SAVI-(String-Associated Vasculopathy-Infancy Dependent)- Causes and Ayurvedic Management

ABSTRACT

String-associated vasculopathy is a rare disorder involving abnormal inflammation of the body, especially lungs, blood vessels, and skin. It is an auto-inflammatory disease. The incidence of SAVI is less than 100 cases worldwide (prevalence is unknown). It is an autosomal dominant genetic disorder and the disease was first described in 2014. Let’s discuss it in detail!

INTRODUCTION

It is an autoinflammatory interferonopathy. It is typically seen as systemic inflammation, severe cutaneous vasculopathy, and interstitial lung disease that occurs in infants probably till 1 year of age. The sting is normally found in the cytoplasm, and the mutant body is found in the Golgi apparatus. It occurs due to mutation in the STING1 gene (Gain of function mutations) and decreased immunity due to disturbance of homeostatic control.

SIGNS AND SYMPTOMS

• Low-grade fever
• Recurrent cough
• Associated with progressive interstitial lung disease that can lead to lung fibrosis and difficult breathing.
• Severe Skin lesions on fingers, nose, cheek, ear, and toes, which get exaggerated by cold exposure, tissue damage, and necrosis at a later stage which may require amputation.
• Vasculopathy (affects blood vessels)
• Swollen lymph nodes
• Purplish skin discoloration
• Joint stiffness
• Rarely myositis (muscle inflammation)
• Failure to Thrive ( no growth rate)

CAUSES

Genetic: It is due to a mutation of the STING1 Gene. That is TMEM173 (stimulator of interferon Gene)

DIAGNOSIS

As per clinical grounds

• Blood Culture- To examine autoantibodies, anemia, C-reactive protein, etc
• Imaging techniques- such as X-ray, and CT – To determine the interstitial lung disease
• Biopsy- skin biopsy or lung biopsy (To check inflammation in capillaries, lung fibrosis, etc).

MANAGEMENT:

AS PER MODERN

There is no specific treatment only supportive management can be done. JAK Inhibitors and Usage of other medications as per the condition is recommended.

AYURVEDIC OVERVIEW

Ayurved is a traditional healing system, which heals with the help of herbal formulations, yoga, and diet, it helps in curing disease from the roots. Ayurveda aims to restore the health of the healthy person and to treat the diseased person. It is treated on the basis of dosha, dhatu, and mala. There are 3 dosha’s (vata, pitta, kapha) and 7 dhatus. Acc to Ayurveda it can be considered as vata kaphaja vyadhi (Predominance of vata and kapha) as well as ayurveda describes genetic disorders as kulaja roga and sahaja roga or adibala pravritta which may occur due to beeja dusti (Beejbhagavayava) and other factors such as regime of mother, an environment of the uterus. It is an autosomal dominant pattern that occurs due to a gene mutation that occurs in formation in intrauterine life or early embryonic development.

This can be Diagnosed with Darshana ( Direct perception), Sparshana (Touch Perception), Prashan (Questioning).

DIET

Diet is considered an important factor for the treatment because as per Ayurveda proper food and nutrition help in early recovery. Take foods that are homemade, fresh vegetables, fruits and lean protein. Avoid processed foods and carbonated drinks.

LIFESTYLE MODIFICATION

Normal exercises, yoga, meditation, and proper sleep. They help in relieving stress as well exercise helps in increasing immunity. HERBS – Herbs such as amla, brahmi, ashwagandha, manjishtha, Haridra, Guduchi, shallaki, etc are useful in managing this condition.

Guduchi (Tinospora cordifolia)

It helps in relieving symptoms of inflammation, Improves immunity, and aids digestion.

Shallaki (Boswellia curcumin)

It subsides pain due to the pacification of vata.

Ashwagandha (Withania somnifera)

Ashwagandha enhances immunity and provides strength to the body as well as reduces inflammation.

Haridra (Curcuma longa)

Haridra acts as an anti-inflammatory and has antibacterial properties. In this condition, it acts as an anti-inflammatory.

Amla (Emblica officinalis)

Amla has oxidative properties which promote health as well as anti-inflammatory properties.

Brahmi

it helps to alleviate mental stress due to disease as well as provides calmness.

HERBAL REMEDIES FOR String associated vasculopathy BY PLANET AYURVEDA.

Planet Ayurveda is an ayurvedic clinic that does holistic healing through herbs and dietary modifications. They also have manufacturing units that are GMP and ISO certified and manufacture various classical as well as patent medications. It has been serving mankind for about more than 20 years. It has its own herbal packs, oils, syrups, tablets, capsules, etc. The supplements are 100% safe, pure, and natural made under the guidance of MD doctors. You can find the medicine and treatment at www.planetayurved.com.

Product List

1. Boswellia Curcumin
2. Arvind Asav
3. Syrup Infee
4. Capsule Manjishtha

PRODUCT DESCRIPTION

1. BOSWELLIA CURCUMIN

Boswellia curcumin is an ayurvedic formulation that is available at planet ayurveda which includes standardized extract of Shallaki (Boswellia serrata) and curcumin (Curcuma longa) . It is useful in pain-related, Inflammatory, and degenerative disorders. Both these drugs together work upon the vata dosha which can eliminate the pain, thus pacifying the vata dosha.

Dosage: 1 capsule twice daily, after meals with plain water.

2. ARVIND ASAV

It is in a liquid form which consists of Amla (Emblica officinalis), Arjuna (Terminalia arjuna), Shahad (Honey), Water, mulethi (Glycyrrhiza glabra), Manjishtha (Rubia cordifolia), Gambari (Gmelina arborea), etc. It is a classical medicine as well as available on the planet Ayurveda. It is an anti-inflammatory, antioxidant useful in all health conditions pertaining to children. It increases strength and improves Appetite.

Dosage: 2 tablespoons twice daily.

3. SYRUP INFEE

Infee syrup is an ayurvedic medicine that includes yashtimadhu (Glycyrrhiza glabra) and Guduchi (Tinospora cordifolia). It is in liquid form. It builds immunity and aids digestion. It acts as a protective shield for children. It is an immunomodulating syrup that protects children from frequent infections such as respiratory and GIT infections.

Dosage: 2 Tablespoons twice daily

4. MANJISHTHA CAPSULE

It consists of Manjistha (Rubia cordifolia), it is an herbal formulation in the form of a capsule available at Planet Ayurveda. Manjishtha helps in skin conditions and does Rakta shodhana ( Blood purification). It also acts as an anti-diabetic, anti-cancer, and anti-platelet.

Dosage: 1 capsule twice a day.

Contact Planet Ayurveda Support Team to provide you the costing / ordering and delivery information at – costing.planetayurveda@gmail.com or Call at 0172-521-4030 (India), +91-172-521-4030 (Outside India) or Whatsapp at (+91) 9915-593-604

CONCLUSION

SAVI (String-associated vasculopathy-infancy dependent) is a rare and autoinflammatory condition, It is caused due to mutation in the STING1 gene, it has no specific treatment but can be only managed and can be life-threatening in some cases if not managed. According to Ayurveda, it is due to Beeja dusti (ovum and sperm) of the parents during the formation of intrauterine life. This condition can be managed by different modes such as diet, lifestyle, herbal remedies, etc. you can find the herbal remedies and treatment at www.planetayurveda.com