese ophthalmologist. Its etiology is still unknown, however, it has been considered to be of autoimmune origin. TA is more common among young women of childbearing age. Takayasu's arteritis is recognized world-wide, although it is commoner in South-east Asia, Africa and South America.3 Despite being the third most frequent vasculitis in childhood, the occurrence of juvenile TA is scarce. The youngest patient reported with TA was 6-month old baby. The pathologic course of the disease is pan arteritis, characterized by granulomatous inflammation of the aorta and its major branches, leading to stenosis, thrombosis and aneurysm formation with resultant morbidity & mortality. Juvenile TA presents majorly with nonspecific clinical features. This leads to delay in its early
* Corresponding author at: L-117, Sarita Vihar, New Delhi 9810494072. E-mail address: [email protected] (N.N. Khanna).
1100076. Tel.: +91
diagnosis and consequently resulting in serious complications. Complications include cardiovascular, pulmonary, neurologic and secondary to long-term steroid and immunosuppressive therapy. Advanced imaging including magnetic resonance angiography play a vital role in the diagnosis of juvenile TA.4 Endovascular intervention or open surgery is required for the treatment of stenosis/occlusive or aneurysmal vessels in chronic juvenile TA. 2. Case report A six years old girl, resident of Kanpur, Uttar Pradesh was referred by a general practitioner with complaints of low grade fever, headache and dyspnea of 2 months duration. There was no history of hypertension or valvular heart disease. On admission, she looked small for her age with body weight 17 kgs, conscious and afebrile. Blood pressure on both arms were 120/80 mmHg and 70/40 mmHg on both legs. On examination, there was absent pulse on tibial and femoral arteries on both legs. Other examination findings were not remarkable. Laboratory investigations showed mild anemia with hemoglobin 11.4 mg/dl, erythrocyte sediment rate (ESR) using the western green method was elevated (25 mm/hour). Chest radiography was normal. Computed Tomography (CT) scan of the thorax and
http://dx.doi.org/10.1016/j.jicc.2017.01.001 1561-8811/© 2017 Indian College of Cardiology. All rights reserved.
Please cite this article in press as: N.N. Khanna, et al., Juvenile Takayasu Arteritis: A case report, J Indian Coll Cardiol. (2017), http://dx.doi.org/ 10.1016/j.jicc.2017.01.001
G Model JICC 392 No. of Pages 3
2
N.N. Khanna et al. / Journal of Indian College of Cardiology xxx (2016) xxx–xxx
between upper and lower limbs. Femoral and tibial pulses were palpable on both legs. Patient was started on aspirin 75 mg once daily and discharged on the second day. Methylprednisolone 20 mg per day was given orally for 3 months and then tapered. After 3 months’ follow-up, blood pressure was stable at 100/60 mmHg and patient was asymptomatic.
3. Discussion
Fig. 1. CT abdomen and aortography. A. Thoraco-abdomen CT showing narrowing of the aorta and left subclavian artery B. Aortography showing narrowing of the aorta & left subclavian artery
abdomen showed narrowing of the mid aorta and left subclavian artery. (Fig. 1A) An aortography revealed narrowing of descending thoracic abdominal aorta. (Fig. 1B) The patient fulfilled the EULAR (European League Against Rheumatism)/PRES (Paediatric Rheumatology European Society) criteria5 and clinically was diagnosed as Takayasu Arteritis with Hypertension. Patient was planned for Percutaneous Transluminal Angioplasty (PTA) for the treatment of stenosed descending thoracic and abdominal aorta. Access was gained through the right femoral artery (retrograde approach) using micro puncture set. Six French long sheath was advanced through a Teflon 0.035-inch guide wire. Predilatation was performed gradually. First predilatation was done using Mustang 6 40 mm balloon (Boston Scientific), followed by Mustang 8 40 mm. (Fig. 2A–C) Aortography post PTA was performed with good results, no dissections. Pressure gradient trans-aorta before procedure was about 50 mmHg, after the procedure pressure gradient became 10 mmHg. No stent was deployed in this patient because she was a growing child. Her symptoms resolved completely post-procedure. Her blood pressure was 100/60 mmHg, there was no significant difference
Patients with juvenile TA generally present below the age of 8 years with non-specific systemic symptoms such as fever and weight loss. This differs from adult TA patients, who usually present with hypertension, decreased peripheral artery pulsations or claudication of the extremities. Hypertension is one of major clinical features in TA. There was unknown history of hypertension in this patient, but at presentation, the blood pressure in the upper limb was 120/80 mmHg, which is stage II hypertension for a six-year-old child.6 The difference in blood pressure of more than 20 mmHg between the upper and lower extremities is a major finding in juvenile TA. Vascular involvement included narrowing of thoraco-abdominal aorta and proximal left subclavian artery, consistent with other studies of juvenile TA. Ultrasound and MR angiography are the preferred mode of diagnosis in children rather than PET scan as it does not involve radiation. Endovascular interventions and surgical procedures are performed for stenotic/occlusive or aneurysmal vessels in chronic TA. Percutaneous Transluminal Angioplasty (PTA) in children has high-success rate with good patency. The success indicator in endovascular treatment is reduction of pressure gradient more than 50%. Stenting is reserved only for the children with recurrent stenosis. In this patient, pressure gradient reduced to more than 50% after PTA and as such, there was no indication for stenting.7 PTA in children has been reported to have success rate of about 80% with good patency on short term. On follow up, restenosis was found in 20% with 60% patients having new lesions. Other pediatric studies have shown success rates of PTA ranging from 83-93%.
Fig. 2. PTA procedure of Juvenile Takayasu Arteritis. A. Thoraco-abdominal aorta before the procedure, Pressure gradient 50 mmHg B. Thoraco-abdominal aorta ballooning with non compliant balloon C. Thoraco-abdominal DSA post procedure; pressure gradient 10 mmHg
Please cite this article in press as: N.N. Khanna, et al., Juvenile Takayasu Arteritis: A case report, J Indian Coll Cardiol. (2017), http://dx.doi.org/ 10.1016/j.jicc.2017.01.001
G Model JICC 392 No. of Pages 3
N.N. Khanna et al. / Journal of Indian College of Cardiology xxx (2016) xxx–xxx
Intervention should be performed during period of remission because of higher complications and restenosis rate in patients with active disease. 4. Conclusion Juvenile Takayasu Arteritis is a rare disease seen in children less than 8 years of age. It differs from adult TA in that it has more systemic symptoms and less specific symptoms. The diagnosis is sometimes difficult and missed because of lack of specific symptoms, and presence of non-specific symptoms such as fever and weight loss. Treatment is restricted to balloon angioplasty as children are in growing age and stents are only reserved for suboptimal angioplasty results or flow limiting dissection. Conflict of interest
3
Reference 1. Jain S, Pondaiah SK. Takayasu’s arteritis: review of epidemiology and etiopathogenesis. Indian J Rheum. 2015;10:S22–29. 2. Kothari SS. Takayasu's arteritis in children a review. Images Paediatr Cardiol. 2001;3(4):4–23. 3. Lande A, Bard R, Rossi P, Passariello R, Castrucci A. Takayasu's arteritis. A worldwide entity. NY State J Med. 1976;32:379–392. 4. Cirillo RL, Cho KJ, Coombs BD. Takayasu arteritis imaging: overview, computed tomography, magnetic resonance imaging. (PubMed) http://emedicine. medscape.com/article/417244-overview#a1. 5. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–941. 6. US Department of Health and Human Service, National Institutes of Health, National Heart, Lung, and Blood Institute. A Pocket Guide to Blood Pressure Measurement in Children. NIH Publication; 2007. 07–5268 May https://www. nhlbi.nih.gov/files/docs/bp_child_pocket.pdf. 7. Khanna NN, Rao S. Takayasu arteritis. In: Khanna NN, Henry M, eds. Handbook of interventions for structural heart and peripheral vascular disease. Jaypee Brothers; 2015:154–162.
The authors have none to declare.
Please cite this article in press as: N.N. Khanna, et al., Juvenile Takayasu Arteritis: A case report, J Indian Coll Cardiol. (2017), http://dx.doi.org/ 10.1016/j.jicc.2017.01.001