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| ORIGINAL ARTICLE |
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| Year : 2020 | Volume
: 41
| Issue : 2 | Page : 79-86 |
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Ayurvedic management for a rare disorder Takayasu arteritis – A case report
Sarvesh Kumar Singh1, Kshipra Rajoria1, Sanjeev Sharma2
1 Department of Panchkarma, National Institute of Ayurveda, Jaipur, Rajasthan, India
2 Department of Shalya Tantra, National Institute of Ayurveda, Jaipur, Rajasthan, India
| Date of Submission | 18-Apr-2019 |
| Date of Decision | 10-Jun-2019 |
| Date of Acceptance | 22-Feb-2021 |
| Date of Web Publication | 23-Oct-2021 |
Correspondence Address:
Sarvesh Kumar Singh
Department of Panchkarma, National Institute of Ayurveda, Jaipur-302002, Rajasthan
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ayu.AYU_61_19
 Abstract | | |
Takayasu arteritis (TA) is a rare disorder and it is a devastating condition of aorta. The presently available treatments for the condition in the modern medicine have limited benefits. This is case of TA which was better managed with Ayurvedic intervention. An Ayurvedic diagnosis for this case was Siragata Vata (vitiated Vata Dosha affecting the blood vessels). A 42-year-old woman was diagnosed with TA and treated on the line of management of Siragata Vata with Shastikashali Pinda Swedana (sudation with bolus of medicated cooked rice) for 16 days, Erandamuladi Niruha Basti (enema mainly with decoction) along with Ashwagandha Taila Anuvasana (enema with medicated oil) for 16 days in Kala Basti Krama (16 days in alternate order of decoction and oleation enema) followed by one day gap and then 7 days of Nasya Karma (nasal therapy) with Triphaladi Taila (oil) on alternate days along with a combination of Ayurvedic oral drugs [Brihadvatachintamani Rasa-125 mg, Dashamula Kwatha-40 ml, Narsinha Churna (powder)-3 g, Yogaraja Guggulu-1g (500mgx2tab) and Shiva Gutika-500 mg, twice a day for 1 month. Same Panchakarma procedures were repeated after 6 months. A similar combination of oral medications were continued in between and during this period. Chyavanaprasha Aveleha in the dose of 10g twice a day with milk were also added after completion of this treatment regime. Patient condition was assessed on Indian Takayasu Clinical Activity Score (ITAS-2010) for disease activity of TA. Satisfactory results were observed in the patient with improvement in ITAS-2010 scoring. TA may be managed with Ayurvedic drugs and Panchakarma procedures.
Keywords: Ayurveda, Siragata Vata, Takayasu arteritis, Vata Vyadhi
How to cite this article:
Singh SK, Rajoria K, Sharma S. Ayurvedic management for a rare disorder Takayasu arteritis – A case report. AYU 2020;41:79-86 |
| Introduction | |  |
Takayasu arteritis (TA) is a chronic granulomatous inflammation of large arteries characterized by nonspecific symptoms-such as hypertension, headache, fever, arthralgia, muscle pain, night sweats and weight loss.[1] If not treated in acute early phase, the disease affects the aorta and its main branches. Vessel wall inflammation leads to concentric wall thickening, fibrosis, thrombus formation, stenosis of affected vessels and vascular remodeling. This leads to end-organ ischemia-such as renal infarction and stroke. Takayasu arteritis was named after Mikito Takayasu by Yasuzo Shinmi in 1939.[2] The disease is also known as pulse less disease. TA is rare, but most commonly seen in Japan, South East Asia, Mexico and India. The incidence of TA in adults is estimated to be 2.6/million/year in North America. In India, female to male ratio is 1.7:1.[3] The onset of disease is mainly in second or third decade of life.[4] There is no specific laboratory marker for TA. However, erythrocytes sedimentation rate (ESR) is considered the best available routine laboratory indicator for disease activity of TA in adolescents. Diagnosis can be confirmed only by imaging such as magnetic resonance angiography (MRA), computed tomography angiography (CTA) or doppler ultrasound.
In this case, patient had been suffering from TA. The patient was treated with Ayurvedic medication and Panchakarma procedures on the general line of management of Vata Vyadhi (various neuro-musculoskeletal diseases). Siragatavata (vitiated Vata Dosha affecting the blood vessels) was considered as an Ayurvedic diagnosis for TA. Indian takayasu clinical activity score (ITAS-2010) scoring and improvement in clinical symptoms were observed in the patient.
| Case Report | | |
A 42-year-old woman reported to the outpatient department of National Institute of Ayurveda Jaipur on April 14, 2015, for complaints of tingling sensation in fingers of the right hand and around neck region, numbness in right upper limb, and right lower limb for few seconds. She had feeling of general fatigue, occasional headache and dizziness when she changed the position of her head. She experienced instant chest pain on the right side which was not related with any activities or time of the day.
The patient had been experiencing these complaints for 16 months. She had consulted rheumatology department of an allopathic hospital and a year ago before coming to Ayurveda hospital. Magnetic resonance imaging angiography indicated TA. An absence of arm pulse bilaterally was noticed during first pregnancy in year 2000 following which the blood pressure was monitored in the lower extremities. Transient cyanosis in hands was noticed during the first pregnancy just before delivery. In 2004, she had right thoracic herpes zoster which resolved completely. The patient had also suffered from longitudinal extensive transverse myelitis (LETM) in 2014 from which she completely recovered clinically. She was on prednisolone 60 mg for a day and mycophenolate mofetil 1000 mg twice a day since August 23, 2014 for TA but had stopped the treatment from last 3 months due to increased weight and abnormal liver profile. She was admitted in the inpatient department of Panchakarma department of National Institute of Ayurveda on April 14, 2015, for the administration of Panchakarma procedures.
Clinical findings
On physical examination, patient was anxious, body weight was 79 kg, height was 155.7 cm and body temperature was 37.5°C. Patient had Vata Pitta pre dominance Prakriti with Madhyama Sara (medium strength), Madhyam Samhanana (medium built), Sama Pramana (proportionate body), Madhyama Satmya (medium homologation), Madhayam Satva (medium mental strength), Madhyam Vyayamshakti (medium capability to carry on physical activities), Madhyama Ahara Shakti (medium food intake capacity) and Uttama Jaranashakti (optimum digestive power).
On neurological examination, higher mental function-attention, memory, calculation abstract thought, spatial perception, visual and body perception, and speech were normal. On cranial nerve examination, Grade I nystagmus to left gaze was found. Visual acuity was normal. Slit-lamp examination of the eye and audiometric results were normal. On motor examination, bulk, tone, power and coordination of upper limbs and lower limbs were normal. Joint position sense and vibration sensation was normal.
On cardiovascular examination, pulse in both upper arms was absent. In the neck region, right carotid pulse was forceful and left carotid pulse was not palpable. Pulse in lower extremities was normal. No bruits on the carotid and renal artery were found. Blood pressure could not be measured from the upper limbs. BP was 120/76 mmHg measured from the lower limbs. Auscultation of the chest showed no heart murmur or crackles. Pulse of the right brachial and radial arteries was palpable but feeble. Pulse of the left brachial and radial arteries was not palpable. Pulse rate and respiratory rate was 60/min and 18/min, respectively. Skin, cardiorespiratory, musculoskeletal, and genitourinary system examination was normal.
Timeline
A detail of the case study and follow-up is given in [Table 1].
Diagnostic focus and assessment
Patient was a known case of TA. CTA of the brain and neck revealed-severe stenosis (approx. 90%–95%) of right subclavian artery, complete block of left subclavian artery and proximal left vertebral artery with normal CTA of brain that is a suggestive of TA [Figure 1]. TA was graded as Type I, on the basis of angiographic findings and in group I, on the basis of Ishikawa clinical classification. These grading denoted the better prognosis of the case. | Figure 1: Supporting material (CT Angiography for brain and neck in July 2014)
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Atherosclerosis, giant cell arteritis, polyarteritis nodosa, infectious aortitis, secondary vasculitides and developmental abnormalities (coarctation of the aorta and marfan syndrome) are the differential diagnoses of TA. Atherosclerotic plaques are commonly found in patients aged 45 years and above and also not usually associated with long segment luminal stenosis. Giant cell arteritis mostly affects patients older than 50 years. Branches of the external and internal carotid arteries are most frequently affected in giant cell arteritis. Polyarteritis nodosa commonly affects 30–50 years old, predominantly males, renal arteries, gastrointestinal arteries as the primary sites diseased and multiple small aneurysms in the involved artery. Development abnormalities are not associated with stenotic lesions in large vessels. Varicella zoster virus (VZV) culture and deoxyribonucleic acid (VZV), serology triple and oligoclonal bands test were negative for the case so infectious aortitis (tuberculosis, syphilis, Staphylococcus aureus and Salmonella More Details, treponema, cyto-megalo virus or herpes virus) is unlikely. Test for antinuclear antibody was negative and no lupus was found, so secondary vasculitides (systemic lupus erythmatosus and sarcoidosis) were ruled out. Antiaquaporin-4 (NMO-IGG) antibodies were seropositive as the patient was suffering from longitudinally extensive transverse myelitis (LETM) in past. Hence, TA was the diagnosis for the patient [Table 2]. Any disease can be considered as Vata Vikara (diseases by Vata Dosha) if it have the symptoms of Shosha (atrophy/weight loss), Shoola (pain), Supti (paresthesia, tingling sensation), Sankocha (concentric wall thickening/contracture), Stambhana (stenosis/stiffness), and Khanjata (limping).[5] All these symptoms remain present in TA, hence, it can be considered as Vata Vikara. More specifically, TA can be correlated with Siragata-Vata, a disease of Vata Vyadhi spectrum. Manda Sharira Ruja (mild body pain) Shopha (inflammation) and Shosha (atrophy/loss of weight), Spandana (fasciculation) in the body, Supta Sira (absence of pulsation in the blood vessels/arteries), Tanavyo Sira (spasm of vessels leading to reduction of lumen/thinning of vessels/stenosis) and Mahat Sira (dilatation of vessels/aneurysm) are the manifestation of the Siragata-Vata.[6] Aneurysms, segmental narrowing, and variations in the calibre of arteries and stenosis/occlusions are the manifestation of vasculitis[7] and these are also the manifestation of Siragata Vata. TA is also described as pulse less disease and having stenosis of the large vessels as cardinal feature. These symptoms are described as Supta Sira and Tanavyo Sira in Siragata-Vata disease. Hence, Siragata-Vata was considered as the Ayurvedic diagnosis for this case of TA.
Therapeutic intervention
There is no specific treatment advised for Siragata-Vata in classical texts. Hence, general line of treatment of Vata Vikara viz. Snehana (oleation), Swedana (sudation) Niruha Basti (enema mainly with decoction), Anuvasana Basti (enema with medicated oil), and Nasya Karma (nasal therapy) was adopted for the patient.[8] According to Ayurveda, Siragata-Vata is a disease of Madhyama Roga Marga (disease pathway related to vascular system and other vital organs); hence, Basti Karma was also adopted for treatment. Castor oil in the dose of 20 ml with milk was given for first three consecutive nights for Koshtha Shuddhi (evacuation of the bowel). Shastikshali Pinda Swedana was done for 16 days starting from the 1st day. From 4th day, Erandamuladi Niruha Basti[9] alternated with Anuvasana Basti of Ashwagandha Taila[10] was given for 16 days. After the completion of Basti procedure, Shirovirechana (nasal therapy for purification), a form of Nasya Karma with Triphaladi Taila (oil) in a dose of 06 drops (0.4 ml)/nostril was done on alternate days for 7 days [Table 3]. Ayurvedic oral medicines such as Yogaraja Guggulu-1 g (500mgx2tab), Shiva Gutika-500 mg, Dashamula Kvatha-40 ml, Narsinha Churna-3 g, Brihadvatachinamani Rasa 125 mg twice a day were prescribed to the patient [Table 4]. These oral medicines except Brihadvatachinamani Rasa were prescribed up to 30 January 2016. Brihadvatachinamani Rasa was prescribed initially only for 1 month. Same Panchakarma procedure was repeated after 6 months from November 17, 2015 to December16, 2015. Chyavanaprasha Avaleha in the dose of 10 g with milk was advised twice a day after meals from February 2016 to June 2018.
Follow-up and outcome
Patient's condition was assessed on Indian ITAS-2010, which is validated in Indian patients for disease activity of TA.[11] Features of domains – systemic, abdomen, cardiovascular system, genitourinary, renal (systolic and diastolic hypertension), and nervous system are comprises in ITAS2010 and are scored if new or worse over the past 3 months. Weightage are given for diastolic hypertension, bruits, new pulse loss, pulse inequality, stroke, carotidynia and claudication. A maximum score of 51 is possible in this scoring and score of 4 or more is considered active. Score was 17 at the time of admission and it changed to 05 after completion of Panchakarma procedures [Table 5]. After 1 month of treatment, BP was measurable from both upper limbs, but pulse in the left upper limb and left carotid artery was weak. Unequal BP in both upper limbs was noted. BP in the right hand was 120/76 mmHg and in the left hand was 110/68 mmHg. MRA which was done on June 25, 2015 showed that the TA was stable and there was no new lesion [Figure 2]. ITAS-2010 score was 03 after 06 months. MRA done on November 2015 and CT angiograph done on January 29, 2016 and July 1, 2016 revealed no new lesion for TA in the case [Figure 3] and [Figure 4]. ITAS-2010 score was 4 on June 13, 2018. Follow-up of the patient was scheduled annually since June 2018. The patient was assessed on June 21, 2019 and her condition was stable and there was no relapse of any manifestation at that time. | Figure 3: Supporting material (CT Angiography for brain and neck in November 2015)
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 | Table 5: Indian Takayasu arteritis scoring (Indian Takayasu clinical activity score-2010)
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| Discussion | | |
TA disease is having the close resemblance with Siragata-Vata. The disease is included in Vata Vyadhi. General line of treatment of Vata Vikara, namely Snehana, Swedana, Asthapana, Anuvasana and Shirovirechana was adopted in the patient. Mridu Virechana was given with castor oil. Shastikshali Pinda Swedana which is a combination of Abhyanga (massage) and Mridu Swedana (mild sudation) was given to the patient. It was done on whole body as TA can affect multiple organs. It is shown to provide a good result in the management of various Vata Vyadhi.[12],[13] In TA, occlusion and stenosis of artery and aorta are more prominent. Shiragranthi (knot in micro-channels) type of Srotodushti (vitiation of micro-channels) is the pathogenesis of stenosis. Stenosis is considered in Ayurveda as Margavarodha (obstruction in natural passage of Vata Dosha) or Stambhana or Tanvi Sira and can be removed by Shastikashali Pinda Swedana and Basti procedures. Erandamuladi Niruha Basti is helpful in treating Vata Kaphaja (diseases due to Vata Dosha and Kapha Dosha) disorders, Pakshaghata etc. It has Srotoshodhana (purification of micro channels) property hence it may remove occlusion of vessels. Right subclavian artery, left subclavian artery and left vertebral artery were affected in the case which affected the blood supply for both arms and the head. Shirovirechana is indicated for the diseases above the clavicle region. Hence, Shirovirechana was done with Triphaladi Taila which has Vata Kaphahara (suppression and removal of deranged Vata Dosha and Kapha Dosha) property.[14] It may be helpful in removing the obstruction of Vata Dosha at supra clavicular region. Brihadvatacintamani Rasa is indicated in all type of Vataja (disease due to Vata Dosha) and respiratory diseases.[15] Dashamula Kwatha is useful in all types of Vataja and respiratory disorders and has Tridoshaghna (alleviating deranged Dosha of the body) property.[16] Yogaraja Guggulu is useful in all types of Vataja (neurodegenerative) disorders.[17] Shiva Gutika can treat Shosha (emaciation or weight loss) and has Rasayana (immunomodulatory) property and helpful in diseases of mouth, head and eye.[18] Narsinha Churna is indicated in all types of Vata Vyadhi and it is also having the property of Vajikarana (aphrodisiac property).[19] The combination of all these drugs may treat all the manifestation and complication due to TA. Brimhana (nourishment up to tissue level) is the main treatment of Nanatamaja Vata Vyadhi and Rasayana must be prescribed to any chronic Vata Vyadhi.[20] Brahadvatacintamani Rasa has Rasayana property and is popular in Ayurvedic practices for various diseases of rheumatic spectrum. Chyavanaprasha Avaleha[21] is important for longer uses as Rasayan and is indicated in chronic Vatavyadhi, Nanatmaja Vata Vikara and Avrita Vata Vikara.[22] Thus, these combinations of Ayurvedic oral medications are useful in treating the patient.
The modern treatments of TA have lots of adverse effects.[23] There is a need to watch liver profile at certain interval as changes in liver profile are more prominent during high dose steroid and mycophenolete mofetil administration. In the present case, ESR, serum glutamic-oxaloacetic transaminase and serum glutamic pyruvic transaminase levels were within normal limits after 1 year of Ayurvedic management. This may suggest the safety of Ayurvedic regime in this case. Pulse was noticeable in both upper limb and BP was measurable from upper limbs. This might be considered as remarkable improvement, since very few cases published in Pub Med reported this improvement even after using high doses of steroid.[23] It is now accepted that approximately half of patients of TA which are treated with steroids may respond.[24] There is uncertainty in success and also more side effects are associated with use of steroid, biological agents and mycophenolate mofetil. Apart from this multi-centric trial involving 34 patients for efficacy of abatacept (CTLA4-Ig)) in maintaining relapse-free survival of TA patients over placebo did not revealed any difference.[25] The primary aim of the Ayurvedic management was to control the disease activity and preserve vascular competence. ITAS 2010 scores were 4 or more than 4 during most treatment periods. This indicated that the disease was active. However low ITAS 2010 scores showed satisfactory response of the treatment. Ayurveda can only provide palliative management to this case of TA considering the incurable nature of the disease. However considering the uncertainty about complications of TA the case needed periodical imaging, cardiovascular assessment and surgical intervention if symptoms worsen. Arterial blood analysis must be done periodically which was lacking in this study due to patient's reluctant nature and economic status. The patient was advised to consult annually. The condition of the patient was stable when she was lastly assessed. There was no worsening in disease condition. This was an important finding considering the prognosis of the disease. Understanding of the etiology and pathophysiology of the Siragata-Vata disease may be helpful in the context of TA which is mostly unknown. The findings of the case is important as it throws new light on the possible treatment of TA through Ayurvedic management. This case study shows that Ayurvedic management may be beneficial in the management of TA.
| Conclusion | | |
The case study shows that Takayasu arteritis (TA) was managed with Ayurvedic medication and Panchakarma procedures with satisfactory outcome. More studies are required to be done to confirm these findings and establish the place of Ayurvedic line of treatment in the management of TA.
Patient consent
Written permission for publication of this case study had been obtained from the patient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Patient's perspective
Patient was satisfied with the provided treatment.[Video-1]
Financial support and sponsorship
The treatment and consultation of the patient was done at the nominal registration fees and most of the medication, therapy and investigation were provided free of cost by the institute. MRA cost and some of the medicines which are not provided by the institute were afforded by the patient.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]
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